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70 year-old woman with a history of CAD recently noted abdominal mass who presents with fevers/rigors and bandemia. Over the last few weeks leading up to admission, she has been experiencing mid-abdominal pain, radiating to the left flank. It lasts throughout the day is not increased by eating though there is associated vomiting and is worsened with coughing. CT abdomen without contrast was then performed on [**4-20**] showing a large 9.5 x 7.5 x 6.0-cm heterogeneous left upper abdominal mass. Patient underwent a EUS with biopsy. The results of the biopsy were consistent with pancreatic adenocarcinoma at the head of pancreas. Splenic flecture/pancreatic tail mass was also seen on CT, likely diverticular abscess given the patients recent likely history of diverticulitis this was thought to be an infected fluid collection or abscess. She was treated with IV antibiotics (Zosyn, then ceftriaxone and flagyl) and will continue on them until seen by ID as an outpatient. Past Medical History: 1. Coronary artery disease with history of angioplasty in [**State 108**] one year ago 2. Mitral valve prolapse 3. Atrial fibrillation 4. Hyperlipemia 5. Hypertension 6. Chronic kidney disease (SCr 2.1 in [**3-17**]) 7. Hypothyroidism? (TSH 10 in [**3-17**]) 8. Anemia (HCT | eligible ages (years): 0.0-999.0, Antithrombotic Therapy Perioperative Care Perioperative Outcomes Patients with continuing antithrombotic therapy (including antiplatelet and/or anticoagulant therapies) for cardiovascular and cerebrovascular diseases for more than 2 weeks Patients who have one or more of the following situations: (1) non-valvular atrial fibrillation with a CHA2DS2-VASc score >4; (2) post-artificial valve replacement (including mitral valve, ball-cage valve or tilting disc aortic valve); (3) venous thromboembolism occurred within 1 year, or venous thromboembolism with other high-risk factors which require long-term anticoagulation treatment; (4) coronary heart disease with coronary stent implantation, thrombolytic therapy or other coronary procedures within 2 years, or who had undergone coronary artery bypass grafting within 1 year, or who experienced myocardial infarction or acute coronary syndrome within 1 year; (5) a history of stroke/transient ischemic accident within 1 year, or a history of cervical or intracranial revascularization treatment within 1 year; (6) peripheral arterial disease Patients who are scheduled to undergo intra-abdominal surgery (including general and urologic surgery) Patients who sign written informed consents Refuse to participate Emergency surgery Unable to complete the preoperative assessment and follow-up plan Other reasons that are considered unsuitable for study participation by the investigators or the attending doctors | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Congestive Heart Failure To be enrolled, patients must: 1. be veterans, 2. have moderately severe CHF (NYHA class II, III or IV), 3. be 18 or older, 4. not have clinically important renal, hepatic or hematological disorders or clinically significant abnormal laboratory findings, 5. not have a pre-existing thyroid disease, 6. not have anemia (hematocrit less than 30%), 7. not have chronic pulmonary disease that limits exercise tolerance or requires use of chronic bronchodilator therapy or steroids, 8. be able to walk on the level for 6 minutes, 9. not have hemodynamically significant pericardial disease, 10. not have angina pectoris severe enough to require frequent administration of sublingual nitroglycerin, 11. not have acute myocardial infarction within 6 months of screening, 12. not have inoperable aortic stenosis, 13. not have symptomatic ventricular arrhythmias or ventricular arrhythmia requiring pharmacological therapy, 14. not have implanted cardioverter defibrillator, 15. not be taking amiodarone, 16. not have demonstrated non-compliance with prior medical regimes; 17. not be on an investigational drug, 18. not have a medical condition that, in the investigator's opinion, would make the patient ineligible, 19. not have an allergy to iodine or shellfish, 20. not be in sinus rhythm, 21. not be of childbearing potential, 22. have an ejection fraction greater than 40% | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-999.0, Pulmonary Disease, Chronic Obstructive Patients with chronic obstructive pulmonary disease | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Disease Stroke Ischemic Heart Disease Myocardial Infarction Cardiac EF <=35% by radionuclide ventriculography, left ventriculography or quantitive echocardiographic measurement or an echocardiographic Wall Motion Index of <=1.2, within three months of enrollment. The patient's clinical cardiac state at enrollment should be similar to their state at the time of the qualifying echocardiogram. The qualifying left ventricular function measurement must be obtained at least three months after an MI, coronary bypass grafting, PTCA, and at least one month after pacemaker insertion. Patients scheduled for mitral valve repair should have qualifying echo after surgery Modified Rankin score <=4 Patient must be taking ACE inhibitors. If intolerant of ACE inhibitor, patient must be on angiotensin II receptor blockers or hydralazine and nitrates Patient is able to follow an outpatient protocol (requiring monthly blood tests and clinic visits every four months for the duration of the study) and is available by telephone Patient understands the purpose and requirements of the study, can make him/herself understood, and has provided informed consent Patients with recent stroke or TIA within twelve (12) months will be eligible to be included in the recent stroke (RS) subgroup Chronic CHF patients (NYHA I * IV) admitted to the hospital can be randomized prior to discharge if the patient is stable, taking oral medications for 24 hours and ambulatory at the time of discharge. Stable New York Heart Association Class IV patients will be eligible for randomization The presence of any of the following unequivocal cardiac sources of embolism: chronic or paroxysmal AF, mechanical valve, endocarditis, intracardiac mobile or pedunculated thrombus, and valvular vegetation Cyanotic congenital heart disease, Eisenmenger's syndrome Decompensated heart failure Cardiac surgery, angioplasty, or MI within the past 3 months prior to randomization A contraindication to the use of either warfarin or aspirin, e.g. active peptic ulcer disease, active bleeding diathesis, platelets <100,000*, hematocrit <30, INR >1.3 (if not on warfarin), clotting factor abnormality that increases the risk of bleeding, alcohol or substance abuse, severe gait instability, cerebral hemorrhage, systemic hemorrhage within the past year, severe liver impairment (AST >3x normal*, cirrhosis), any condition requiring regular use of non-steroidal anti-inflammatory agents, allergy to aspirin or warfarin, uncontrolled severe hypertension (systolic pressure >180 mm Hg or diastolic pressure > 110 mm Hg), positive stool guaiac not attributable to hemorrhoids, creatinine >3.0*. *on most recent test done within 30 days prior to randomization Patient needs continuing therapy with intravenous heparin or low molecular weight heparin or a specific antiplatelet agent Dementia or psychiatric or physical problem that prevents the patient from following an outpatient program reliably Comorbid conditions that may limit survival to less than five years Pregnancy, or female of childbearing potential who is not sterilized or is not using a medically accepted form of contraception* (see procedure manual). *A pregnancy test is required for all women of childbearing age Enrollment in another study that would conflict with | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 60.0-999.0, Heart Failure, Congestive Dyspnea Pulmonary Edema Men or women ≥ 60 years of age. 2. Diagnosis of congestive heart failure with one or both of the following a Heart Failure Clinical Score ≥ 3 based on the and/or a history of either acute pulmonary edema or the occurrence of 2 or more of the following with subsequent improvement with diuretic therapy and with no other identifiable cause: dyspnea on exertion, paroxysmal nocturnal dyspnea, orthopnea, systemic edema, exertional fatigue. 3. Left ventricular ejection fraction ≥ 50% based on the baseline Doppler echocardiography test. 4. Ability to provide written informed consent. 5. Ability to comply with procedures specified in the study protocol Valvular heart disease as the primary etiology of congestive heart failure. 2. Significant change in cardiovascular medication(s) <3 weeks prior to the baseline visit. 3. Uncontrolled hypertension. 4. History of stroke, or any sequelae of a transient ischemic attack (TIA), reversible ischemic neurologic defect (RIND), or stroke, within the last 12 months prior to entry into the study. 5. Cancer or other noncardiovascular conditions with life expectancy less than 2 years. 6. Significant anemia defined as a hemoglobin <11 gm/dL. 7. Significant renal insufficiency defined as a serum creatinine >2.5 mg/dL. 8. Significant hepatic insufficiency defined as an SGPT (ALT) or SGOT (AST) >2.5 times the upper limit of normal. 9. Psychiatric disease (including uncontrolled major psychoses, depression, dementia, or personality disorder) or any additional condition(s) which, in the investigator's opinion, would prohibit the patient from completing the study, or not be in the best interest of the patient. 10. Presence or history of drug or alcohol abuse. 11. Prior exposure to ALT-711 or use of any other investigational drugs within 30 days prior to screening. 12. Known seropositivity for HIV or hepatitis C, or presence of hepatitis B surface antigen. 13. Severe COPD requiring recurrent oral steroids, oxygen at home or more than one inhaler. 14. Baseline echocardiogram demonstrating the presence of left ventricular ejection fraction <50%. 15. Unstable or uncontrolled myocardial ischemia, with no wall abnormality. 16. Screening Familiarization Exercise Test demonstrating the presence of any of the following findings: evidence of significant ischemia (consisting of ECG finding of > 1 mm flat ST depression confirmed with echocardiogram wall motion, or wall motion abnormality or decrease in global contractility on echocardiogram, or inability to continue exercising due to significant chest or leg pain or any reason other than exhaustion/fatigue/dyspnea, exercise SBP >240 mm Hg, DBP >110 mmHg, unstable hemodynamics or rhythm, or unwilling or unable to complete test adequately. 17. Any contraindications to magnetic resonance imaging including but not limited to indwelling metal-containing prosthesis (orthopedic, valvular, other), pacemaker or defibrillator, history of welding occupation (ocular metal debris), or uncontrollable claustrophobia | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-99.0, Secondary Pulmonary Arterial Hypertension Pilot: The pilot study will enroll two groups of individuals: 1) patients who have either IPAH or a secondary form known to have similar histopathology (PAH), and 2) age, gender, and race matched control subjects for each patient. Main: The main study will enroll three groups of individuals: 1) patients who have either IPAH or a secondary form known to have similar histopathology (PAH), 2) patients with PH ascribed to a nonvascular injury process, and 3) age, gender, and race matched control subjects for each PAH patient. Subjects must be at least 18 years of age and must be able to provide informed, written consent for participation in this study. There is no based on race or gender. FOR The for this study are as follows: 1. Patients diagnosed with IPAH 2. Patients diagnosed with secondary pulmonary hypertension known to have histopathology similar to the primary form or PAH. Clinical conditions causing pulmonary hypertension with histopathology similar to the primary form are listed below. i. Eisenmenger Syndrome ii. Collagen vascular disease iii. Liver disease with portal hypertension iv. Toxin induced injury (anorexic agents, rapeseed oil) v. HIV disease vi. Sickle cell disease FOR WITH 1. Pregnant women (all women of childbearing age will be required to have a screening urine or blood pregnancy test) 2. Age less than 18 years 3. Inability to provide informed written consent for participation in the study 4. Mean PA less than or equal to 25mmHg or PVR less than 3 wood units 5. PCW greater than 16 mmHg unless increase accounted for by increased transpulmonary gradient greater than or equal to 10 mmHg 6. Patients receiving more than 1 year of oral therapy or more than 6 months of IV therapy. FOR WITH The are as follows: Patients diagnosed with pulmonary hypertension not known to have histopathology similar to the primary form. Etiologies are listed below. 1. Congenital or acquired valvular or myocardial disease 2. Pulmonary parasitic diseases 3. Arterial hypoxemia with hypercapnea 4. COPD with hypoxemia and forced expiratory volume/forced vital capacity (FEV1/FVC) greater than 2 standard deviations from normal 5. Interstitial lung disease with reduced total lung capacity greater than 2 standard deviations from normal and infiltrates on chest x-ray 6. Pulmonary thromboembolic disease as evidenced by lung perfusion scan or pulmonary angiogram, or intravenous drug abuse 7. Pulmonary hypertension due to congenital abnormalities of the lungs, thorax and diaphragm FOR WITH 1. Pregnant women (all women of childbearing age will be required to have a screening urine or blood pregnancy test) 2. Age less than 18 years 3. Inability to provide informed written consent for participation in the study 4. Mean PA less than or equal to 25mmHg or PVR less than 3 wood units FOR MRI IN WITH 1) Implanted cardiac pacemaker or defibrillator 2) Central nervous system aneurysm clips 3) Cochlear implants 4) Neural stimulator 5) Ocular foreign body (e.g. metal shavings) 6) Insulin pump 7) Metal shrapnel or bullets 8) Claustrophobia. Furthermore, the following patient groups will be excluded from studies involving the administration of MRI contrast agents: 1) lactating women 2) renal disease (CrCl less than 20 ml/min) The creatinine clearance (CrCl) will be calculated using the Cockroft formula where age is in years, kg is weight in kilograms, and Cr is the serum creatinine. If there is no history of kidney disease from the patient or referring physician, additional testing will not be performed. If a patient has a history of renal insufficiency, a recent blood Cr will be used unless the physician performing the test believes the Cr may have changed since the last test. If the Cr may have changed, a blood sample will be obtained for Cr or the subject will be excluded from receiving gadolinium. CrCl = (140-age) (wt in kg)/72 X serum Cr (mg/dl) for men CrCl = (0.85) (140-age) (kg)/72 X serum Cr (mg/dl) for women FOR 1. Any healthy man or woman who is the appropriate age, race, and gender for matching to a PAH patient 2. No history of HIV infection 3. EKG and echocardiogram with no evidence of clinically relevant heart disease 4. Spirometry with no evidence of clinically relevant lung disease 5. No history of causes of pulmonary hypertension such as collagen vascular disease, chronic liver disease with ALT or AST greater than 2 times the upper limit of normal or cirrhosis of the liver, chronic thromboembolic disease, congenital heart defects, or pulmonary parenchymal disease with hypoxemia 6. No history of diseases thought to be related to development of endothelial dysfunction including systemic hypertension or diabetes requiring drug therapy, hypercholesterolemia and obesity 7. No history of anemia, thrombocytopenia or coagulopathy 8. No history of renal insufficiency 9. No medical conditions requiring chronic medication use with the exception of: a. Heartburn, GERD b. Environmental allergies, post nasal drip or non-allergic rhinitis c. Asthma with no history of oral steroid use, weekly inhaled steroids, or hospitalization for asthma exacerbation d. Dermatologic conditions that do not require the use of oral steroids or other immunosuppressants e. Treated and stable thyroid disease, depression, or anxiety. 10. No more than 20 cigarettes per years for the previous 2 years and no cigarette use for 30 days prior to the screening evaluation until completion of the study FOR 1. Current pregnancy, lactation or women not currently using medically acceptable birth control. (All women of childbearing age will be required to have a screening urine or blood pregnancy test) 2. Contraindication to MRI scanning including individuals with the following devices: A) Implanted cardiac pacemaker or defibrillator B) Central nervous system aneurysm clips C) Cochlear implants D) Implanted Neural stimulator E) Ocular foreign body (e.g. metal shavings) F) Insulin pump G) Metal shrapnel or bullet H) Claustophobia 3. Contraindications to MRI contrast agent administration 4. Inability to provide informed written consent for participation in the study 5. Chronic, medically refractory atrial tacharrhythmias 6. Symptoms of heart failure. 7. Mean PA greater than 25mmHG or PVR greater than 1.5 wood units, or PCWP greater than 15 mmHg 8. History of recreational drug use with the exception of marijuana. No marijuana use within 3 months of protocol screening through completion of the study. 9. Intravenous drug abuse. Volunteers may be excluded if in the opinion of the study investigators they have a condition that may adversely affect the outcome of the study or the safety of the volunteer | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, B-cell Chronic Lymphocytic Leukemia Prolymphocytic Leukemia Refractory Chronic Lymphocytic Leukemia Histologically confirmed B-cell chronic lymphocytic leukemia or prolymphocytic leukemia requiring treatment, defined by 1 of the following Massive or progressive splenomegaly and/or lymphadenopathy Anemia (hemoglobin < 11 g/dL) OR thrombocytopenia (platelet count < 100,000/mm^3) Weight loss > 10% within the past 6 months Grade 2 or 3 fatigue Fevers > 100.5°F or night sweats for > 2 weeks with no evidence of infection Progressive lymphocytosis with an increase of > 50% over a 2 month period OR an anticipated doubling time of < 6 months Relapsed disease Failed prior fludarabine or pentostatin therapy OR cannot receive fludarabine Lymphocyte count ≥ 5,000/mm^3 | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-999.0, Chronic Obstructive Pulmonary Disease Diagnosis of COPD, clinically stable for 1 month Spirometry results showing at least mild disease (FEV1/FVC < 70% and FEV1 < 80% predicted after bronchodilator) ADL limited by dyspnea Ability to speak English and sign consent form Actively use computer and the Internet Maintain O2 saturation > 85% on < 6 L/min of nasal oxygen during the six minute walk Understands and is able to rate shortness of breath during exercise Active symptomatic illness other than COPD Formal pulmonary rehabilitation training in the past 6 months | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure, Congestive Patient receives all cardiology care at a VISN 11 VAMC Must have chronic heart failure (CHF) secondary to systolic or diastolic dysfunction, as defined by meeting (1) or (2) below: 1. Systolic Dysfunction, defined by a history or documentation of LVEF = 40% (or fractional shortening = 20%-if LVEF not documented) AND at least ONE of the following Current diagnosis or history of CHF and/or Physical findings and/or symptoms of CHF within two months of enrollment and/or Hospitalization for CHF within 12 months OR 2. Diastolic Dysfunction, defined by a history of documentation of LVEF > 40% (or fractional shortening > 20% if LVEF not documented) AND Physical findings and/or symptoms of CHF within two months of enrollment Based on medical record data, patient does not have any co-morbidity other than CHF which, in the opinion of the investigator, limits life expectancy to less than six months (e.g., metastatic CA, hospice care, renal failure with a creatinine above 5.0 mg/dL or on dialysis, HIV) | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-999.0, Pulmonary Disease, Chronic Obstructive Male or female of African descent years of age or older Diagnosis of COPD History of smoking at least one pack per day for at least 10 years Currently experiencing shortness of breath at least with exertion Asthma Recent myocardial infarction or hospitalization for congestive heart failure | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Pancreatitis All patients (men and women) more than 18 years old who are going to have an ERCP procedure performed at the different centers from September 1, 2004 to about January 31, 2005 will be included Before the study: active acute pancreatitis (defined as: acute upper abdominal pain and S-amylases x 3 upper normal limit OR upper abdominal pain + radiological findings [CAT-/MR-scan] consistent with acute pancreatitis OR pathoanatomical findings consistent with acute pancreatitis by surgery) Known previous sphincterotomy Chronic pancreatitis with known calcifications Hypotension (definition: systolic blood pressure < 100 mmHg) Anemia, men/women (hemoglobin < 6 mmol/l or <9.7 g/dl) Constrictive pericarditis Pericardial tamponade Hypertrophic obstructive cardiomyopathy, aortic stenosis Mitral stenosis sildenafil within 24 hours before the ERCP procedure and 24 hours after the procedure Hypersensibility to nitroglycerine | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 6.0-999.0, Cystic Fibrosis Compliance with Studies CP-AI-005 (NCT00104520) or CP-AI-007 (NCT00112359) by taking at least 50% of expected study medication Completion of CP-AI-005 or CP-AI-007 or was withdrawn due to need for antipseudomonal antibiotics or for an AE unrelated to study medication tolerance Ability to provide written informed consent/assent prior to initiation of study-related procedures Ability to perform reproducible pulmonary function tests Use of any investigational medication or device between the last visit of CP-AI-005 or CP-AI-007 and Visit 1 of this study Concurrent participation in a study of another investigational drug or device Current use of oral corticosteroids in doses exceeding the equivalent of 10 mg prednisone/day or 20 mg prednisone every other day History of sputum or throat swab culture yielding Burkholderia cepacia in the previous 2 years History of daily continuous oxygen supplementation or requirement for more than 2 liters/minute at night Inability to tolerate study medication in CP-AI-005 or CP-AI-007 Known local or systemic hypersensitivity to aztreonam Inability to tolerate inhalation of a short acting beta-2 agonist Abnormal renal or hepatic function based on results of most recent test Female of child-bearing potential who was pregnant, lactating, or not (in the opinion of the investigator) practicing an acceptable method of birth control | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-0.115, Hernia, Diaphragmatic Hypertension, Pulmonary Hypoplasia, Pulmonary Congenital diaphragmatic hernia 42 days (d) of age Significant illness severity as demonstrated by Receiving assisted ventilation and FiO2 >= 0.40 at 10-14d of age, or FiO2 >= 0.40 for >=48hours at 15-27d of age, or FiO2 >= 0.35 at 28-42d of age Or, need for extracorporeal support at >=10d of age Or, estimated pulmonary arterial or right ventricular systolic pressure of >= 2/3 systemic pressure at 14-42d of age Structural congenital heart disease (other than patent ductus arteriosus or patent foramen ovale/atrial septal defect [ASD] or non-hemodynamically significant ventricular septal defect [VSD]) Sildenafil contraindicated (until condition resolves) Unable to absorb oral medication, or Unstable systemic blood pressure, or Receiving a drug that may interfere with sildenafil metabolism, or Renal insufficiency Hepatic insufficiency Previous use of sildenafil | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Pulmonary Hypertension Candidates for enrollment in this clinical trial are limited to adult patients diagnosed with severe portopulmonary hypertension (PTPH). Specifically, other causes of pulmonary hypertension (PH) such as left ventricular dysfunction, valvular heart disease, chronic lung disease, chronic thromboembolic disease, chronic hypoxemia, rheumatologic conditions, and significant untreated obstructive sleep apnea will be excluded in the usual fashion in patients with underlying portal hypertension and usually cirrhosis. The diagnosis of pulmonary hypertension will be confirmed by invasive hemodynamic measurements (i.e. right heart catheterization) and severe PH is defined as a mean pulmonary artery pressure (MPAP) >35 mmHg. If evidence of right ventricular (RV) dysfunction is present (RV dilation, reduced RV ejection fraction (EF), or elevated RV end-diastolic pressure (>10 mmHg) and the MPAP is 30-35 mmHg, then the patient may be enrolled. Study participants will be selected from those patients referred for hemodynamic assessment of pulmonary hypertension, such as potential liver transplant recipients. They must be capable of giving informed consent. The University of Chicago referring physician will be contacted for concurrence of agreement Patients with pulmonary arterial hypertension, but not portopulmonary hypertension will be excluded. Patients will be excluded from consideration if they do not have severe PTPH, if invasive hemodynamic assessment is contraindicated, or if there are any contraindications to sildenafil. Patients with unstable coronary syndromes or otherwise significant unrevascularized coronary artery disease and myocardial ischemia as determined by cardiac stress testing and/or coronary angiography will also be excluded. Moribund patients will not be considered for enrollment | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-85.0, Chronic Obstructive Pulmonary Disease Chronic Obstructive Pulmonary Disease Aspirin therapy Cardiac Pacemaker - | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 16.0-999.0, Pulmonary Hypertension Patients with pulmonary arterial hypertension caused by primary PAH, associated with connective tissue disease or following surgical repair of a congenital heart lesion PH other than PAH | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 1.0-17.0, Pulmonary Arterial Hypertension, Children Male or female subjects aged from 1 to 17 years old and weighing >= 8 kg with a mean pulmonary artery pressure >= 25 mmHg at rest, PCWP <= 15 mmHg, and PVRI >= 3 Wood units x m2 (if PCWP is not available, then mean LA pressure <= 15 mmHg or LVEDP <= 15 mmHg in the absence of left atrial obstruction) Females of child bearing potential who were sexually active must have been practicing a suitable method of birth control so that in the opinion of the investigator, they would not become pregnant during the study Subjects who have symptomatic pulmonary arterial hypertension due to: primary pulmonary hypertension; pulmonary arterial hypertension in the presence of a small or hemodynamically insignificant congenital systemic to pulmonary shunt lesion that in the opinion of the investigator is not the cause of pulmonary hypertension; collagen vascular disease; congenital systemic-to-pulmonary shunts with a baseline resting room air oxygen saturation >= 88% unrepaired or repaired at least 6 months prior to screening; d-transposition of the great arteries repaired within the first 30 days of life; or surgical repair of other congenital heart lesions at least 6 months prior to screening and do not have clinically significant residual left-sided heart disease consistent with the Subjects, developmentally able to exercise, whose CPX exercise test functional capacity is within the following parameters: Peak VO2 >= 10 mL/kg/min and <= 28 mL/kg/min during screening CPX test Written informed consent and assent where applicable before the subject is screened for the study Subjects who undergo a large shift in altitude (defined as approximately 5000 feet or 1524 meters) in order to participate in the study must reside at the "in study" altitude for at least 90 days prior to baseline and during the study period Subjects with pulmonary hypertension secondary to sickle cell disease, any other disease known to be associated with PAH, or any etiology other than those specified in the criteria Left-sided heart disease, including aortic or mitral valve disease (greater than mild), restrictive or congestive cardiomyopathy; PCWP or LVEDP > 15 mmHg; LVEF < 40% determined by MUGA, angiography or echocardiography; LV shortening fraction < 22% determined by echocardiography, symptomatic coronary disease (demonstrable ischemia) Pericardial constriction; significant (2+ for regurgitation) valvular disease other than tricuspid or pulmonary regurgitation; acutely decompensated heart failure within previous 30 days from screening; atrial septostomy within previous 6 months of screening Hemodynamic instability or hypo or hypertension at screening, i.e., SBP outside of 70-140 mmHg A history of stroke, myocardial infarction or life threatening arrhythmia within 6 months of screening Moderate to severe restrictive pulmonary disease (Total Lung Capacity or Forced Vital Capacity <= 60% of normal) or history of severe lung disease | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 21.0-999.0, Heart Valve Diseases Patients a) 21 years old or older, with b) MR observed with color flow imaging, c) due to organic mitral valve disease demonstrated by Echocardiography (not normal valve as in functional or ischemic MR), c) isolated (no valve disease other than functional tricuspid regurgitation by Doppler-Echocardiography), d) pure (no mitral stenosis by Doppler-Echocardiography), e) quantifiable by Doppler-Echocardiography, f) of degree * moderate, defined as RVol * 30 mL/beat, g) occurring on native valves, h) with echocardiographic imaging allowing assessment of LA and LV, and i) asymptomatic (or mildly symptomatic but not considered as candidates for immediate surgery by their attending physician) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-95.0, COPD Subject is able to ambulate. Defined as a 6MWD greater than 110 meters Subject is diagnosed with COPD years-old and older Currently or previously smoking with a smoking history of at least 10 pack-years Forced expiratory volume on one second (FEV1) after the use of a bronchodilator between 25 an 70% of the predicted value, and FEV1 to forced vital capacity ratio less than 70% No previous diagnosis of asthma, left heart congestive heart failure (either radiographic evidence of pulmonary congestion, echocardiographic or ventriculographic evidence of a reduced ventricular ejection fraction), terminal disease, dementia or uncontrolled psychiatric illness Never participated to a respiratory rehabilitation program and not staying or planning to stay in a long term care facility Subject understands and is able to read and write French or English MRC dyspnea scale of at least 2 The need for supplemental oxygen at rest or during exercise will not be an criterion | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Heart Septal Defects, Ventricular Aortic Valve Insufficiency ventricular septal defect patients receiving total repair from Jan, 1987 to Dec, 2002 follow up more than 1 year after operation complex cardiac lesions | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 55.0-999.0, Heart Failure Chronic Obstructive Lung Disease diagnosis of Chronic Heart Failure diagnosis of Chronic Obstructive Pulmonary Disease living in a nursing home inability to give informed consent involved in other studies of CHF or COPD | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Agoraphobia Social Phobia PTSD One or more of the study diagnoses agoraphobia, social phobia and/or PTSD Ongoing psychosis Ongoing serious drug problems | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 3.0-999.0, Atrial Septal Defect Patent Foramen Ovale ASD PFO with a medical history of cryptogenic stroke due to presumed paradoxical embolism caused by a PFO that catheterization (e.g. size too small, disseminated intravascular coagulopathy (DIC), high risk of bleeding such as coagulation or clotting disorders) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-2.0, Pulmonary Hypertension Informed consent by parents or legal representatives Age: Infants older than 4 weeks of age and children less than 1½ years of age Presence of a left-to-right shunt with increased pulmonary blood flow. The specific defects that will be present are Aortopulmonary (AP) Window Atrioventricular septal defect (AVSD) Double outlet right ventricle (DORV) Total anomalous pulmonary venous drainage (TAPVD) Truncus arteriosus Ventricular septal defect (VSD) Specific cardiac defects Atrial septal defect (ASD) Cyanotic congenital heart disease Univentricular atrio-ventricular connexion Valvular or subvalvular pulmonary or aortic stenosis Specific circumstances Emergency cardiac surgery Children presenting with infection after cardiac surgery Infants on extracorporeal membrane oxygenation (ECMO) before cardiac surgery | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Chronic Obstructive Pulmonary Disease diagnosis of COPD, GOLD 3 or 4 having subjective sleeping problems longer latency to falling asleep frequent arousals excessive daytime sleepiness clinical stable health for minimally 6 weeks usage of some sort of medication that influences sleep in any kind of way (like benzodiazepines, barbiturates, opiates, amfetamines) which can not be discontinued during the study period alcohol abuse hospitalisation 6 weeks or shorter before enrollment in the study hyperreactivity / allergy to benzodiazepines history of benzodiazepine-dependence myasthenia gravis obstructive sleep apnea syndrome (OSAS) severe liver failure age under 18 years participation in another study less than 6 weeks before enrollment | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-999.0, Chronic Obstructive Pulmonary Disease Patients with chronic obstructive pulmonary disease and an acute exacerbation After the acute treatment a simple pulmonary function test shows a value of 30%-60% of the predicted normal value Patients, who, based on the clinical examination after the initial acute treatment, are candidates for a course of oral steroids for the treatment of acute symptoms due to chronic obstructive pulmonary disease Diagnosis/history of asthma Oxygen uptake (saturation) is <92% after the initial acute treatment A requirement for regular use of oxygen therapy Regular treatment with any inhaled steroid >1 000 µg/day at study entry Additional and will be evaluated by the investigator | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 35.0-999.0, Pulmonary Disease, Chronic Obstructive Outpatient with the diagnosis or referral diagnosis of COPD COPD must be the primary reason for this outpatient contact (for both new patients and controls) Any co-morbidity that hampers the diagnosis and treatment of COPD in accordance with the COPD guidelines used locally (e.g. malignant disease, dementia or sequelae of apoplexy) Asthma without the presence of COPD | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Symptomatic Decompensated Congestive Heart Failure Congestive Heart Failure in Acute Coronary Syndrome Patients with dyspnea (difficulty breathing and shortness of breath) at rest, while supine, or immediately upon minimal activity such as talking, eating, or bathing having evidence of heart disease, rather than pulmonary disease, as the primary cause for the dyspnea (by demonstrating at least two of the following: jugular venous distension, paroxysmal nocturnal dyspnea or 2-pillow orthopnea within 72 hours before the start of study drug, abdominal discomfort due to hepatosplanchnic congestion, chest x-ray with findings indicative of heart failure) having elevated cardiac filling pressures either by clinical estimate in non-catheterized patients, or a measured pulmonary capillary wedge pressure (PCWP) >= 20 mm Hg in catheterized patients requiring hospitalization and intravenous therapy for at least 24 hours for the treatment of acutely decompensated heart failure NPatients having systolic blood pressure consistently less than 90 mm Hg having cardiogenic shock (a sudden decrease in blood pressure that results in decreased perfusion of body tissues and organs), volume depletion, or any other clinical condition that would contraindicate the administration of an intravenous agent with potent vasodilating properties having their most recent pulmonary capillary wedge pressure (PCWP) < 20 mm Hg within 24 hours before randomization having a clinical status so acutely unstable that the potential subject could not tolerate placement of a right heart catheter or the 3-hour placebo period unable to have intravenous nitroglycerin withheld (e.g., intravenous nitroglycerin for management of an acute coronary syndrome) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-999.0, Chronic Obstructive Pulmonary Disease Patients admitted with will be recruited for the study. will be defined when a patient with background COPD has at least two of the major symptoms (increased dyspnoea, increased sputum purulence, increased sputum amount) or one major and one minor symptom (nasal discharge/congestion, wheeze, sore throat, cough) for at least two consecutive days patients who are ambulatory (no major joint problem and no Class III or IV angina) patients agree to join exercise programme if offered, the forced expiratory volume in 1 second (FEV1) <70% predicted normal and an age of over 40 years old Patients who have had PRP within the past 1 year will not be recruited for this study Patient who are unable or refuse to consent for the study will also be excluded | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 12.0-85.0, Pulmonary Hypertension Aged 12-85 years; of either gender Confirmed PAH due to idiopathic pulmonary arterial hypertension (IPAH) or familial pulmonary arterial hypertension (FPAH) minute walk distance (6-MWD) between 100-450 meters at screening On a stable dose of sildenafil, with or without bosentan Any treatment for PAH with prostacyclins, prostacyclin analogues, endothelin-1 antagonists, or phosphodiesterase-5 (PDE-5) inhibitors other than sildenafil within the past 12 weeks Pulmonary hypertension due to conditions other than those stated in criteria Additional PAH medications added within the past 12 weeks | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Eisenmenger Syndrome Eisenmenger syndrome Negative pregnancy test Elevated liver enzymes to more than 3´times normal value Hypotension (SBP < 90 mmhg) Mandatory treatment with nitrates Myocardial infarction within 3 months Stroke within 3 months Known allergy to Bosentan or Sildenafil inherited degenerative diseases in retina Breast feeding Suspicion of risc of noncompliance | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 12.0-999.0, Pulmonary Arterial Hypertension Signed informed consent prior to initiation of any study-mandated procedure 2. Males or females >=12 years of age (except for countries where this age limit is contrary to specific regulatory requirements) Women of childbearing potential must have a negative pretreatment pregnancy test and must use a reliable method of contraception during study treatment and for at least 3 months after study treatment termination. ·Reliable methods of contraception are: O Barrier type devices (e.g., female condom, diaphragm, contraceptive sponge) only in combination with a spermicide. O Intrauterine devices. O Oral, transdermal, injectable or implantable contraceptives only in combination with a barrier method Hormone-based contraceptives alone, regardless of the route of administration, are not considered as reliable methods of contraception Abstention, rhythm method, and contraception by the partner alone are not acceptable methods of contraception Women not of childbearing potential are defined as postmenopausal (i.e., amenorrhea for at least 1 year), or documented surgically or naturally sterile. 3. Patients with symptomatic PAH 4. Patients with the following types of PAH belonging to WHO Group I Idiopathic (IPAH) Familial (FPAH) Associated with (APAH): i. Collagen vascular disease with normal left ventricular function (ejection fraction (EF) > 50%) ii. Congenital systemic-to-pulmonary shunts at least 2 years post surgical repair iii. Drugs and toxins 5. PAH diagnosed by right heart catheter showing Mean pulmonary arterial pressure (mPAP) >= 25 mm Hg AND Pulmonary capillary wedge pressure (PCWP) =< 15 mm Hg or left ventricular end diastolic pressure (LVEDP) =< 15 mmHg If both PCWP and LVEDP are available then the LVEDP value is retained for inclusion. 6. Treatment with a stable dose of sildenafil equal to or greater than 20 mg t.i.d. for at least 12 weeks prior to randomization (no sildenafil dosage adjustment should occur in this period) 7)150 m =< 6-minute walk test (6MWT) =< 480 m, documented by 2 tests with second 6MWT within 15% of first 6MWT distance or a third test required 1. PAH belonging to WHO group II-V 2. PAH associated with portal hypertension and HIV infection 3. PAH associated with thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders and splenectomy 4. PAH associated with significant venous or capillary involvement (PCWP > 15 mmHg): pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis 5. Persistent pulmonary hypertension of the newborn 6. Significant valvular disease with valvular lesions to be excluded by echocardiogram within 2 years prior to randomization (i.e. patients with tricuspid or pulmonary insufficiency secondary to PAH can be included) 7. Restrictive lung disease: total lung capacity (TLC) < 60% of normal predicted value (see Appendix 3) 8. Obstructive lung disease: forced expiratory volume/forced vital capacity (FEV1/FVC) < 0.5 9. Moderate to severe hepatic impairment, i.e., Child-Pugh Class B or C 10. Known HIV infection 11. Acute or chronic impairment (other than dyspnea), limiting the ability to comply with study requirements or that may interfere with the safety or the evaluation of the study, such as chronic infection, chronic renal failure etc. 12. Psychotic, addictive or other disorder limiting the ability to provide informed consent or to comply with study requirements 13. Pregnancy or breast-feeding 14. Condition that prevents compliance with the protocol or adherence to therapy 15. Systolic blood pressure < 85 mmHg 16. Body weight < 40 kg 17. Hemoglobin <75% of the lower limit of the normal range 18. Aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT) > 1.5 times the upper limit of normal ranges 19. Known hypersensitivity or history of drug-related adverse events with bosentan (e.g. increase in liver function test results), or any of the excipients of its formulation 20. Receipt of an investigational product other than sildenafil within 3 months before start of study treatment 21. Treatment with endothelin receptor antagonists (ERAs), prostanoids or phosphodiesterase (PDE) 5 inhibitors other than sildenafil within 3 months prior to randomization 22. Concomitant systemic treatment within 1 week prior to randomization with calcineurin inhibitors (e.g., cyclosporine A and tacrolimus), sirolimus and everolimus glibenclamide (glyburide) both cytochrome P2C9 (CYP2C9) and cytochrome P3A4 (CYP3A4) (e.g., fluconazole, amiodarone, voriconazole) combination of drugs that inhibit CYP2C9 and CYP3A4 23. Treatment with nitrates and alpha-blockers at time of randomization 24. In the opinion of the investigator patients in need for treatment with any prostanoid up to Visit 4 25. Significant left ventricular dysfunction | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Congestive Heart Failure Age >18 years Ejection fraction <40% Heart failure limited by fatigue and shortness of breath Pulmonary artery hypertension Patients taking the following medications: nitroglycerine pill/patch/paste, isordil, Imdur, antifungal agents and certain antidepressants. Patients with a history of optic neuropathy or unexplained visual impairment. Patients with anemia. - | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Hypercholesterolemia Patients are eligible for study entry based on the following 1. Males or females greater than or equal to 18 years of age 2. Females must not be pregnant or lactating. Females of childbearing potential and males must use a reliable means of contraception. 3. LDL-C level greater than the NCEP goals, as determined by patients' risk category according to NCEP ATP III 4. Risk category for coronary heart disease and coronary heart disease equivalent with LDL goal of < 100 mg/dL 5. Baseline lipid LDL-C = 100 to160 mg/dL and triglyceride level = 100 to 500 mg/dL 6. Normal thyroid function tests (total T3, total T4, and thyroid-stimulating hormone [TSH]) 7. Hemoglobin A1C < 8.5% on a stable oral hypoglycemic or insulin regimen 8. On stable lipid modification pharmacotherapy (including a statin) for at least 2 weeks prior to study entry. Patients must be on at least half of the maximal doses of statins (as assessed by the Investigator), or be intolerant to statins such that the doses are not achievable. 9. Able to give informed consent Pre-Randomization Patients will not be eligible for the study based on the following 1. History of thyroid disorders of any form within 24 weeks prior to study entry 2. Active liver disease and/or liver transaminases greater than 1.5 X upper limit of normal 3. Active myocarditis, hypertrophic cardiomyopathy, uncorrected primary valvular disease, restrictive cardiomyopathy, uncorrected congenital heart disease, or constrictive pericarditis 4. Myocardial infarction, unstable ischemic heart disease, stroke, or coronary revascularization procedure within 24 weeks prior to study entry 5. Moderate or severe symptomatic congestive heart failure (New York Heart Association class III and IV) 6. Drug or alcohol dependence, or other conditions which may affect study compliance 7. Renal insufficiency (serum creatinine > 2 mg/dL) 8. Subjects taking other hormonal therapies (other than oral contraceptive agents and postmenopausal hormone replacement therapy) e.g., glucocorticoids, androgens, or growth hormones 9. Use of thyroid supplements (levothyroxine, liothyronine, etc.) or any preparation containing thyromimetic agents within 24 weeks prior to study entry 10. History of coagulopathy or use of anticoagulants such as warfarin 11. Unstable endocrine/metabolic syndrome that may affect lipid metabolism 12. History of atrial or ventricular arrhythmia 13. Diagnosis of other non-cardiac underlying medical conditions expected to impact mortality within 24 weeks after randomization | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Pulmonary Embolism Patients >= 18 years of age with pulmonary embolism and cardiogenic shock, defined as a systolic arterial pressure <= 90 mmHg, a drop in systolic arterial pressure >= 40 mmHg for >= 15 minutes, or ongoing administration of catecholamines for systemic arterial hypotension 2. Subtotal or total filling defect in the left and/or right main pulmonary artery due to massive PE, as assessed by chest computed tomography or by conventional pulmonary angiography 3. Right ventricular dysfunction on echocardiography: RV systolic hypokinesis and/or RV dilation (optional) 4. Failed thrombolysis or at least one of the following contraindications to PE thrombolysis present Active bleeding History of intracranial bleeding Surgery, delivery, organ biopsy, puncture of a non-compressible vessel within 10 days History of stroke Gastrointestinal bleeding within 10 days Significant trauma within 15 days Head injury requiring hospitalization within 1 year Active cancer with known hemorrhagic risk Neurosurgery or ophthalmologic surgery within the past year Systemic (paradoxical) embolism in the presence of an atrial septal defect or patent foramen ovale 2. Free floating right heart thrombi, left heart thrombi 3. Life expectancy due to underlying disease less than one month | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Chronic Obstructive Pulmonary Disease Admission for chronic obstructive pulmonary disease Other severe acute or chronic diseases | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-70.0, Chagas Cardiomyopathy Chronic Heart Failure Males or females aged 18 to 70 years Heart failure symptoms NYHA functional class II to IV Left ventricular ejection fraction <40% determined by bi-dimensional echocardiography using modified Simpson's rule for ventricular volumes Subjects must be on standard and stable outpatient doses of ACEIs or angiotensin II receptor antagonist for at least four weeks Subjects receiving diuretics must be on a stable dose for at least two weeks Clinical Euvolemia:as evidenced by absence of rales, no pleural effusion or ascitis and no more than minimal peripheric edema CHF due to ischemic heart disease, valvular disease or any other etiology different than CD Severe aortic insufficiency Baseline advanced AV block defined as Mobitz type 2 or third degree AV block Serum creatinine >2.5 mg/dl Resting Heart rate less < 45 bpm Known malignancy and other severe disease which shorten life expectancy < 6 months Subjects with contraindications for beta-blockers: severe obstructive chronic pulmonary disease, asthma, severe pulmonary hypertension, type 1 diabetes mellitus or history of hypoglicemia Suspected or confirmed chronic infectious disease including HIV and hepatitis B History of active substance or alcohol abuse within the last year Clinically significant psychiatric illness which can negatively affect the subject compliance and participation in the trial | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, HIV All volunteer subjects must be at least 18 years of age and must be able to provide informed, written consent for participation in this study. in the study is determined prior to enrollment on the basis of the following and criteria. For Healthy Volunteers: 1. Males or females, 18 years of age or older. 2. No history of HIV infection. 3. EKG and echocardiogram with no evidence of clinical relevant heart disease (including TRV less than 2.5 m/s) 4. No history of causes of pulmonary hypertension such as collagen vascular disease, chronic liver disease with ALT or AST greater than 2 times the upper limit of normal or cirrhosis of the liver, chronic thromboembolic disease, congenital heart defects, or pulmonary parenchymal disease with hypoxemia. 5. No history of diseases thought to be related to development of endothelial dysfunction including systemic hypertension or diabetes requiring drug therapy, hypercholesterolemia and obesity. 6. No history of anemia, thrombocytopenia or coagulopathy. 7. No history of renal insufficiency. 8. No medical conditions requiring chronic medication use with the exception of: 1. Heartburn, GERD 2. Environmental allegeries, post nasal drip or non-allergic rhinitis 3. Asthma with no history of oral steroid use, weekly inhaled steroids, or hospitalization for asthma exacerbation. 4. Dermatologic conditions that do not require the use of oral steroids or other immunosuppressants. 9. No more than 20 cigarettes per year for the previous 2 years and no cigarette use for 30 days prior to the screening evaluation until completion of the study. For HIV Positive Volunteers: 1. Males or females, 18 years of age or older. 2. Diagnosis of HIV infection. 3. Under the care of a primary care physician. 4. No history of other potential causes of pulmonary hypertension such as collagen vascular disease, chronic liver disease with ALT or AST greater than 2 times the upper limit of normal or cirrhosis of the liver, chronic thromboembolic disease, congenital heart defects, or pulmonary parenchymal disease with hypoxemia. 5. For stage 3 enrollment: symptomatic pulmonary hypertension with MPAP greater than or equal to 25 mmHg and six-minute walk distance between 150-450 m For Healthy volunteers: 1. Current pregnancy, lactation or women not currently using medically acceptable birth control. (All women of childbearing age will be required to have a screening urine or blood pregnancy test) 2. Contraindication to MRI scanning including individuals with the following devices: A) Central nervous system aneurysm clips B) Implanted neural stimulator C) Implanted cardiac pacemaker or defibrillator prior to enrollment D) Cochlear implant E) Ocular foreign body (e.g. metal shavings) F) Insulin pump G) Metal shrapnel or bullet 3. Contraindications to MRI contrast agent administration: 4. Inability to provide informed written consent for participation in the study. 5. Chronic, medically refractory atrial tachyarrhythmias 6. Symptoms of heart failure 7. Mean PA greater than 25 mmHg or PVR greater than 1.5 wood units, or PCWP greater than 15 mmHg 8. History of recreational drug use with the exception of marijuana. No Marijuana use within 3 months of protocol screening through completion of the study. Volunteers may be excluded if in the opinion of the study investigators they have a condition that may adversely affect the outcome of the study or the safety of the volunteer. For HIV Positive Volunteers: 1. Current pregnancy, lactation or women not currently using medically acceptable birth control. 2. Active, untreated opportunistic infection with the exception of oral thrush or neoplasm with the exception of Kaposi's Sarcoma. 3. Any of the following medical conditions: A) Severe renal insufficiency (on hemodialysis or serum creatinine greater than 2.5 mg/dl) or (CrCl less than 20 ml/min). B) Severe coagulopathy (platelets less than 100,000, PT INR greater than 1.5, PTT greater than 40 s) 4. Chronic, medically refractory atrial tachyarrhythmias 5. Current recreational drug use or use within 4 weeks of protocol screening with the exception of marijuana 6. Contraindication to MRI scanning including individuals with the following devices: A) Central nervous system aneurysm clips B) Implanted neural stimulator C) Implanted cardiac pacemaker or defibrillator prior to enrollment D) Cochlear implant E) Ocular foreign body (e.g. metal shavings) F) Insulin pump G) Metal shrapnel or bullet 7. Contraindications to MRI contrast agent administration: A) Individuals with hemoglobinopathies Volunteers may be excluded if in the opinion of the study investigators they have a condition that may adversely affect the outcome of the study or the safety of the volunteer | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 66.0-999.0, Heart Failure, Congestive Coronary Arteriosclerosis Atrial Fibrillation Cerebrovascular Accident Pulmonary Disease, Chronic Obstructive Diabetes Mellitus Hypertension Osteoarthritis Osteoporosis > 65 years old Confirmed or probable dual eligible Have at least one chronic illness (chronic obstructive pulmonary disease [COPD], diabetes, stroke/atrial fibrillation, ischemic heart disease, hypertension, congestive heart failure [CHF], osteoporosis, osteoarthritis) and at least 1 impaired activity of daily living (ADL) 11 or 2 impaired instrumental activities of daily living (IADLs) Be discharged home or to a skilled nursing facility (or acute rehabilitation) for a maximum of 8 weeks before being discharged to home Enrolled in this health system's care management program Chemically dependent Those with a Mental Status Questionnaire score > 5 Diagnosed psychosis Dialysis Terminal diagnosis/hospice | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 25.0-79.0, Pulmonary Hypertension Chronic Obstructive Pulmonary Disease 25 to 79 years old. 2. Provide voluntary written informed consent to participate. 3. Smoking history of at least 10 pack years (1 pack of cigarettes per day for 10 years). 4. Diagnosis of chronic obstructive pulmonary disease. 5. Become short of breath with physical activity. 6. Elevated systolic pulmonary artery pressures. 7. Not pregnant, nursing, or planning a pregnancy Left ventricular ejection fraction <30%. 2. Heart attack or stroke within the last 6 months. 3. History of lung resection surgery. 4. Use of supplemental oxygen >20 hours/day. 5. Blood donation or significant blood loss within the last 56 days. 6. Plasma donation within the last 14 days. 7. Use of any drugs for another research study within the last 30 days. 8. Use of vasodilators, long acting nitrates, prostacyclin analogs, endothelin antagonists, or phosphodiesterase inhibitor drugs within the last 14 days. 9. Positive blood screen Hepatitis B surface antigen (HBsAg) or Hepatitis C Antibody. 10. Major surgery within the last 28 days. 11. Any other medical condition that may jeopardize the safety of the subject, the validity of the study results, or interfere with the completion of the study according to the protocol | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Pulmonary Hypertension Males or females, 18 years of age or older Diagnosis of sickle cell disease (electrophoretic documentation of SS, SC, or S thalassemia genotype is required) or thalassemia For female subjects, on a reliable method of birth control or not physically able to bear children. <TAB> For Stage II Mild to severe pulmonary hypertension with mean pulmonary artery pressure greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise, measured by pulmonary artery catheterization Pulmonary artery wedge pressure or left ventricular end-diastolic pressure less than or equal to 18 mm Hg or gradient between pulmonary artery diastolic pressure and wedge pressure greater than 8 mm Hg or echocardiographic to left ventricular dysfunction For female subjects, on a reliable method of birth control or not physically able to bear children. For Stage III Mild to severe pulmonary hypertension with mean pulmonary artery pressure greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise, measured by pulmonary artery catheterization in patients on chronic sildenafil therapy for at least three months. Patients receiving combination drug therapy with other drugs (e.g., endothelin antagonists, prostacyclins) are allowed to participate in stage III as long as sildenafil is one of the components of the combination regimen Pulmonary artery wedge pressure or left ventricular end-diastolic pressure less than or equal to 18 mm Hg or gradient between pulmonary artery diastolic pressure and wedge pressure greater than 8 mm Hg or echocardiographic to left ventricular dysfunction Must be on chronic sildenafil therapy on a stable dose for at least three months For female subjects, on a reliable method of birth control or not physically able to bear children For Stage I Current pregnancy or lactation Any condition that in the opinion of the study investigators would adversely affect the outcome of the study or the safety of the volunteer. For Stage II and III Current pregnancy or lactation Any of the following medical conditions 1. Stroke within the last six weeks. 2. History of sustained priapism requiring medical or surgical treatment, unless currently impotent or on exchange transfusion therapy, within two years | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Pulmonary Hypertension Summarized 1. 18 years of age or older 2. Current diagnosis of PH associated with an acceptable etiology as outlined in the protocol, including: PH due to the following etiologies: 1) PAH including idiopathic and familial PAH and PAH associated with collagen vascular disease, congenital systemic-to-pulmonary shunts (including Eisenmenger's syndrome), human immunodeficiency virus (HIV) infection, drugs and toxins, thyroid disorders, glycogen storage disease, Gaucher disease, hemoglobinopathies, and splenectomy (WHO Group 1); 2) PH associated with lung diseases and/or hypoxemia, including chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD), sleep-disordered breathing, and alveolar hypoventilation disorders (WHO Group 3); 3) PH due to proximal or distal chronic thromboembolic obstruction (WHO Group 4); and 4) PH due to sarcoidosis (WHO Group 5). 3. Stable regimen (within four weeks) of chronic prostanoid, PDE-5 inhibitor, calcium channel blocker, or 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitor therapy 4. Right heart catheterization completed prior to screening must meet pre-specified 5. Female participants of childbearing potential must have a negative serum pregnancy test and must agree to use a reliable double method of contraception until study completion and for at least four weeks following their final study visit. 6. Male participants must be informed of the potential risks of testicular tubular atrophy and infertility associated with taking ambrisentan and queried regarding his understanding of the potential risks as described in the Informed Consent Form. Summarized Participation in a previous clinical study with ambrisentan 2. Bosentan or sitaxsentan use within four weeks prior to the screening visit 3. Aspartate aminotransferase (AST) or alanine aminotransferase (ALT) lab value that is greater than 3 times the upper limit of normal at the screening visit 4. Pulmonary function tests not meeting the following pre-specified 1) mean pulmonary arterial pressure (PAP) >= 25 mm Hg; 2) PVR > 3 mm Hg/L/min; 3) pulmonary capillary wedge pressure (PCWP) or left ventricle end diastolic pressure (LVEDP) < 15 mm Hg; 4) total lung capacity (TLC) >= 70% of predicted normal for participants without ILD or >= 60% of predicted normal in participants with ILD; forced expiratory volume in 1 second (FEV1) >= 65% of predicted normal in participants without COPD or >= 50% of predicted normal in participants with COPD 5. Contraindication to treatment with endothelin receptor antagonist (ERA) 6. History of malignancies other than basal cell carcinoma of the skin or in situ carcinoma of the cervix within the past five years 7. Female participant who is pregnant or breastfeeding | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Chronic Obstructive Pulmonary Disease Exertional dyspnea Activity limitation COPD Recent exacerbation (< 4 wks) Supplemental O2 Significant cardiovascular/musculoskeletal abnormalities | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 21.0-85.0, Sleep Critical Illness Patients requiring mechanical ventilation with a diagnosis of Acute Respiratory Distress Syndrome Cardiogenic Pulmonary Edema Pneumonia Chronic obstructive pulmonary disease Patients who are considered too unstable to undergo this investigation by their primary physician Comatose patients, or patients with severe debilitating neurological disease Renal insufficiency (Sr. Creatinine > 2 mg/dL) Heart block (second or third degree heart block) or sinus bradycardia (heart rate < 60 beats per minute) Pregnancy | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 30.0-80.0, Heart Failure were: consent to participate in the study after detailed information about procedures, possible clinical benefits and risks; ability to complete a maximal exercise test; forced expiratory volume in 1 sec/forced vital capacity ratio>70%; left ventricular ejection fraction 45%, determined by echocardiography Patients were not recruited if they had systolic blood pressure > 140 and <110 mmHg, diabetes mellitus, therapy with nitrate preparations, history of sildenafil intolerance, significant lung or valvular diseases, neuromuscular disorders, exercise-induced myocardial ischemia, atrial fibrillation (6), claudication, peripheral vascular disease | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure Dyspnea Age 18 years or older Acute dyspnea as their most prominent complaint Acute dyspnea due to a trauma Acute dyspnea due to cardiogenic shock Renal failure requiring dialysis | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 12.0-999.0, Pulmonary Arterial Hypertension Signed informed consent prior to initiation of any study-mandated procedures. 2. Males or females ≥ 12 years of age (females of child-bearing potential must have been surgically sterilized or use a reliable method of contraception). 3. Symptomatic patients with the following types of PAH belonging to World Health Organization (WHO) Pulmonary Hypertension Classification Group I Idiopathic (IPAH) Familial (FPAH) Associated with PAH (APAH) Collagen vascular disease Drugs and toxins 4. Patients naïve to treatment with advanced PAH therapies (i.e., endothelin receptor antagonists (ERAs), phosphodiesterase-5 (PDE-5) inhibitors or prostacyclins) with a right heart catheterization (RHC) showing all of the following Mean pulmonary arterial pressure (mPAP) ≥ 25 mm Hg Pulmonary capillary wedge pressure (PCWP) ≤ 15 mm Hg or left ventricular end diastolic pressure (LVEDP) ≤ 15 mm Hg when PCWP is not accurately obtained Pulmonary vascular resistance ≥ 3 Wood units 5. 6-MWT distance ≥ 150 meters and < 360 meters Patients with Pulmonary Hypertension (PH) belonging to WHO Classification Group II-V. 2. Patients with PAH (WHO PH Classification Group I) other than that listed in the Criteria. 3. Pregnant and/or nursing. 4. Women of childbearing potential not using a reliable method of contraception. 5. Patients with known human immunodeficiency virus (HIV) infection. 6. Patients with significant vasoreactivity during right heart catheterization (i.e., a fall in mPAP to < 40 mm Hg with a decrease of ≥ 10 mm Hg and with a normal cardiac index ≥ 2.5 l/min.m^2). 7. Patients with restrictive lung disease (i.e., total lung capacity (TLC) < 60% of normal predicted value). 8. Patients with obstructive lung disease (i.e., forced expiratory volume/ forced vital capacity (FEV1/FVC) < 0.5). 9. Patients with impaired left ventricular function (LVEF <50%) or diastolic dysfunction. 10. Patients with portal hypertension, cirrhosis, moderate to severe liver impairment (Child-Pugh Class B or C), or liver enzymes (Aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT)) > 3.0 times the upper limit of normal range. 11. Treatment with glibenclamide (glyburide) and calcineurin inhibitors (cyclosporine A, tacrolimus) sirolimus, everolimus up to 1 week prior to Baseline (Day 1). 12. Patients currently receiving or predicted to require treatment, during the course of the study, with nitrates, protease inhibitors, or alpha-blockers. 13. Patients with a hemoglobin concentration < 75 % of the lower limit of the normal range or < 8.5 g/dL. 14. Patients currently receiving or predicted to require treatment with a prostanoid during the course of the study. 15. Patients with systolic blood pressure < 85 mm Hg. 16. Patients with body weight < 40 kg. 17. Patients who have received any investigational product within 90 days prior to Baseline. 18. Patients who previously received any advanced therapy for PAH (e.g., ERAs, PDE-5 inhibitors or prostacyclins). 19. Patients with hypersensitivity to sildenafil or any excipients of its formulation. 20. Patients with any contraindication to sildenafil treatment (i.e., nitrates). 21. Patients with any recent medical condition limiting the ability to comply with the study requirements (i.e., stroke, myocardial infarction). 22. Patients with unstable PAH whose disease state would prohibit the completion of study procedures, in the opinion of the investigator. 23. Patients unable to complete a MRI scan (e.g., claustrophobia). 24. Patients with permanent cardiac pacemakers, automatic internal cardioverter defibrillators (AICD's), neurostimulators, hearing aides, and other implanted metallic devices that are contraindicated during a MRI study. 25. Patients with conditions that would interfere with proper cardiac gating during MRI, such as atrial fibrillation or multiple premature ventricular contractions (PVCs)/premature atrial contractions (PACs). 26. Patients with conditions that prevent compliance with the protocol or the ability to adhere to therapy | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Chronic Obstructive Pulmonary Disease moderate to severe COPD (according to GOLD classification) oxygen saturation >= 92 KPa symptomatically stable habitually low fruit and vegetable intakes (<=2 portions daily) exercise limited by shortness of breath (rather than e.g. angina, arthritis) diabetes taking antioxidant supplements or drugs oxygen saturation <8KPa | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Congestive Heart Failure Adult patient > 18 years of age Have a working diagnosis of HF, as determined by the emergency physician using the Boston Criteria. A score of 8-12 is required for in the study Alert, oriented and able to provide informed consent Able to be contacted by telephone for follow up after discharge, and have none of the study Dialysis Dependent Renal Failure Temperature > 38.5 degrees celsius Pneumonia (Infiltrates on Chest X-ray) Requiring IV vasoactive agents (Other than Nesiritide) Killip Class III/IV Systolic blood pressure < 90 mmHg EKG diagnostic or suggestive of Acute myocardial infarction or ischemia Abnormal Cardiac Markers Lack of a telephone Inability to provide informed consent due to cognitive impairment or a severe psychiatric disorder | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 16.0-999.0, Pulmonary Hypertension Subjects aged 16 and over, and classified as having pulmonary arterial hypertension Subjects who meet the following conditions on right heart catheterization at screening or baseline: mean pulmonary arterial pressure of ≥ 25mmHg and pulmonary capillary wedge pressure of ≤ 15mmHg at rest Subjects whose baseline 6-Minute Walk test distance is >100 m and <450 m Significant Hepatic and/or renal disorder Subjects with known hereditary degenerative retinal disorders (such as retinitis pigmentosa) or history of non-arteritic ischemic optic neuropathy (NAION) Subjects who are currently receiving nitrates or nitric oxide donors in any form, ritonavir, ketoconazole and itraconazole | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Diseases Hypertension, Pulmonary Patients ≥ 18 years of age Male or female patients (females of child-bearing potential must have been surgically sterilized or use a reliable method of contraception) Patients undergoing complex* cardiac surgery on CPB and having systolic PAP > 40 mmHg or mean PAP > 30 mmHg (*surgery on 2 valves, 1 valve and revascularization, or reoperation of a valve) Patients undergoing cardiac surgery on CPB and having pre-operative pulmonary hypertension due to left heart disease with systolic PAP > 60 mmHg Signed written informed consent Systolic blood pressure < 100 mmHg Significant chronic lung disease Emergency surgery Pregnant/breast-feeding Investigational drug use within 28 days prior to randomization Complex adult congenital heart disease Severe concomitant illness limiting life expectancy to < 6 months Participation in a device study that will affect the outcome of the study Pre-operative use of balloon pump, inotropes/vasopressors, treatment of pulmonary arterial hypertension Known hypersensitivity to tezosentan or drugs of the same class, or any of their excipients | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-75.0, Acromegaly Growth Hormone Deficiency for acromegaly Clinical and biochemical diagnosis of acromegaly. Thyroid and glucocorticoid replacement if necessary stable for at least 4 weeks before the study. Gonadotrophin status will be recorded and whenever possible patients will be studied in the same status Males and females aged 18-70 years willing to give informed consent At least 6 months after the onset of symptoms of acromegaly and on stable medication for heart failure treatment (if any) for at least 4 weeks prior to into the study Systolic blood pressure < 180 mmHg, diastolic blood pressure < 110 mmHg. for GHD Clinical and biochemical diagnosis of GHD. All hormones replaced (if clinically necessary) except GH. Thyroid and glucocorticoid replacement if necessary stable for at least 4 weeks before the study. Gonadotrophin status will be recorded and whenever possible patients will be studied in the same status Males and females aged 18-70 years willing to give informed consent At least 6 months after the onset of symptoms of acromegaly and on stable medication for heart failure treatment (if any) for at least 4 weeks prior to into the study Systolic blood pressure < 180 mmHg, diastolic blood pressure < 110 mmHg for acromegaly Change in medication in the preceding 4 weeks Patients on subcutaneous insulin therapy Hyperthyroidism Not being in sinus rhythm Unstable angina pectoris and decompensated heart failure (define as NYHA 3-4) Clinically significant valvular disease, clinically significant chronic obstructive pulmonary disease History of myocardial infarction or stroke within the last 6 months, major cardiac surgery within the last 6 months Significant history of drug or alcohol abuse or unable to give informed consent | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-85.0, Pulmonary Hypertension Male or female, aged 18-85 years Have a current diagnosis of symptomatic pulmonary arterial hypertension (PAH) classified by one of the following: a) idiopathic pulmonary arterial hypertension (IPAH) or familial pulmonary arterial hypertension (FPAH); b) PAH associated with one of the following connective tissue diseases and mild or no lung parenchymal disease: scleroderma spectrum of disease, systemic lupus erythematosis, or mixed connective tissue disease, c) PAH associated with repaired atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosis (PDA) ≥ 1 year post-operative from Screening, d) PAH associated with human immunodeficiency virus (HIV), or e) PAH associated with the use of anorexigens (e.g. fenfluramine-phentermine) On a stable and well tolerated dose regimen of Ventavis (5 μg per dose) for at least 4 weeks prior to the Screening visit, using the I-neb AAD System equipped with Power Disc-6 Receipt of any prostacyclin or prostacyclin analogue other than Ventavis within the 12 weeks preceding the Screening visit Receipt of atrial septostomy within the 6 months preceding Screening History of left-sided heart disease Clinically relevant obstructive lung disease Chronic renal or liver disease Uncontrolled systemic hypertension or hypotension Cerebrovascular event within the 6 months preceding Screening | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Chronic Heart Failure chronic heart failure left ventricular systolic dysfunction (ejection fraction <45%) stable clinical conditions in the previous three months optimal medical therapy for CHF normal sinus rhythm written informed consent age >18 years renal failure (s-creatinine >2.5 mg/dl) already on allopurinol malignancies chronic inflammatory diseases | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-0.022, Congenital Disorders case report of a patient with trisomy 21 and primum atrial septum defect with associated cleft mitral valve who was diagnosed with pulmonary hypertension at 8 days of life those who do not fit into | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Coronary Artery Disease Left Ventricular Dysfunction Sudden Cardiac Death Randomized Arm 1. Evidence of Coronary Artery Disease (CAD)a. 2. Evidence of prior Myocardial Infarction defined by either: A. Clinical history of prior myocardial infarction OR B. Mild-moderate systolic LV dysfunction with an EF ≤50% 3. LVEF>35% by any current standard evaluation technique (e.g., echocardiogram, MUGA, angiography). • Patients who have an EF between 30-35% and NYHA Class I heart failure who do not have a history of ventricular tachyarrhythmias, or inducible ventricular tachycardia during electrophysiological (EP) testing can be enrolled (Target Population). 4. CE-MRI measure of infarct mass > 10% of LV mass (as measured by the MRI core lab) • If CE-MRI performed ≤ 40 days after myocardial infarction infarct mass must be ≥ 15% of the LV mass. 5. Patients aged 18 years or above 1. CAD will be confirmed by evidence of one of the following three (3) 1) Prior myocardial infarction, 2) Significant stenosis of a major epicardial vessel (>50% proximal or 70% distal) by coronary angiography, 3) Prior revascularization (percutaneous coronary intervention or coronary artery bypass surgery. Patients may not be randomized until 90 days after revascularization. 2. MI should be documented by the presence of two (2) of the following three (3) 1) Symptoms consistent with myocardial infarction (i.e. chest pain, shortness of breath), 2) Q-waves on electrocardiogram and 3) Elevated cardiac enzymes (CPK elevation > two times or troponin elevation > three times the upper limit of normal for the lab). Patients may not be randomized until 40 days after myocardial infarction History of cardiac arrest or spontaneous or inducible sustained VT (15 beats or more at a rate of 120 BPM or greater)* 2. Unexplained syncope 3. Need for revascularization based on investigator's clinical assessment within the next 12 months (patients may be reevaluated 90 days after revascularization) 4. Currently implanted permanent pacemaker and/or pacemaker/ICD lead 5. Contraindication to a ICD implant (i.e. inadequate venous access, bleeding disorder) 6. Acute or chronic severe renal insufficiency (< 30mL/min/1.73m2); acute renal insufficiency of any severity due to hepato-renal syndrome 7. Current or planned renal or liver transplant 8. End stage renal disease on hemodialysis or peritoneal dialysis 9. Contraindication to CE-MRI or history of allergy to gadolinium-based contrast dye 10. Metal fragments in the eyes or face, implantation of any electronic devices such as (but not limited to) cardiac pacemakers, cardiac defibrillators, cochlear implants or nerve stimulators, surgery on the blood vessels of the brain, body piercing 11. Recent MI (<40 days) or revascularization (<90 days) 12. CVA within 90 days 13. Antiarrhythmic drug therapy for ventricular arrhythmias 14. New York Heart Association CHF functional class IV at enrollment Non-Investigational Registry Evidence of CAD a with either a history of prior myocardial infarction OR any LV dysfunction Evidence of LV dysfunction (ejection fraction) as measured by any current standard screening technique (e.g., echocardiogram, MUGA, angiography).c Clinical CE-MRI within the past 12 months (scheduled or completed) Patients aged 18 years or above CAD will be confirmed by evidence of one of the following three (3) 1) Prior myocardial infarction, 2) Significant stenosis of a major epicardial vessel (>50% proximal or 70% distal) by coronary angiography, 3) Prior revascularization (percutaneous coronary intervention or coronary artery bypass surgery MI should be documented by the presence of two (2) of the following three (3) 1) Symptoms consistent with myocardial infarction (i.e. chest pain, shortness of breath), 2) Q-waves on electrocardiogram and 3) Elevated cardiac enzymes (CPK elevation > two times or troponin elevation > three times the upper limit of normal for the lab) Patients can be enrolled in the registry even if they have received or are about to receive an ICD for primary prevention History of cardiac arrest or spontaneous or inducible sustained VT (15 beats or more at a rate of 120BPM or greater)* Contraindication to CE-MRI or history of allergy to gadolinium-based contrast | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 12.0-70.0, Sickle Cell Disease Pulmonary Hypertension • based on the following and Screening Phase Males or females, greater than or equal to 12 years of age and less than or equal to 70 years of age Diagnosis of sickle cell disease (including, but not limited to SS, SC, SD, or S-beta zero thalassemia) Provision of informed consent and, where applicable, assent. Observational Follow-up Study Satisfaction of screening criteria In the opinion of the investigator, ability to maintain follow-up contact Failure to satisfy the requirements of the Main Interventional Trial (MIT) OR discontinuation/completion of the MIT/Open-label Follow-up Phase Provision of informed consent and, where applicable, assent. Main Interventional Trial Males or females, 12 years of age or older and less than or equal to 70 years of age Female subjects, on a reliable method of birth control or not physically able to bear children | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure, Congestive Sleep Apnea Syndromes Moderate to severe heart failure (as determined via Echocardiogram or treating physician) Clinically significant asthma requiring therapy Significant (parenchymal) lung disease Severe pulmonary hypertension Hypercapnia (PCO2 > 60mmHg) Cardiogenic Shock Severe chronic renal failure Narcotic abuse/intravenous drug use (including heart failure due to alcohol abuse or chemotherapy, HIV+ and Hepatitis C) Methicillin resistant Staphylococcus aureus infection Tracheotomy | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Atrial Septal Defect Suspected Atrial Septal Defect (ASD) undergoing evaluation for possible transcatheter closure Contraindications to magnetic resonance imaging known sinus venosus or primum defects | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 50.0-80.0, Chronic Obstructive Pulmonary Disease Diagnosis of COPD according GOLD Stable clinical conditions for the previous two months Severe comorbidities other than COPD Hypoxemia with partial arterial oxygen pressure lower than 50 mmHg Incapacity to come to the research center in a weekly basis | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Lung Cancer Precancerous Condition Meets 1 of the following Current smoker, defined as ≥ a 20 pack year smoking history and/or 20 year duration of smoking Ex-smoker who has quit smoking within the past 8 years AND has ≥ a 20 pack year smoking history and/or 20 year duration of smoking Mild to moderate chronic obstructive pulmonary disease (COPD) as defined by the GOLD Mild COPD: FEV_1/forced vital capacity (FVC) < 70%; FEV_1 ≥ 80% of predicted* Moderate COPD: FEV_1/FVC < 70%; FEV_1 50-80% of predicted* NOTE: *Spirometric values will be obtained post bronchodilator according to the recommendations in the GOLD Inadequate lung function (FEV_1 < 50% of predicted after bronchodilator) Life expectancy must be at least 5 years History of malignant disease within the past 5 years except non-melanomatous skin cancers Other serious co-morbidity Evidence of severe or uncontrolled systemic diseases that, in the view of the investigator, makes it undesirable for the patient to participate in this trial Any disorder making reliable informed consent impossible Unlikely to co-operate with a 5 year follow-up PRIOR Patients may receive all concurrent therapy deemed to provide adequate care as decided by their medical doctors | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Pulmonary Fibrosis Hypertension, Pulmonary Clinical diagnosis of IPF Diffusing capacity of the lung (DLCO) level less than 35% (adjusted for hemoglobin) Current enrollment in another investigational study Six-minute walk distance of less than 50 meters at screening or study entry Difference of greater than 15% between the screening and study entry 6-minute walk distance Acute or long-term impairment other than dyspnea (e.g., angina pectoris, intermittent claudication) that limits the ability to comply with the 6-minute walk test or other study requirements Forced Expiratory Volume 1-second (FEV1)/forced vital capacity (FVC) ratio of less than 0.65 after bronchodilator use Extent of emphysema greater than the extent of fibrotic change (e.g., honeycombing, reticular changes) on high-resolution computed tomography (HRCT) scan Acute heart attack within the 6 months prior to study entry Nitrate use Hypersensitivity to sildenafil or any component of the formulation Presence of aortic stenosis (AS) | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Coronary Artery Disease Obstructive Sleep Apnea Patients with angiographically-verified CAD who have newly undergone PCI or CABG treatment Written, informed study consent OSA (AHI>=15 per hour) or non-OSA (AHI<5 per hour) diagnosis on the unattended sleep recording at home Patients with already treated OSAS Patients presenting mainly central apneas (Cheynes-Stokes breathing) Patients with borderline OSA (AHI <15 and >=5 per hour) upon the unattended sleep recording at home | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 45.0-999.0, Hypertension Diastolic Dysfunction Age 45 years or older Male and female patients are eligible. Female patients must be post-menopausal for one year, surgically sterile, or using effective contraceptive methods such as a double barrier method with spermicide, an intra-uterine device, or hormonal contraceptives. Post-menopausal women on a stable dose of hormone replacement therapy (HRT) for at least three (3) months prior to the screening visit are eligible for the study Uncontrolled systolic hypertension on a maximum of two (2) antihypertensive medications at the time of screening Echocardiographic ejection fraction ≥50% and evidence of diastolic dysfunction Provide written informed consent to participate in the study prior to any screening or study procedures Have the ability to communicate well and comply with all study requirements Severe hypertension defined as a MSSBP >200 mmHg and/or MSDBP >120 mmHg History of a secondary cause of hypertension including but not limited to: coarctation of the aorta, hyperaldosteronism, unilateral or bilateral renal artery stenosis, Cushing's disease, pheochromocytoma, polycystic kidney disease, etc Ejection fraction <50 % History of stroke, transient ischemic attack, myocardial infarction, coronary artery bypass graft surgery, or unstable angina pectoris within 6 months of screening Presence of clinically significant ventricular or supraventricular arrhythmias (e.g. atrial fibrillation/flutter) History of congestive heart failure History of diabetes mellitus History of renal impairment with serum creatinine >2.0 mg/dL at screening, history of dialysis, or history of nephritic syndrome Antihypertensive therapy with three (3) or more medications at the time of screening Active and/or treated malignancy of any organ system within twelve (12) months of enrollment, with the exception of localized basal cell carcinoma of the skin | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Congestive Heart Failure age18 or older primary diagnosis of CHF in the Emergency Department admission to the hospital or transfer to the Observation Unit minors, prisoners,pregnant women,unable to provide consent unstable angina or acute myocardial infarction in the ED dialysis dependent patients use of nesiritide as a therapy in the ED | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 45.0-70.0, COPD Age 45-70 yrs of age, incl, women as described above as far as child-bearing potential and subjects who if they have coexisting disease, the disease is stable and does not place an untoward risk or result in compromise of data Non smokers will be lifelong and have not worked in a chronic dusty environment Smokers will require a minimum of 10 pkyr history and currently smoking at least a pack per day. The smokers without symptoms of COPD must demonstrate a pulmonary function with an FEV1 > 80%. The FEV1/FVC ratio may be less than .7 however they can not demonstrate symptoms of chronic bronchitis by medical history. Smokers with symptoms must demonstrate an FEV1 < 80% and FEV1/FVC <.7 Any female subject who is pregnant or breast-feeding or a female subject who is of child-bearing potential who is unwilling to sue two acceptable methods of birth control Any condition that would place the subject at risk for endobronchial brushings(such as bleeding diathesis) or subjects who are currently taking anticoagulants Any subject who has had an exacerbation of COPD within the last six months of screening or who has been hospitalized for any reason within the last three months Illicit substances indicated by positive urine drug screen | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-75.0, Chronic Obstructive Pulmonary Disease Hypertension, Pulmonary Age ≥ 18 years old and ≤ 75 years old (*) Chronic Obstructive Pulmonary Disease with FEVs/VC < 70% (**) Respiratory pulmonary hypertension with mean pulmonary arterial pressure ≥ 25 mmHg (**) related to normal pulmonary capillary pressure assessed by catheterization of the right side of the heart (pulmonary capillary wedge pressure ≤12mmHg) PO2 ≤ 60 mmHg assessed by arterial gazometry at ease (**) or PO2 > 60 mmHg, but related with high hypoxemia after exercise (six-minute walk test ) Oxygenotherapy more than 6 months before pre-screening Written informed consent (*) of young adults concerns COPD related to asthma or cystic fibrosis (**) assessed from last health check or the last exams for COPD diagnosis clinical instability and/or respiratory exacerbation within the previous three months clinical instability and/or respiratory exacerbation dangerous for catheterization Pregnancy (ßHCG > 20 UI /l) or breastfeeding on going General corticotherapy > 0,5 mg/kg/day prédnisolone equivalent Hepatic insufficiency (TP < 50%) or renal insufficiency (creatininemia > 130 µmol/l) or diabetes mellitus type I or II (treated by oral antidiabetic or insulin) Left-heart failure (coronary heart disease and/or left valvulopathy) High level of prostatic specific antigen (PSA) (> 2,5ng/ml) Cancer antecedent or treatment on going | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Septal Defect, Atrial ASD less than or equal to 18 mm Conditions that would confound treatment of ASD or complicate distinguishing onset of adverse events Unable to accommodate device delivery catheter | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 14.0-999.0, Pulmonary Arterial Hypertension (PAH) Non-specific: 1. Written informed consent obtained. 2. No participation in another AMG driven study attendancing this treatment protocol Specific: 1. Age at least 14 years 2. Presence of cyanosis with < 93 % arterial oxygen saturation (measured by transcutaneous pulse oximetry) 3. Clinical indication for the invasive diagnostic procedures planned for the study is given; this is evaluated on the basis of observation before, during and after medicinal therapy) 4. Presence of PAH as diagnosed by invasive methods with Rp:Rs > 0.5 measured at rest, before testing of pulmonary vasodilatory reserve 5. One of the following diagnoses: 1. non-corrected large congenital shunting defect at atrial, ventricular or arterial level PAPVD ASD SVD VSD AVSD TAC APW PDA combinations thereof. 2. Surgically corrected shunting defect (diagnoses as above) with significant residual defect 3. Other diagnoses with univentricular physiology/ hemodynamics Non-specific: 1. pregnancy or lactation 2. women of child-bearing age who are sexually active without practising highly effective methods of contraception 3. any diseases or impairment that, in the opinion of the investigator a subject from participation 4. substance abuse (alcohol, medicines, drugs) 5. other medical, psychological or social circumstances that would adversely affect a patient's ability to participate reliably in the study or increase the risk to themselves or others if they participated 6. insufficient compliance 7. missing willingness to storaging and transferring pseudonymous disease data within this study. 8. subjects who are not able to perform Cardio-Pulmonary Exercise Testing (CPX). Specific: 1. pulmonary hypertension secondary to any etiology other than those specified in the 2. subjects with known intolerance of NO and iloprost or their constituents 3. acute decompensated heart failure within the 7 days before the invasive diagnostic procedure 4. clinically significant haemoptysis within the last 6 months 5. hemodynamic instability which would represent an unjustifiable risk during testing of pulmonary arterial vasoreagibility 6. arterial hypotension (as defined by age-specific values) 7. anemia (Hb < 10 g/dl) 8. decompensated symptomatic policythemia; (details: 4.2.2 ) 9. thrombocytopenia (< 50.000/µl) 10. secondary impairment of organic function impairment of renal function (GFR < 30 ml/min/1,73 m2 BSA) impairment of hepatic function (ALT and/or AST > 3 x ULN and bilirubin ≥ 2 mg/dl) 11. other sources of pulmonary blood flow which prohibit measurement of the blood flow into the lungs and therefore of the pulmonary vascular resistance Glenn BT shunt significant number of MAPCAs; (details: 4.2.2 ) 12. Obstruction of pulmonary blood outflow obstruction of pulmonary venous return mitral valve dysfunction 13. Left heart diseases | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 65.0-999.0, Congestive Heart Failure Dyspneic patients 65 years or older presenting to the ED with chief complaint of shortness of breath hemodynamically unstability | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Mitral Valve Insufficiency Heart Failure Symptomatic chronic heart failure, New York Heart Association (NYHA) class II to IIIb Left ventricular ejection fraction of 0.35 due to non-ischemic etiology Evidence by transthoracic echocardiography (TTE) of moderate or severe MR without obvious primary mitral valve pathology Peak VO2 less than or equal to 22 ml/kg/min, as obtained at study entry Optimal heart failure therapy for at least 6 months prior to study entry Significant coronary artery disease (greater than 75% lesion in any vessel) by coronary angiography or by a history of a prior heart attack Heart failure due to active myocarditis, congenital heart disease, or obstructive hypertrophic cardiomyopathy Significant ventricular arrhythmias not treated with an implantable defibrillator Primary MR due to significant chordal or leaflet abnormalities by TTE Other hemodynamically relevant stenotic or regurgitant valvular diseases Severe tricuspid regurgitation (TR) (moderate TR is allowed) Severe pulmonic regurgitation (PR) (moderate PR is allowed) Moderate to severe aortic regurgitation Any moderate to severe stenotic lesions using American Heart Association/American College of Cardiology (AHA/ACC) 31 Dependence on chronic inotropic therapy | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, COPD Pulmonary Hypertension Diagnosis of COPD Prior admittance to hospital with exacerbation in COPD Informed consent Exacerbation in COPD less than 6 weeks before examination | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, High Altitude Pulmonary Edema Acute Mountain Sickness Participant in Apex 2 high altitude expedition Previous history of high altitude pulmonary edema | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-85.0, Heart Failure, Diastolic Clinical features consistent with heart failure LVEF ≥ 50%, with no evidence of significant valvular disease, no hypertrophic cardiomyopathy, and no evidence of pericardial constriction A peak VO2 < 80% predicted, with a pattern of gas exchange on metabolic exercise testing indicating a cardiac cause for limitation Objective evidence of lung disease on formal lung function testing Reversible myocardial ischaemia on contrast-enhanced myocardial stress Echocardiography, and evidence of exercise-induced mitral regurgitation Obesity (BMI >35) or a locomotor cause for exercise limitation | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Hypertension, Pulmonary Included patients had primary pulmonary arterial hypertension, pulmonary hypertension secondary to connective tissue disease, or pulmonary hypertension with surgical repair at least 5 years previously; a mean pulmonary artery pressure greater than or equal to 25 mmHg and a pulmonary artery wedge pressure of less than or equal to 15 mmHg at rest, via right heart catheterisation within 21 days prior to randomisation; and a baseline 6-Minute Walk test distance of 100 m to 450 m Subjects with congenital heart disease, pulmonary hypertension due to thromboembolism, HIV, chronic obstructive airway disease, congestive heart failure or schistosomiasis; subjects with significant (ie > 2+) valvular disease other than tricuspid regurgitation or pulmonary regurgitation; and acutely decompensated heart failure within the previous 30 days; subjects with pulmonary hypertension secondary to any other etiology other than those specified in the criteria | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Fat Embolism Syndrome Must be a candidate for Computer Assisted Total Knee Arthroplasty Mentally incompetent patients excluded | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-80.0, Lung Diseases, Obstructive Chronic Heart Failure Chronic Hypoxemia Age 40 > 80 years Confirmed COPD (GOLD criteria) Moderate and stable hypoxemia (55< PaO2 <65 mmHg) Clinical signs of CHF (ESC criteria) Ex-smokers (> 20 pack/years) since at least 3 months Clinical instability and/or exacerbation Congenital heart diseases Lung cancer Thoracic restrictive disorders Other coexisting diseases influencing 3-yr survival | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure Obstructive Sleep Apnea Heart Failure, Congestive Previously unrecognized OSA with an Apnea Hypopnea Index (AHI) > 15 events per hour on the attended in-hospital sleep study. Patients with apnea index of less than 5 events / hour are excluded (see design consideration for rationale) Projected length of stay 3 days or more on the morning following the cardio-respiratory sleep study Ongoing or planned targeted treatment for heart failure including one of the following: IV diuretics, IV infusion of inotropes or vasodilators, or planned revascularization, or device therapy Patients who are already diagnosed with OSA Patients with Central Sleep Apnea Patients with diastolic only heart failure Cardiogenic shock and hemodynamic instability with MAP less than 55 mmHg off vasopressors, or concurrently on vasopressor treatment, left ventricular assist devices, or intra-aortic Balloon Pump. Inotropic agents will not constitute an criterion. Patients will be eligible once off vasopressors Acute respiratory failure or insufficiency defined by P/F (PaO2/FIO2) ratio less than 250, or FIO2 requirement more than 50% Overt neurological deficit Renal failure requiring renal replacement therapy; Patients will not be excluded if they were undergoing ultra-filtration for volume removal Patients scheduled for procedures that will interfere with post randomization measurement: This includes scheduled coronary bypass surgery, or expected left ventricular assist device placement Patients who arrived from a long-term care facility or expected to be discharged to one; and patients who have very poor functional outcome precluding ability to use the CPAP device independently Patients on long term or "bridging" inotropic infusion, or short life expectancy due to concomitant illness | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 40.0-999.0, Pulmonary Disease, Chronic Obstructive Pulmonary Fibrosis Cough For all patients history of clinically significant chronic cough for > 3 months For COPD patients >40 years of age pack-year history of smoking GOLD classification of Stage 1 or higher For IPF patients > 50 years of age history of unexplained dyspnea on exertion of > 3 months exhibits coughing and bilateral, basilar, inspiratory crackles on physical exam ACE inhibitor use GERD current cancer or history of lung cancer non-ambulatory hospitalized in the previous 12 months for heart failure | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Chronic Obstructive Pulmonary Disease Asthma Pulmonary Embolism Pneumonia Pulmonary Hypertension Presenting to the Emergency Department with signs and symptoms of dyspnea (short of breath, tachypnea, hyperventilation, etc) within the last 24 hours Normal cardiac output as measured by noninvasive bioimpedance Greater than or equal to 18 years of age Patient or legal representative willing and able to provide informed consent and comply with study procedures A history of congestive heart failure and a BNP > 500pg/mL (or NTproBNP > 900pg/mL) if obtained by the treating physician Treating physician suspects patient has new onset congestive heart failure ECG changes diagnostic of acute myocardial ischemia/infarction Ischemic chest pain within the prior 24 hours by history Obvious chest trauma | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Primary Myelofibrosis Primary Pulmonary Hypertension Secondary Myelofibrosis Pulmonary Arterial Hypertension > 18 years of age 2. Signed Informed Consent 3. Subjects diagnosed with Primary Pulmonary Hypertension or Myelofibrosis (primary or secondary) Anyone not meeting the above 2. Pregnant women | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure Patients with an dual chamber ICD or CRTD (with an existing functional atrial lead) with remote monitoring capabilities and EF <= 40% within the last 3 months by echocardiogram, nuclear imaging, MRI or cardiac catheterization, 2. Persistent or chronic symptomatic AF resistant to anti-arrhythmic medication other than Amiodarone. Resistant defined as recurrent AF of greater than 5 minutes duration at least once per month. 3. Therapeutic anticoagulation for at least three weeks prior to initiation of therapy 4. Ability to complete 6 minute walk test. 5. Age >= 18 years old. (Females must be either post-menopausal >12 months, practicing a protocol-acceptable method of birth control [defined as injectable or implantable hormonal contraceptives, oral contraceptives, intrauterine device, diaphragm plus spermicide], or have had a hysterectomy, bilateral oophorectomy, or tubal ligation performed at least 6 months prior to enrollment). 6. All patients optimized on CHF medications including beta-blocker and ace-inhibitor or angiotensin-receptor blocker. 7. patients receiving low dose amiodarone <200 mg for 2 or less months The are: 1. Reversible causes of AF such as pericarditis, hyperthyroidism, 2. Presently with Valvular Heart disease requiring surgical intervention 3. Presently with coronary artery disease requiring surgical intervention 4. Early Post-operative AF (within three months of surgery) 5. Previous MAZE or left atrial instrumentation 6. Prolonged QT interval 7. Hypothyroidism 8. Liver Failure 9. Life expectancy <= 2 years 10. Social factors that would preclude follow up or make compliance difficult. 11. Contraindication to the use of anti-arrhythmic medications and/or coumadin and heparin 12. Enrollment in another investigational drug or device study. 13. Patients with severe pulmonary disease i.e. COPD or asthma 14. Documented intra-atrial thrombus, tumor, or another abnormality which precludes catheter introduction 15. Any ophthalmologic disorders (other than requiring glasses for vision correction) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-80.0, COPD Pulmonary Hypertension A mean pulmonary arterial pressure of 25 mmHg measured by right heart catheterization A diagnosis of COPD-associated pulmonary hypertension, implicating that no other potential cause of pulmonary hypertension is present Stable COPD, defined by no subjective alterations of symptoms or use of short acting beta-antagonists for minimally six weeks. Any exacerbation resulting in admission to hospital or other contacts to a physician must have ceased for at least 6 weeks before entering the study Age > 18 Informed written consent Reliable anticonception for fertile women Rheumatic disease limiting walking capacity Exacerbation in COPD during the study Age>80 years FEV1 < 25 % of predicted Allergy towards contents of sildenafil or placebo tablets Fall in blood pressure of > 30 mmHg systolic or >20 mmHg diastolic after intake of the first dose of trial medication Fall in peripheral saturation of > 5% after intake of the first dose of trial medication Treatment with nitrous vasodilators or aminophyllamines | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-85.0, Peripheral Vascular Disease Cardiovascular Disease Subject must be 18-85 Subject must be an acceptable candidate for an elective,non-emergent diagnostic procedure performed percutaneously via the common femoral artery through either a 5F or 6F procedural sheath Subject is an acceptable candidate for emergent vascular surgery Subject agrees to follow-up evaluations to assess for complications related to femoral access site If among the 50 ultrasound sub-study Subjects enrolled,Subject agrees to have an ultrasound of femoral artery performed post-procedure during the 30 ± 7 days follow-up visit Subject or legal representative has been informed of the nature of the study and agrees to provisions and has provided written informed consent as approved by the Institutional Review Board of respective clinical site History of bleeding diathesis or coagulopathy including hemophilia, von Willebrand's disease, and/or a current, known platelet count <100,000 cells/mm3, or baseline INR > 1.7 Body Mass Index (BMI) ³ 35 kg/m2 Presence of significant anemia (Hgb < 10 g/dL, Hct < 30%) Advanced Subject refusal of blood transfusions, should transfusion become necessary | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 0.0-999.0, Chronic Obstructive Pulmonary Disease Chronic Obstructive Pulmonary Disease Cardiac failure Renal failure Liver failure Diabetes mellitus Systemic inflammatory diseases eg Rheumatoid arthritis, SLE Warfarin, coagulation problems | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure Previous clinical diagnosis of heart failure with current New York Heart Association (NYHA) Class II-IV symptoms Has experienced at least one of the following in the 12 months before study entry Hospitalization for decompensated heart failure Acute treatment with intravenous loop diuretic or hemofiltration Mean pulmonary capillary wedge pressure greater than 15 mm Hg or left ventricular end diastolic pressure (LVEDP) greater than 18 mm Hg at catheterization for dyspnea Long term treatment with a loop diuretic and chronic diastolic dysfunction on echocardiography, as determined by left atrial enlargement Left ventricular ejection fraction greater than or equal to 50%, as determined by a clinical echocardiogram or ventriculogram in the 12 months before study entry Receiving stable medical therapy in the 30 days before study entry, as determined by no addition or removal of angiotensin converting enzyme inhibitor (ACE), angiotensin receptor blocker (ARB), beta-blockers, or calcium channel blockers (CCB) and no change in dosage of ACE, ARBs, beta-blockers, or CCBs of more than 100% Has a neuromuscular, orthopedic, or other non-cardiac condition that prevents individual from exercise testing on a bicycle ergometer or from walking in a hallway Non-cardiac condition that limits life expectancy to less than 1 year at the time of study entry, based on the judgment of the physician Current or anticipated future need for nitrate therapy Valve disease (i.e., greater than mild aortic or mitral stenosis; greater than moderate aortic or mitral regurgitation) Hypertrophic cardiomyopathy Infiltrative or inflammatory myocardial disease (e.g., amyloid, sarcoid) Pericardial disease Primary pulmonary arteriopathy Has experienced a heart attack or unstable angina, or has undergone percutaneous transluminal coronary angiography (PTCA) or coronary artery bypass grafting (CABG) in the 60 days before study entry, or requires either PTCA or CABG at the time of study entry Other clinically important causes of dyspnea, such as morbid obesity or significant lung disease, as defined by clinical judgment or use of steroids or oxygen for lung disease | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Left Atrial Appendage Exclusion Subject is greater than or equal to 18 years of age. 2. Subject has any one of the following risk factors and is thought to benefit from LAA occlusion CHADS score > 2 Age > 75 years Hypertension and age > 65 years Previous stroke History of atrial fibrillation (any classification) 3. Subject is scheduled to undergo elective non-endoscopic cardiac surgical procedure(s) including cardiac surgery for one or more of the following: Mitral valve repair or replacement, Aortic valve repair or placement, Tricuspid valve repair or replacement, Coronary artery bypass procedures, concomitant surgical (ablation or cut-and-sew) Maze procedure, patent foramen ovale (PFO), atrial septal defect (ASD) repair with the device deployed while on or prepared for cardio-pulmonary bypass support. 4. Subject is willing and able to provide written informed consent. 5. Subject has a life expectancy of at least 1 year. 6. Subject is willing and able to return for scheduled follow-up visits Previous cardiac surgery 2. Thrombus in the LAA/LA which cannot be evacuated prior to placement of the Clip. 3. Patients requiring surgery other than CABG and/or cardiac valve and/or surgical maze procedure and/or PFO closure and/or ASD repair. 4. NYHA Class IV heart failure symptoms 5. Need for emergent cardiac surgery (i.e. cardiogenic shock) 6. Creatinine >200 µmol/L 7. LAA is not appropriate for based on intraoperative evaluations 8. Current diagnosis of active systemic infection 9. Renal failure requiring dialysis or hepatic failure 10. A known drug and/or alcohol addiction 11. Mental impairment or other conditions which may not allow the subject to understand the nature, significance and scope of the study 12. Pregnancy or desire to get pregnant within 12-months of the study treatment 13. Preoperative need for an intra-aortic balloon pump or intravenous inotropes 14. Patients who have been treated with thoracic radiation 15. Patients in current chemotherapy 16. Patients on long term treatment with steroids not including intermittent use of inhaled steroids for respiratory diseases. 17. Patients with known connective tissue disorders | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-70.0, Congestive Heart Failure Patients with congestive heart failure NYHA I-II Stable clinical condition Resting systolic blood pressure < 100 Resting heart rate < 50 Hypersensitivity to dexmedetomidine, phenylephrine, isoproterenol, terbutaline | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure Age greater than 18 years Diagnosis of chronic heart failure with an ejection fraction less than or equal to 45% per either an echocardiogram, nuclear cardiolyte stress test, or cardiac catheterization performed within the past 6 months New York Heart Association Functional Class III Must be on optimal heart failure therapy according to AHA/ACC heart failure guidelines, including treatment with ACEI and beta-blocker therapy, or have documented rationale for variation, including intolerance, contraindication, patient preference, or personal physician's judgment Comorbid disease or behavioral or other limitations that: 1) interfere with performing exercise test, or 2) prevent completion of 12 week study Currently pregnant or intent to become pregnant in the next 12 weeks or currently breastfeeding Major cardiovascular event or cardiovascular procedure within the prior 6 weeks History of Chronic Obstructive Pulmonary Disease or Reactive Airway Disease Known hypersensitivity to sildenafil Current use of medications known to be a potent inhibitor of CYP3A4 (e.g., ritonavir, ketoconazole, itraconazole) Current or recent (within 6 months) use of organic nitrate medications (e.g. isosorbide dinitrate, sublingual nitroglycerin) Known diagnosis of pulmonary veno-occlusive disease or non-arteritic anterior ischemic optic neuropathy | 1 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 16.0-80.0, Pulmonary Arterial Hypertension Pulmonary Hypertension Current diagnosis of symptomatic pulmonary arterial hypertension (PAH) classified by one of the following: idiopathic arterial hypertension (IPAH), primary pulmonary hypertension (PPH), familial pulmonary arterial hypertension (FPAH) or pulmonary arterial hypertension (PAH) associated with connective tissue diseases. Has WHO functional class III symptoms Previous exposure to an endothelin receptor antagonist (ETRA) such as sitaxsentan, bosentan or ambrisentan | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Heart Failure Sleep Apnea Syndromes Patients over the age of 18 and able to consent NYHA Class III/IV Symptoms Objective Evidence of Heart Failure Physical Exam consistent with Heart Failure BNP greater than 500 Radiographic Evidence of Heart Failure SDB (AHI > 5/hour) on Nexan CPS Ability to tolerate appropriate medical therapy for Heart Failure Willingness to follow-up as an outpatient after discharge Patients with known Sleep-Disordered Breathing currently using CPAP or BiPAP Patients with known COPD Patients with known restrictive lung disease Patients with tracheostomy in place Acute MI/Acute Coronary Syndrome at time of hospitalization Cardiogenic Shock Troponin Leak greater than 0.08 Patients with major multisystem disorders Expected survival less than 6 months End Stage Renal Disease on Dialysis | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Mitral Valve Insufficiency Coronary Artery Disease Chronic severe ischemic mitral regurgitation (often with tethering as a major mechanism) in the judgment of the clinical site echocardiographer, assessed by transthoracic echocardiogram. Assessment of mitral regurgitation will be performed using an integrative method (Zoghbi W. et al. J. American Society of Echocardiography. 2003:16:777-802. see appendix). Quantitative guidelines as proposed would be: ERO ≥ 0.4 cmsq. If ERO < 0.4, then the degree of mitral regurgitation will be guided by other color Doppler quantitative methods (jet area/left atrial area ratio, vena contracta, supportive in an integrated fashion Eligible for surgical repair and replacement of mitral valve CAD with or without the need for coronary revascularization Any evidence of structural (chordal or leaflet) mitral valve disease or ruptured papillary muscle Prior mitral valve repair Severe irreversible pulmonary hypertension in the judgment of the investigator Medically unable to undergo cardiopulmonary bypass (CPB) Inability to derive ERO and end-systolic volume index (ESVI) by transthoracic echocardiography Planned concomitant intra-operative procedures (with the exception of tricuspid valve repair, closure of patent foramen ovale [PFO] or atrial septal defect [ASD] or Maze procedure) Clinical signs of cardiogenic shock at the time of surgery Treatment with long-term intravenous inotropic therapy at the time of surgery ST segment elevation myocardial infarction (MI) requiring intervention in the 7 days before surgery Congenital heart disease (except PFO or ASD) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-75.0, Right Heart Failure Pulmonary Hypertension Patients with World Health Organization (WHO) group 1 pulmonary arterial hypertension [51], excluding patients with portal hypertension, meeting the following hemodynamic parameters Mean pulmonary artery pressure (mPAP) >35 mmHg at rest, and Pulmonary capillary wedge pressure (PCWP) <15 mmHg, and Pulmonary vascular resistance (PVR) >1.5 wood units, and 2. Age 18 to 75 years 3. Right ventricular failure defined by right atrial pressure >7 mmHg along with either dilated right ventricle, or absence of inferior vena cava collapse or BNP >100 pg/ml 4. Patients of childbearing age must be practicing effective birth control. 5. Normal left ventricular function as assessed by echocardiogram, multiple gated acquisition (MUGA) cardiac scan, or invasive left ventriculography Group 2-5 pulmonary hypertension as defined by WHO Pulmonary hypertension with left heart failure (as assessed by echocardiogram, multiple gated acquisition (MUGA) cardiac scan, or invasive left ventriculography) Pulmonary hypertension associated with lung disease and/or hypoxemia (e.g. chronic obstructive pulmonary disease, interstitial lung disease, sleep disordered breathing, chronic exposure to high altitude, alveolar hypoventilation syndrome Pulmonary hypertension due to chronic thrombotic and/or embolic diseases Miscellaneous such as sarcoidosis, compression of pulmonary vessels by adenopathy, tumor 2. Systemic hypertension, defined as a systolic pressure >140 mmHg or a diastolic blood pressure >90 mmHg 3. Patients taking angiotensin converting enzyme (ACE) inhibitor or angiotensin receptor blockers (ARBs) 4. Pregnancy 5. Chronic kidney disease (serum creatinine > 2.5mg/dl, proteinuria >500 mg/day, hematuria) 6. Cirrhosis or portal hypertension 7. Inability to provide informed consent. 8. Allergy to conivaptan or spironolactone. 9. Active malignancy 10. Patients receiving spironolactone 11. Enrollment in other interventional studies. 12. Patients on Highly Active Antiretroviral Therapy (HAART) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-75.0, Heart Failure, Diastolic Hypertension, Pulmonary Clinically signs or history of congestive heart failure NYHA II-III (Fatigue, dyspnea on exertion, lung crepitations, pulmonary edema, ankle and or lower leg swelling, jugular pressure enhancement, hepatomegaly) Echocardiographic signs of diastolic dysfunction (heart failure with normal ejection fraction) Right ventricle enlargement with pulmonary hypertension minute walking distance > 150 m < 400 m Right Heart Catheterization: Mean PAP > 25 mmHg, PCWP > 15 mmHg Echocardiographic requirements for definition of heart failure with normal ejection fraction E/E' > 15, or E/E' > 8 + NTpBNP > 220 pg/ml, or E/E' > 8 + E:A < 0.5 + DT > 280 ms or Ard-Ad > 30 ms or atrial enlargement or Patients who are not on guideline conform treatments for cardiovascular disease Left ventricle systolic dysfunction (EF < 50 %), aortic stenosis with peak gradient (instantane) > 40 mm Hg,moderate and severe aortic insufficiency moderate and severe mitral regurgitation acute coronary disease, stable coronary artery disease or peripheral vascular disease limiting exercise Other causes of pulmonary artery hypertension relevant obstructive ventilatory disease > grade II (lung functions tests) collagen disease (Tests: MSCT and ANA, ANCA) chronic thrombo | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Chronic Obstructive Pulmonary Disease Diagnosis of advanced Chronic Obstructive Pulmonary Disease (COPD) must be made on the basis of current findings, medical history and physical examination Subject not qualifying because of physical or psychological condition that may put them at risk of participating | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 16.0-90.0, Diastolic Heart Failure HFpEF will be defined as Clinical features consistent with heart failure LVEF ≥ 50%, with no evidence of significant valvular disease No hypertrophic cardiomyopathy, and no evidence of pericardial constriction Peak VO2 < 80% predicted, with RER>1 and with a pattern of gas exchange on metabolic exercise testing indicating a cardiac cause for limitation) Patients recruited will be in sinus rhythm BMI >35 Objective evidence of lung disease on formal lung function testing Reversible myocardial ischaemia on contrast-enhanced myocardial stress Echocardiography, and no evidence of exercise-induced mitral regurgitation (>2+) Impaired hepatic function; known hypersensitivity to perhexiline | 2 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-70.0, Pulmonary Hypertension Heart Failure With Preserved Ejection Fraction Catheterization 1. Elevated pulmonary arterial pressure (PA mean >25mmHg) 2. Elevated pulmonary vascular resistance (>240 dynes.cm.sec-5) or transpulmonary gradient (>12 mmHg) 3. Elevated LVEDP (>15mmHg, but ≤23 mmHg) 2. Evidence of left ventricular diastolic dysfunction: LA>4.0, LVH or diastolic dysfunction by mitral filling pattern 3. Echocardiogram: Normal or mildly reduced LV ejection fraction (greater than or equal to 40%) 4. Symptomatic chronic HF (WHO functional class II-IV) 5. Baseline walk distance 100 to 400 meters 6. Age 18 (increased from 70) Maximal treatment of diastolic dysfunction as noted by the treating physicians with no change in medical therapy for one month prior to entry Use of endothelin receptor antagonist, prostacyclin or PDE-5 inhibitor within 4 weeks of enrollment 2. Exercise capacity limited by other illness (other lung disease, arthritis, mobility limitations) 3. Uncontrolled systemic hypertension 4. Uncontrolled atrial fibrillation 5. Severe valvular disease 6. Pregnant females females of child bearing potential will need to use contraceptive agent barrier given the teratogenicity associated with ERA's 7. Uncontrolled OSA | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 55.0-999.0, Stroke Transient Ischemic Attack Atrial Fibrillation Atrial Flutter Diagnosis of the index event* made by a stroke specialist of an acute ischemic stroke or TIA (WHO definition) of undetermined etiology (cryptogenic) occurring within the previous 6 months (180 days).The event must be either an embolic arterial ischemic stroke confirmed by neuroimaging; or a transient ischemic attack, defined as involving a focal unilateral motor deficit, speech/language deficit, or hemianopia, with symptom duration <24 hours (note: amaurosis fugax/transient monocular blindness, pure sensory spells, isolated vertigo spells, etc. do not qualify for enrolment given the potential for misdiagnosis of such events). 2. Patient meets the following At least one 12-lead ECG has already been obtained as part of the routine clinical post-stroke/TIA work-up, and no ECGs have shown any episodes of atrial fibrillation or atrial flutter, and A Holter monitor has already been obtained as part of the routine clinical post-stroke/TIA work-up, and does not show any episodes of atrial fibrillation or atrial flutter ≥30 seconds. 3. The patient is being actively investigated for the etiology of the stroke/TIA event and additional cardiac monitoring is desired to screen further for the possibility of occult paroxysmal atrial fibrillation/flutter, i.e. patients selected for this study are those for whom the investigator, in his/her clinical judgment, would consider ordering a repeat Holter monitor as part of clinical care. 4. The following diagnostic tests have already been completed as part of clinical routine post-stroke/TIA brain imaging with CT or MRI vascular imaging of the extracranial and intracranial circulation with either CT angiography or MR angiography to significant large vessel occlusive disease as the most likely mechanism for the index ischemic event (carotid Doppler ultrasound is acceptable for those presenting with anterior circulation ischemic events) transthoracic (or transesophageal) echocardiography to thrombus or other structural heart disease that in the opinion of the investigator is the most likely cause for the stroke/TIA event. [Note: If a baseline echocardiogram cannot be obtained clinically after the index event and prior to study enrollment, then it is acceptable for study purposes for an echocardiogram to be obtained after patient enrollment into the study but prior to the 90-day follow-up visit. Alternatively, an echocardiogram already performed within one year prior to study enrollment may serve as the baseline echocardiogram for study purposes.] 5. Age 55 years or older [Note: Participants aged 55-59 years should have imaging confirmation of the index stroke/TIA event with an embolic imaging pattern of acute cerebral ischemia] 6. Informed consent from the patient (or from a legally authorized representative if the patient is not competent, e.g. due to stroke-related cognitive impairment, aphasia, or anosognosia). 7. The patient is expected to survive at least 6 months. 8. The patient has a valid provincial health insurance number The index event will be defined as the event leading to medical presentation 1. Any previously documented atrial fibrillation or atrial flutter, i.e. a past history of atrial fibrillation/flutter or atrial fibrillation/flutter detected on ECG, Holter, or telemetry following the index stroke/TIA event (a remote history of transient perioperative atrial fibrillation is not exclusionary 2. Exclusively retinal stroke or TIA event. 3. A most responsible etiological diagnosis for the qualifying stroke/TIA event has already been determined, i.e. probable small-vessel (lacunar) disease, probable large vessel disease, cervicocephalic artery dissection, venous sinus thrombosis, hypercoagulable states, or other known cause. 4. Planned carotid endarterectomy within 90 days. 5. Patient is already currently participating in a clinical trial involving an investigational medication or device.* 6. Any finding on echocardiography for which there is already an evidence-based indication for long-term anticoagulation (e.g. mechanical heart valve, thrombus, etc.). 7. Endocarditis 8. Pacemaker or ICD device. 9. Patients with known skin reactions to synthetic polymers or to silver. (Some people who display sensitivity to silver jewellery are sensitive to the impurities present in silver alloys and not to the silver itself. These people may participate.) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 50.0-999.0, Pulmonary Disease, Chronic Obstructive Signed and dated written informed consent obtained from the subject and/or subject's legally acceptable representative prior to study participation Males or females greater then or equal to 50 years of age A post-albuterol FEV1/FVC ratio of < or equal to 0.70 A post-albuterol FEV1 < 80% of predicted normal Patients can be current or fomer smoker and must have a cigarette smoking history of > greater then or equal to 10 pack-years A current diagnosis of asthma A body mass index (BMI) of > or equal to 35kg/m2 A respiratory diagnosis other than COPD (e.g., lung cancer, bronchiectasis, sarcoidosis, tuberculosis, lung fibrosis) | 0 |
79 yo F with multifactorial chronic hypoxemia and dyspnea thought due to diastolic CHF, pulmonary hypertension thought secondary to a chronic ASD and COPD on 5L home oxygen admitted with complaints of worsening shortness of breath. Cardiology consult recommended a right heart cath for evaluation of response to sildenafil but the patient refused. Pulmonary consult recommended an empiric, compassionate sildenafil trial due to severe dyspneic symptomology preventing outpatient living, and the patient tolerated an inpatient trial without hypotension. Patient to f/u with pulmonology to start sildenifil chronically as outpatient as prior authorization is obtained. Past Medical History: - Atrial septal defect repair [**6-17**] complicated by sinus arrest with PPM placement. - Diastolic CHF, estimated dry weight of 94kg - Pulm HTN (RSVP 75 in [**11-24**]) thought secondary to longstanding ASD - COPD on home O2 (5L NC) with baseline saturation high 80's to low 90's on this therapy. - OSA, not CPAP compliant - Mild mitral regurgitation - Microcytic anemia - Hypothyroidism - S/p APPY, s/p CCY ('[**33**]) - Gallstone pancreatitis s/p ERCP, sphincterotomy - Elevated alk phos secondary to amiodarone | eligible ages (years): 18.0-999.0, Gallstone Pancreatitis All adults are included between the age of 18 and 100 with mild to moderate gallstone pancreatitis A subject is classified as having gallstone pancreatitis if they had the following: 1. upper abdominal pain, nausea, vomiting and epigastric tenderness; 2. absence of ethanol abuse; 3. elevated amylase level to at least twice the upper limit of normal and elevated lipase level to at lease three times the upper limit of normal; and 4. imaging confirmation of gallstones The classification of mild to moderate pancreatitis is defined by the presence of the following: 1. three or fewer Ranson's on admission: age > 55 years, glucose > 200 mg/dL , LDH> 350 mg/dL, AST > 250 units/L, and WBC>16 K/mm3; 2. clinical stability with admission to a non-monitored ward bed; 3. absence of acute cholangitis: defined as a temperature >38.6°C, right upper quadrant pain and tenderness, and significant hyperbilirubinemia; and 4. low suspicion for a retained common bile duct (CBD) stone (total bilirubin <4 mg/dl on admission) Severe pancreatitis (as defined by the presence of more than three Ranson's on admission) Suspected concomitant acute cholangitis High suspicion for retained common bile duct stone (total bilirubin ≥ 4 mg/dl on admission or ultrasound demonstration of CBD stone) Patient refusal to participate Severe preexisting medical comorbidities contraindicating cholecystectomy (as determined by the primary physicians) Pregnancy Prior gastric bypass surgery (making ERC difficult ) Admission to a monitored unit. The need for admission to a monitored bed is determined by the admitting surgeon and is guided primarily by a need for aggressive fluid administration as demonstrated by severe volume depletion (e.g., admission tachycardia >110 beats/minute, blood urea nitrogen > 15 mg/dl) or evidence of cholangitis | 0 |
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