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Surgery_Schwartz_5202 | Surgery_Schwartz | repair was undertaken by subtotal closure of the ASD, extensive resection of the right atrium, and vertical plication of the atrialized chamber.132 Five-year follow-up revealed all patients to be asymptomatic and in sinus rhythm without medi-cations. Recently, they have reported on their 20-year experi-ence with treating 32 such neonates with an overall mortality of 40%. Surgical management of neonates with Ebstein’s anom-aly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair.132Results. In the neonatal period, the most common postopera-tive problem, whether after a simple palliative procedure such as a BT shunt or following a more extensive procedure such as attempted exclusion of the RV, has been low cardiac out-put. Supraventricular tachycardia also has been problematic postoperatively. Complete heart block necessitating pacemaker | Surgery_Schwartz. repair was undertaken by subtotal closure of the ASD, extensive resection of the right atrium, and vertical plication of the atrialized chamber.132 Five-year follow-up revealed all patients to be asymptomatic and in sinus rhythm without medi-cations. Recently, they have reported on their 20-year experi-ence with treating 32 such neonates with an overall mortality of 40%. Surgical management of neonates with Ebstein’s anom-aly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair.132Results. In the neonatal period, the most common postopera-tive problem, whether after a simple palliative procedure such as a BT shunt or following a more extensive procedure such as attempted exclusion of the RV, has been low cardiac out-put. Supraventricular tachycardia also has been problematic postoperatively. Complete heart block necessitating pacemaker |
Surgery_Schwartz_5203 | Surgery_Schwartz | procedure such as attempted exclusion of the RV, has been low cardiac out-put. Supraventricular tachycardia also has been problematic postoperatively. Complete heart block necessitating pacemaker implantation should be uncommon if the techniques described to avoid suturing between the coronary sinus and the tricuspid annulus are used.There are few published reports of outcomes, due to the rarity of this defect. However, based on the natural history of this condition, which is remarkably benign for the majority of older patients, the outlook should be excellent for patients who have survived the neonatal period.127,131,132,137Transposition of the Great ArteriesAnatomy. Complete transposition is characterized by connec-tion of the atria to their appropriate ventricles with inappropriate ventriculoarterial connections. Thus, the aorta arises anteriorly from the RV, while the pulmonary artery arises posteriorly from the LV. Van Praagh and coworkers introduced the term dextro-transposition | Surgery_Schwartz. procedure such as attempted exclusion of the RV, has been low cardiac out-put. Supraventricular tachycardia also has been problematic postoperatively. Complete heart block necessitating pacemaker implantation should be uncommon if the techniques described to avoid suturing between the coronary sinus and the tricuspid annulus are used.There are few published reports of outcomes, due to the rarity of this defect. However, based on the natural history of this condition, which is remarkably benign for the majority of older patients, the outlook should be excellent for patients who have survived the neonatal period.127,131,132,137Transposition of the Great ArteriesAnatomy. Complete transposition is characterized by connec-tion of the atria to their appropriate ventricles with inappropriate ventriculoarterial connections. Thus, the aorta arises anteriorly from the RV, while the pulmonary artery arises posteriorly from the LV. Van Praagh and coworkers introduced the term dextro-transposition |
Surgery_Schwartz_5204 | Surgery_Schwartz | connections. Thus, the aorta arises anteriorly from the RV, while the pulmonary artery arises posteriorly from the LV. Van Praagh and coworkers introduced the term dextro-transposition of the great arteries (D-TGA) to describe this defect, whereas levo-transposition of the great arteries (L-TGA) describes a form of corrected transposition where there is concomitant AV discordance.138,139D-TGA requires an obligatory intracardiac mixing of blood, which usually occurs at both the atrial and the ventricu-lar levels or via a patent ductus. Significant coronary anomalies occur frequently in patients with D-TGA. The most common pattern, occurring in 68% of cases, is characterized by the left main coronary artery arising from the leftward coronary sinus, giving rise to the left anterior descending and circumflex arteries. The most common variant is for the circumflex coro-nary artery to arise as a branch from the right coronary artery instead of from the left coronary | Surgery_Schwartz. connections. Thus, the aorta arises anteriorly from the RV, while the pulmonary artery arises posteriorly from the LV. Van Praagh and coworkers introduced the term dextro-transposition of the great arteries (D-TGA) to describe this defect, whereas levo-transposition of the great arteries (L-TGA) describes a form of corrected transposition where there is concomitant AV discordance.138,139D-TGA requires an obligatory intracardiac mixing of blood, which usually occurs at both the atrial and the ventricu-lar levels or via a patent ductus. Significant coronary anomalies occur frequently in patients with D-TGA. The most common pattern, occurring in 68% of cases, is characterized by the left main coronary artery arising from the leftward coronary sinus, giving rise to the left anterior descending and circumflex arteries. The most common variant is for the circumflex coro-nary artery to arise as a branch from the right coronary artery instead of from the left coronary |
Surgery_Schwartz_5205 | Surgery_Schwartz | left anterior descending and circumflex arteries. The most common variant is for the circumflex coro-nary artery to arise as a branch from the right coronary artery instead of from the left coronary artery.Pathophysiology. D-TGA results in parallel pulmonary and systemic circulations, with patient survival dependent on intracardiac mixing of blood. After birth, both ventricles are relatively noncompliant, and thus, infants initially have higher pulmonary flow due to the decreased downstream resistance. This causes left atrial enlargement and a left-to-right shunt via the patent foramen ovale.Postnatally, the LV does not hypertrophy because it is not subjected to systemic afterload. The lack of normal extrauter-ine left ventricular maturation has important implications for the timing of surgical repair because the LV must be converted to the systemic ventricle and be able to function against sys-temic vascular resistance. If complete repair is done within the first few weeks of life, | Surgery_Schwartz. left anterior descending and circumflex arteries. The most common variant is for the circumflex coro-nary artery to arise as a branch from the right coronary artery instead of from the left coronary artery.Pathophysiology. D-TGA results in parallel pulmonary and systemic circulations, with patient survival dependent on intracardiac mixing of blood. After birth, both ventricles are relatively noncompliant, and thus, infants initially have higher pulmonary flow due to the decreased downstream resistance. This causes left atrial enlargement and a left-to-right shunt via the patent foramen ovale.Postnatally, the LV does not hypertrophy because it is not subjected to systemic afterload. The lack of normal extrauter-ine left ventricular maturation has important implications for the timing of surgical repair because the LV must be converted to the systemic ventricle and be able to function against sys-temic vascular resistance. If complete repair is done within the first few weeks of life, |
Surgery_Schwartz_5206 | Surgery_Schwartz | repair because the LV must be converted to the systemic ventricle and be able to function against sys-temic vascular resistance. If complete repair is done within the first few weeks of life, the LV usually adapts easily to systemic resistance since it is conditioned to high intrauterine pulmonary vascular resistance. After a few weeks of life, the LV that is conditioned to the decrease in pulmonary resistance that occurs when the lungs inflate after birth may have difficulty adapting to systemic vascular resistance without preoperative preparation or postoperative support. Novel techniques of LV “preparation” using a pulmonary arterial band have been used in cases where complete repair has been delayed (Fig. 20-47A,B).Clinical Manifestations and Diagnosis. Infants with D-TGA and an intact ventricular septum are usually cyanotic at birth, with an arterial Po2 between 25 and 40 mmHg. If duc-tal patency is not maintained, deterioration will be rapid with ensuing metabolic acidosis and | Surgery_Schwartz. repair because the LV must be converted to the systemic ventricle and be able to function against sys-temic vascular resistance. If complete repair is done within the first few weeks of life, the LV usually adapts easily to systemic resistance since it is conditioned to high intrauterine pulmonary vascular resistance. After a few weeks of life, the LV that is conditioned to the decrease in pulmonary resistance that occurs when the lungs inflate after birth may have difficulty adapting to systemic vascular resistance without preoperative preparation or postoperative support. Novel techniques of LV “preparation” using a pulmonary arterial band have been used in cases where complete repair has been delayed (Fig. 20-47A,B).Clinical Manifestations and Diagnosis. Infants with D-TGA and an intact ventricular septum are usually cyanotic at birth, with an arterial Po2 between 25 and 40 mmHg. If duc-tal patency is not maintained, deterioration will be rapid with ensuing metabolic acidosis and |
Surgery_Schwartz_5207 | Surgery_Schwartz | ventricular septum are usually cyanotic at birth, with an arterial Po2 between 25 and 40 mmHg. If duc-tal patency is not maintained, deterioration will be rapid with ensuing metabolic acidosis and death. Conversely, those infants with a coexisting VSD may be only mildly hypoxemic and may come to medical attention after 2 to 3 weeks, when the falling pulmonary vascular resistance leads to symptoms of congestive heart failure.The ECG will reveal right ventricular hypertrophy, and the chest radiograph will reveal the classic egg-shaped con-figuration. Definitive diagnosis is made by echocardiography, which reliably demonstrates ventriculoarterial discordance and any associated lesions. Cardiac catheterization is rarely nec-essary, except in infants requiring surgery after the neonatal period, to assess the suitability of the LV to support the sys-temic circulation. Limited catheterization, however, is useful for performance of atrial septostomy in neonates with inadequate intracardiac | Surgery_Schwartz. ventricular septum are usually cyanotic at birth, with an arterial Po2 between 25 and 40 mmHg. If duc-tal patency is not maintained, deterioration will be rapid with ensuing metabolic acidosis and death. Conversely, those infants with a coexisting VSD may be only mildly hypoxemic and may come to medical attention after 2 to 3 weeks, when the falling pulmonary vascular resistance leads to symptoms of congestive heart failure.The ECG will reveal right ventricular hypertrophy, and the chest radiograph will reveal the classic egg-shaped con-figuration. Definitive diagnosis is made by echocardiography, which reliably demonstrates ventriculoarterial discordance and any associated lesions. Cardiac catheterization is rarely nec-essary, except in infants requiring surgery after the neonatal period, to assess the suitability of the LV to support the sys-temic circulation. Limited catheterization, however, is useful for performance of atrial septostomy in neonates with inadequate intracardiac |
Surgery_Schwartz_5208 | Surgery_Schwartz | to assess the suitability of the LV to support the sys-temic circulation. Limited catheterization, however, is useful for performance of atrial septostomy in neonates with inadequate intracardiac mixing.Surgical Repair. Blalock and Hanlon introduced the first operative intervention for D-TGA with the creation of an atrial septectomy to enhance intracardiac mixing.140 This initial proce-dure was feasible in the pre-CPB era, but carried a high mortal-ity rate. Later, Rashkind and Causo developed a catheter-based balloon septostomy, which largely obviated the need for open septectomy.42These early palliative maneuvers, however, met with lim-ited success, and it was not until the late 1950s, when Senning and Mustard developed the first “atrial repair,” that outcomes improved. The Senning operation consisted of rerouting venous flow at the atrial level by incising and realigning the atrial sep-tum over the pulmonary veins and using the right atrial free wall to create a pulmonary venous | Surgery_Schwartz. to assess the suitability of the LV to support the sys-temic circulation. Limited catheterization, however, is useful for performance of atrial septostomy in neonates with inadequate intracardiac mixing.Surgical Repair. Blalock and Hanlon introduced the first operative intervention for D-TGA with the creation of an atrial septectomy to enhance intracardiac mixing.140 This initial proce-dure was feasible in the pre-CPB era, but carried a high mortal-ity rate. Later, Rashkind and Causo developed a catheter-based balloon septostomy, which largely obviated the need for open septectomy.42These early palliative maneuvers, however, met with lim-ited success, and it was not until the late 1950s, when Senning and Mustard developed the first “atrial repair,” that outcomes improved. The Senning operation consisted of rerouting venous flow at the atrial level by incising and realigning the atrial sep-tum over the pulmonary veins and using the right atrial free wall to create a pulmonary venous |
Surgery_Schwartz_5209 | Surgery_Schwartz | consisted of rerouting venous flow at the atrial level by incising and realigning the atrial sep-tum over the pulmonary veins and using the right atrial free wall to create a pulmonary venous baffle (Fig. 20-48).141Although the Mustard repair (Fig. 20-49) was similar, it made use of either autologous pericardium or synthetic material to create the interatrial baffle.142 These atrial switch procedures Brunicardi_Ch20_p0751-p0800.indd 78022/02/19 2:56 PM 781CONGENITAL HEART DISEASECHAPTER 20ABFigure 20-47. A. Echocardiographic appearance of the LV (‘*’) prior to “LV training”. B. Echocardiographic appearance of the LV (‘*’) after “LV training” achieved by the application of a tight PA band and a mBTS.ACBDFigure 20-48. The Senning operation. A. The atrial septum is cut near the tricuspid valve, creating a flap attached posteriorly between the caval veins. B. The flap of atrial septum is sutured to the anterior lip of the orifices of the left pulmonary veins, effectively separating | Surgery_Schwartz. consisted of rerouting venous flow at the atrial level by incising and realigning the atrial sep-tum over the pulmonary veins and using the right atrial free wall to create a pulmonary venous baffle (Fig. 20-48).141Although the Mustard repair (Fig. 20-49) was similar, it made use of either autologous pericardium or synthetic material to create the interatrial baffle.142 These atrial switch procedures Brunicardi_Ch20_p0751-p0800.indd 78022/02/19 2:56 PM 781CONGENITAL HEART DISEASECHAPTER 20ABFigure 20-47. A. Echocardiographic appearance of the LV (‘*’) prior to “LV training”. B. Echocardiographic appearance of the LV (‘*’) after “LV training” achieved by the application of a tight PA band and a mBTS.ACBDFigure 20-48. The Senning operation. A. The atrial septum is cut near the tricuspid valve, creating a flap attached posteriorly between the caval veins. B. The flap of atrial septum is sutured to the anterior lip of the orifices of the left pulmonary veins, effectively separating |
Surgery_Schwartz_5210 | Surgery_Schwartz | valve, creating a flap attached posteriorly between the caval veins. B. The flap of atrial septum is sutured to the anterior lip of the orifices of the left pulmonary veins, effectively separating the pulmonary and systemic venous channels. C. The posterior edge of the right atrial incision is sutured to the remnant of the atrial septum, diverting the systemic venous channel to the mitral valve. D. The anterior edge of the right atrial incision (lengthened by short incisions at each corner) is sutured around the cava above and below to the lateral edge of the LA incision, completing the pulmonary channel and diversion of pulmonary venous blood to the tricuspid valve area. (Reproduced with permission from Mavroudis C, Backer CL: Pediatric Cardiac Surgery, 2nd ed. St. Louis, MO: Mosby; 1994.) Figure 20-49. Angiographic appearance of a Mustard type baffle repair for dTGA.resulted in a physiologic correction, but not an anatomic one, as the systemic circulation is still based on the RV. | Surgery_Schwartz. valve, creating a flap attached posteriorly between the caval veins. B. The flap of atrial septum is sutured to the anterior lip of the orifices of the left pulmonary veins, effectively separating the pulmonary and systemic venous channels. C. The posterior edge of the right atrial incision is sutured to the remnant of the atrial septum, diverting the systemic venous channel to the mitral valve. D. The anterior edge of the right atrial incision (lengthened by short incisions at each corner) is sutured around the cava above and below to the lateral edge of the LA incision, completing the pulmonary channel and diversion of pulmonary venous blood to the tricuspid valve area. (Reproduced with permission from Mavroudis C, Backer CL: Pediatric Cardiac Surgery, 2nd ed. St. Louis, MO: Mosby; 1994.) Figure 20-49. Angiographic appearance of a Mustard type baffle repair for dTGA.resulted in a physiologic correction, but not an anatomic one, as the systemic circulation is still based on the RV. |
Surgery_Schwartz_5211 | Surgery_Schwartz | Figure 20-49. Angiographic appearance of a Mustard type baffle repair for dTGA.resulted in a physiologic correction, but not an anatomic one, as the systemic circulation is still based on the RV. Still, survival rose to 95% in most centers by using an early balloon septostomy fol-lowed by an atrial switch procedure at 3 to 8 months of age.141,142Despite the improved early survival rates, long-term problems, such as superior vena cava or pulmonary venous obstruction, baffle leak, arrhythmias, tricuspid valve regurgita-tion, and right ventricular failure, prompted the development of the arterial switch procedure by Jatene in 1975.143 The arterial switch procedure involves the division of the aorta and the pul-monary artery, posterior translocation of the aorta (LeCompte maneuver), mobilization of the coronary arteries, placement of a pantaloon-shaped pericardial patch, and proper alignment of the coronary arteries on the neoaorta (Fig. 20-50).The most important consideration is the | Surgery_Schwartz. Figure 20-49. Angiographic appearance of a Mustard type baffle repair for dTGA.resulted in a physiologic correction, but not an anatomic one, as the systemic circulation is still based on the RV. Still, survival rose to 95% in most centers by using an early balloon septostomy fol-lowed by an atrial switch procedure at 3 to 8 months of age.141,142Despite the improved early survival rates, long-term problems, such as superior vena cava or pulmonary venous obstruction, baffle leak, arrhythmias, tricuspid valve regurgita-tion, and right ventricular failure, prompted the development of the arterial switch procedure by Jatene in 1975.143 The arterial switch procedure involves the division of the aorta and the pul-monary artery, posterior translocation of the aorta (LeCompte maneuver), mobilization of the coronary arteries, placement of a pantaloon-shaped pericardial patch, and proper alignment of the coronary arteries on the neoaorta (Fig. 20-50).The most important consideration is the |
Surgery_Schwartz_5212 | Surgery_Schwartz | of the coronary arteries, placement of a pantaloon-shaped pericardial patch, and proper alignment of the coronary arteries on the neoaorta (Fig. 20-50).The most important consideration is the timing of surgical repair because arterial switch should be performed within 2 weeks after birth, before the LV loses its ability to pump against sys-temic afterload. In patients presenting later than 2 weeks, the LV can be retrained with preliminary pulmonary artery banding Brunicardi_Ch20_p0751-p0800.indd 78122/02/19 2:56 PM 782SPECIFIC CONSIDERATIONSPART IIFigure 20-50. The Arterial Switch Operation. A. The maneuver of Lecompte (positioning the pulmo-nary artery anterior to the aorta) is shown with aortic cross-clamp repositioning to retract the pulmonary artery during the neoaortic reconstruction. A and B. After the coronary patches are rotated for an optimal lie, they are sutured to the linearly incised sinuses of Valsalva at the old pulmonary artery (neoaorta) (C). (Reproduced with | Surgery_Schwartz. of the coronary arteries, placement of a pantaloon-shaped pericardial patch, and proper alignment of the coronary arteries on the neoaorta (Fig. 20-50).The most important consideration is the timing of surgical repair because arterial switch should be performed within 2 weeks after birth, before the LV loses its ability to pump against sys-temic afterload. In patients presenting later than 2 weeks, the LV can be retrained with preliminary pulmonary artery banding Brunicardi_Ch20_p0751-p0800.indd 78122/02/19 2:56 PM 782SPECIFIC CONSIDERATIONSPART IIFigure 20-50. The Arterial Switch Operation. A. The maneuver of Lecompte (positioning the pulmo-nary artery anterior to the aorta) is shown with aortic cross-clamp repositioning to retract the pulmonary artery during the neoaortic reconstruction. A and B. After the coronary patches are rotated for an optimal lie, they are sutured to the linearly incised sinuses of Valsalva at the old pulmonary artery (neoaorta) (C). (Reproduced with |
Surgery_Schwartz_5213 | Surgery_Schwartz | A and B. After the coronary patches are rotated for an optimal lie, they are sutured to the linearly incised sinuses of Valsalva at the old pulmonary artery (neoaorta) (C). (Reproduced with permission from Mavroudis C, Backer CL: Arterial Switch. Cardiac Surgery: State of the Art Review. Vol. 5, no. 1. Philadelphia, PA: Hanley & Belfus; 1991.) Figure 20-51. Angiographic appearance of the pulmonary arteries before and after balloon dilation. The RV pressures dropped from “systemic” to “1/2 systemic” after dilation.and aortopulmonary shunt followed by definitive repair. Alter-natively, the unprepared LV can be supported following arterial switch with a mechanical assist device for a few days while it recovers ability to manage systemic pressures. Echocardiogra-phy can be used to assess left ventricular performance and guide operative planning in these circumstances.The subset of patients who present with D-TGA compli-cated by LVOT obstruction and VSD may not be suitable for an arterial | Surgery_Schwartz. A and B. After the coronary patches are rotated for an optimal lie, they are sutured to the linearly incised sinuses of Valsalva at the old pulmonary artery (neoaorta) (C). (Reproduced with permission from Mavroudis C, Backer CL: Arterial Switch. Cardiac Surgery: State of the Art Review. Vol. 5, no. 1. Philadelphia, PA: Hanley & Belfus; 1991.) Figure 20-51. Angiographic appearance of the pulmonary arteries before and after balloon dilation. The RV pressures dropped from “systemic” to “1/2 systemic” after dilation.and aortopulmonary shunt followed by definitive repair. Alter-natively, the unprepared LV can be supported following arterial switch with a mechanical assist device for a few days while it recovers ability to manage systemic pressures. Echocardiogra-phy can be used to assess left ventricular performance and guide operative planning in these circumstances.The subset of patients who present with D-TGA compli-cated by LVOT obstruction and VSD may not be suitable for an arterial |
Surgery_Schwartz_5214 | Surgery_Schwartz | ventricular performance and guide operative planning in these circumstances.The subset of patients who present with D-TGA compli-cated by LVOT obstruction and VSD may not be suitable for an arterial switch operation. The Rastelli operation, first performed in 1968, uses placement of an intracardiac baffle to direct left ventricular blood to the aorta and an extracardiac valved conduit to establish continuity between the RV and the pulmonary artery, which has led to successful outcomes in these complex patients.144Results. For patients with D-TGA, intact ventricular septum, and VSD, the arterial switch operation provides excellent long-term results with a mortality rate of less than 5%. Operative risk is increased when unfavorable coronary anatomic configu-rations are present or when augmentation of the aortic arch is required. The most common complication is supravalvular pul-monary stenosis, occurring 10% of the time, which may require ballooning or reoperation (Fig. | Surgery_Schwartz. ventricular performance and guide operative planning in these circumstances.The subset of patients who present with D-TGA compli-cated by LVOT obstruction and VSD may not be suitable for an arterial switch operation. The Rastelli operation, first performed in 1968, uses placement of an intracardiac baffle to direct left ventricular blood to the aorta and an extracardiac valved conduit to establish continuity between the RV and the pulmonary artery, which has led to successful outcomes in these complex patients.144Results. For patients with D-TGA, intact ventricular septum, and VSD, the arterial switch operation provides excellent long-term results with a mortality rate of less than 5%. Operative risk is increased when unfavorable coronary anatomic configu-rations are present or when augmentation of the aortic arch is required. The most common complication is supravalvular pul-monary stenosis, occurring 10% of the time, which may require ballooning or reoperation (Fig. |
Surgery_Schwartz_5215 | Surgery_Schwartz | or when augmentation of the aortic arch is required. The most common complication is supravalvular pul-monary stenosis, occurring 10% of the time, which may require ballooning or reoperation (Fig. 20-51).145Results of the Rastelli operation have improved substan-tially, with an early mortality rate of 5%.146 Late mortality rate results were less favorable because conduit failure requiring reoperation, pacemaker insertion, or relief of LVOT obstruc-tion was frequent.Brunicardi_Ch20_p0751-p0800.indd 78222/02/19 2:56 PM 783CONGENITAL HEART DISEASECHAPTER 20Double-Outlet Right VentricleAnatomy. Double-outlet RV (DORV) accounts for 5% of CHD and exists when both the aorta and pulmonary artery arise wholly, or in large part, from the RV (Fig. 20-52). DORV encompasses a spectrum of malformations because the incom-plete shift of the aorta toward the LV is often associated with other abnormalities of cardiac development, such as ventricular looping and infundibular-truncal spiraling.147 | Surgery_Schwartz. or when augmentation of the aortic arch is required. The most common complication is supravalvular pul-monary stenosis, occurring 10% of the time, which may require ballooning or reoperation (Fig. 20-51).145Results of the Rastelli operation have improved substan-tially, with an early mortality rate of 5%.146 Late mortality rate results were less favorable because conduit failure requiring reoperation, pacemaker insertion, or relief of LVOT obstruc-tion was frequent.Brunicardi_Ch20_p0751-p0800.indd 78222/02/19 2:56 PM 783CONGENITAL HEART DISEASECHAPTER 20Double-Outlet Right VentricleAnatomy. Double-outlet RV (DORV) accounts for 5% of CHD and exists when both the aorta and pulmonary artery arise wholly, or in large part, from the RV (Fig. 20-52). DORV encompasses a spectrum of malformations because the incom-plete shift of the aorta toward the LV is often associated with other abnormalities of cardiac development, such as ventricular looping and infundibular-truncal spiraling.147 |
Surgery_Schwartz_5216 | Surgery_Schwartz | because the incom-plete shift of the aorta toward the LV is often associated with other abnormalities of cardiac development, such as ventricular looping and infundibular-truncal spiraling.147 The vast majority of hearts exhibiting DORV have a concomitant VSD, which varies in its size and spatial association with the great vessels. The VSD is usually nonrestrictive and represents the only out-flow for the LV; its location relative to the great vessels dictates the dominant physiology of DORV, which can be analogous to that of a large isolated VSD, tetralogy of Fallot, or D-TGA. In 1972, Lev et al148 suggested considering DORV as a spectrum of hearts that “pass imperceptibly from tetralogy with VSD with overriding aorta into double-outlet right ventricle with subaor-tic VSD.” Thus, Lev and colleagues described a classification scheme for DORV based on the “commitment” of the VSD to either or both great arteries.148 The VSD can be subaortic, dou-bly committed, noncommitted, or | Surgery_Schwartz. because the incom-plete shift of the aorta toward the LV is often associated with other abnormalities of cardiac development, such as ventricular looping and infundibular-truncal spiraling.147 The vast majority of hearts exhibiting DORV have a concomitant VSD, which varies in its size and spatial association with the great vessels. The VSD is usually nonrestrictive and represents the only out-flow for the LV; its location relative to the great vessels dictates the dominant physiology of DORV, which can be analogous to that of a large isolated VSD, tetralogy of Fallot, or D-TGA. In 1972, Lev et al148 suggested considering DORV as a spectrum of hearts that “pass imperceptibly from tetralogy with VSD with overriding aorta into double-outlet right ventricle with subaor-tic VSD.” Thus, Lev and colleagues described a classification scheme for DORV based on the “commitment” of the VSD to either or both great arteries.148 The VSD can be subaortic, dou-bly committed, noncommitted, or |
Surgery_Schwartz_5217 | Surgery_Schwartz | Lev and colleagues described a classification scheme for DORV based on the “commitment” of the VSD to either or both great arteries.148 The VSD can be subaortic, dou-bly committed, noncommitted, or subpulmonic.The subaortic type is the most common (47%) and occurs when the VSD is located directly beneath the aortic annulus. Doubly committed VSD (4%) is present when the VSD lies beneath both the aorta and the pulmonary artery, which are usually side-by-side in this lesion. The noncommitted VSD (26%) exists when the VSD is remote from the great vessels. The subset of DORV hearts with the VSD located beneath the pulmonary valve also are classified as the Taussig–Bing syn-drome (Fig. 20-53).149 This occurs in 23% of cases of DORV with VSD, and it occurs when the aorta rotates more anteriorly, with the pulmonary artery rotated more posteriorly.150Clinical Manifestations and Diagnosis. Patients with DORV typically present with one of the following three scenar-ios: (a) those with doubly | Surgery_Schwartz. Lev and colleagues described a classification scheme for DORV based on the “commitment” of the VSD to either or both great arteries.148 The VSD can be subaortic, dou-bly committed, noncommitted, or subpulmonic.The subaortic type is the most common (47%) and occurs when the VSD is located directly beneath the aortic annulus. Doubly committed VSD (4%) is present when the VSD lies beneath both the aorta and the pulmonary artery, which are usually side-by-side in this lesion. The noncommitted VSD (26%) exists when the VSD is remote from the great vessels. The subset of DORV hearts with the VSD located beneath the pulmonary valve also are classified as the Taussig–Bing syn-drome (Fig. 20-53).149 This occurs in 23% of cases of DORV with VSD, and it occurs when the aorta rotates more anteriorly, with the pulmonary artery rotated more posteriorly.150Clinical Manifestations and Diagnosis. Patients with DORV typically present with one of the following three scenar-ios: (a) those with doubly |
Surgery_Schwartz_5218 | Surgery_Schwartz | with the pulmonary artery rotated more posteriorly.150Clinical Manifestations and Diagnosis. Patients with DORV typically present with one of the following three scenar-ios: (a) those with doubly committed or subaortic VSD present with congestive heart failure and a high propensity for pulmo-nary hypertension, much like infants with a large single VSD; (b) those with a subaortic VSD and pulmonary stenosis present with cyanosis and hypoxia, much like infants with tetralogy of Fallot; and (c) those with subpulmonic VSD present with cya-nosis, much like those with D-TGA, because streaming directs desaturated systemic venous blood to the aorta and oxygenated blood to the pulmonary artery.140 Thus, the three critical factors influencing the clinical presentation and subsequent manage-ment of infants with DORV are the size and location of the VSD, the presence or absence of important RVOT obstruc-tion, and the presence of other anomalies (especially associ-ated hypoplasia of left-sided | Surgery_Schwartz. with the pulmonary artery rotated more posteriorly.150Clinical Manifestations and Diagnosis. Patients with DORV typically present with one of the following three scenar-ios: (a) those with doubly committed or subaortic VSD present with congestive heart failure and a high propensity for pulmo-nary hypertension, much like infants with a large single VSD; (b) those with a subaortic VSD and pulmonary stenosis present with cyanosis and hypoxia, much like infants with tetralogy of Fallot; and (c) those with subpulmonic VSD present with cya-nosis, much like those with D-TGA, because streaming directs desaturated systemic venous blood to the aorta and oxygenated blood to the pulmonary artery.140 Thus, the three critical factors influencing the clinical presentation and subsequent manage-ment of infants with DORV are the size and location of the VSD, the presence or absence of important RVOT obstruc-tion, and the presence of other anomalies (especially associ-ated hypoplasia of left-sided |
Surgery_Schwartz_5219 | Surgery_Schwartz | of infants with DORV are the size and location of the VSD, the presence or absence of important RVOT obstruc-tion, and the presence of other anomalies (especially associ-ated hypoplasia of left-sided structures sometimes seen with subpulmonary VSD).Echocardiography is the mainstay of diagnosis and can also provide valuable information regarding the feasibility of biventricular repair. Specific anatomic questions that should be resolved to assist in surgical planning in addition to those mentioned earlier include the coronary anatomy (presence of a conal branch or left anterior descending from the right coronary coursing across the conus), the presence of additional muscular VSDs remote from either great vessel, and the distance between the tricuspid and pulmonary valve. Cardiac catheterization is rarely necessary in neonates or infants, except to determine the degree of pulmonary hypertension and to determine the effects of previous palliative procedures on the pulmonary arterial | Surgery_Schwartz. of infants with DORV are the size and location of the VSD, the presence or absence of important RVOT obstruc-tion, and the presence of other anomalies (especially associ-ated hypoplasia of left-sided structures sometimes seen with subpulmonary VSD).Echocardiography is the mainstay of diagnosis and can also provide valuable information regarding the feasibility of biventricular repair. Specific anatomic questions that should be resolved to assist in surgical planning in addition to those mentioned earlier include the coronary anatomy (presence of a conal branch or left anterior descending from the right coronary coursing across the conus), the presence of additional muscular VSDs remote from either great vessel, and the distance between the tricuspid and pulmonary valve. Cardiac catheterization is rarely necessary in neonates or infants, except to determine the degree of pulmonary hypertension and to determine the effects of previous palliative procedures on the pulmonary arterial |
Surgery_Schwartz_5220 | Surgery_Schwartz | is rarely necessary in neonates or infants, except to determine the degree of pulmonary hypertension and to determine the effects of previous palliative procedures on the pulmonary arterial anatomy.Therapy. The goals of corrective surgery are to relieve pul-monary stenosis, to provide separate and unobstructed outflow pathways from each ventricle to the correct great vessel, and to achieve separation of the systemic and pulmonary circulations.Double-Outlet Right Ventricle With Noncommitted Ventricular Septal DefectThe repair of hearts with DORV and noncommitted VSD can be accomplished by constructing an intraventricular tunnel con-necting the VSD to the aorta, closing the pulmonary artery, and placing a valved extracardiac conduit from the RV to the pulmonary artery. In patients without pulmonary stenosis who have intractable congestive failure, a pulmonary artery band can be placed in the first 6 months to control pulmonary artery Figure 20-53. Angiographic appearance of the aorta in | Surgery_Schwartz. is rarely necessary in neonates or infants, except to determine the degree of pulmonary hypertension and to determine the effects of previous palliative procedures on the pulmonary arterial anatomy.Therapy. The goals of corrective surgery are to relieve pul-monary stenosis, to provide separate and unobstructed outflow pathways from each ventricle to the correct great vessel, and to achieve separation of the systemic and pulmonary circulations.Double-Outlet Right Ventricle With Noncommitted Ventricular Septal DefectThe repair of hearts with DORV and noncommitted VSD can be accomplished by constructing an intraventricular tunnel con-necting the VSD to the aorta, closing the pulmonary artery, and placing a valved extracardiac conduit from the RV to the pulmonary artery. In patients without pulmonary stenosis who have intractable congestive failure, a pulmonary artery band can be placed in the first 6 months to control pulmonary artery Figure 20-53. Angiographic appearance of the aorta in |
Surgery_Schwartz_5221 | Surgery_Schwartz | stenosis who have intractable congestive failure, a pulmonary artery band can be placed in the first 6 months to control pulmonary artery Figure 20-53. Angiographic appearance of the aorta in a patient with Taussig-Bing anomaly. Note the hypoplastic arch (‘*’).Figure 20-52. DORV, aortomitral discontinuity (‘*’), aorta mostly arising from RV (arrow).Brunicardi_Ch20_p0751-p0800.indd 78322/02/19 2:56 PM 784SPECIFIC CONSIDERATIONSPART IIovercirculation and prevent the development of pulmonary hypertension.Infants with pulmonary stenosis can be managed with a systemic-to-pulmonary shunt followed by biventricular repair as described by Belli and colleagues in 1999, or with a modi-fied Fontan.151 There is no consensus on the timing of repair, but recent literature suggests that repair within the first 6 months is associated with better outcome. However, in cases where an extracardiac-valved conduit is necessary, it is better to delay definitive repair until the child is 2 to 3 years of | Surgery_Schwartz. stenosis who have intractable congestive failure, a pulmonary artery band can be placed in the first 6 months to control pulmonary artery Figure 20-53. Angiographic appearance of the aorta in a patient with Taussig-Bing anomaly. Note the hypoplastic arch (‘*’).Figure 20-52. DORV, aortomitral discontinuity (‘*’), aorta mostly arising from RV (arrow).Brunicardi_Ch20_p0751-p0800.indd 78322/02/19 2:56 PM 784SPECIFIC CONSIDERATIONSPART IIovercirculation and prevent the development of pulmonary hypertension.Infants with pulmonary stenosis can be managed with a systemic-to-pulmonary shunt followed by biventricular repair as described by Belli and colleagues in 1999, or with a modi-fied Fontan.151 There is no consensus on the timing of repair, but recent literature suggests that repair within the first 6 months is associated with better outcome. However, in cases where an extracardiac-valved conduit is necessary, it is better to delay definitive repair until the child is 2 to 3 years of |
Surgery_Schwartz_5222 | Surgery_Schwartz | the first 6 months is associated with better outcome. However, in cases where an extracardiac-valved conduit is necessary, it is better to delay definitive repair until the child is 2 to 3 years of age because this allows placement of a larger conduit and possibly reduces the number of future obligatory conduit replacements.147Double-Outlet Right Ventricle With Subaortic or Doubly Committed Ventricular Septal Defect Without Pulmonary StenosisThis group of patients can be treated by creating an intracardiac baffle that directs blood from the LV into the aorta. Enlargement of the VSD may be necessary to allow ample room for the baf-fle; this should be done anterosuperiorly to avoid injury to the conduction system that normally lies inferoposteriorly along the border of the VSD. In addition, other important considerations in constructing the LV outflow tunnel include the prominence of the conal septum, the attachments of the tricuspid valve to the conal septum, and the distance between | Surgery_Schwartz. the first 6 months is associated with better outcome. However, in cases where an extracardiac-valved conduit is necessary, it is better to delay definitive repair until the child is 2 to 3 years of age because this allows placement of a larger conduit and possibly reduces the number of future obligatory conduit replacements.147Double-Outlet Right Ventricle With Subaortic or Doubly Committed Ventricular Septal Defect Without Pulmonary StenosisThis group of patients can be treated by creating an intracardiac baffle that directs blood from the LV into the aorta. Enlargement of the VSD may be necessary to allow ample room for the baf-fle; this should be done anterosuperiorly to avoid injury to the conduction system that normally lies inferoposteriorly along the border of the VSD. In addition, other important considerations in constructing the LV outflow tunnel include the prominence of the conal septum, the attachments of the tricuspid valve to the conal septum, and the distance between |
Surgery_Schwartz_5223 | Surgery_Schwartz | other important considerations in constructing the LV outflow tunnel include the prominence of the conal septum, the attachments of the tricuspid valve to the conal septum, and the distance between the tricuspid and pulmonary valves. In some instances, unfavorable anatomy may preclude placement of an adequate intracardiac baffle, neces-sitating single ventricle repair.Double-Outlet Right Ventricle With Subaortic or Doubly Committed Ventricular Septal Defect With Pulmonary StenosisRepair of this defect is similar to the above except that concomi-tant RVOT reconstruction must be performed in addition to the intracardiac tunnel. The RVOT augmentation can be accom-plished with the placement of a transannular patch or with place-ment of an extracardiac-valved conduit when an anomalous left anterior descending artery precludes use of a patch.Taussig–Bing Syndrome Without Pulmonary StenosisThese infants are best treated with a balloon septostomy dur-ing the neonatal period to improve | Surgery_Schwartz. other important considerations in constructing the LV outflow tunnel include the prominence of the conal septum, the attachments of the tricuspid valve to the conal septum, and the distance between the tricuspid and pulmonary valves. In some instances, unfavorable anatomy may preclude placement of an adequate intracardiac baffle, neces-sitating single ventricle repair.Double-Outlet Right Ventricle With Subaortic or Doubly Committed Ventricular Septal Defect With Pulmonary StenosisRepair of this defect is similar to the above except that concomi-tant RVOT reconstruction must be performed in addition to the intracardiac tunnel. The RVOT augmentation can be accom-plished with the placement of a transannular patch or with place-ment of an extracardiac-valved conduit when an anomalous left anterior descending artery precludes use of a patch.Taussig–Bing Syndrome Without Pulmonary StenosisThese infants are best treated with a balloon septostomy dur-ing the neonatal period to improve |
Surgery_Schwartz_5224 | Surgery_Schwartz | anterior descending artery precludes use of a patch.Taussig–Bing Syndrome Without Pulmonary StenosisThese infants are best treated with a balloon septostomy dur-ing the neonatal period to improve mixing, followed by VSD closure baffling LV egress to the pulmonary artery and an arte-rial switch operation. The Kawashima procedure,152 in which an intraventricular tunnel is used to baffle LV egress directly to the aorta, may alternatively be used when the aorta is more posterior or when there is associated pulmonary stenosis.Taussig–Bing Syndrome With Pulmonary StenosisThis defect may be treated with a variety of techniques, depend-ing on the specific anatomic details and the expertise of the treat-ment team. A Rastelli-type repair, which involves construction of an intraventricular tunnel through the existing VSD that con-nects the LV to both great vessels, followed by division of the pulmonary artery at its origin and insertion of a valved conduit from the RV to the distal pulmonary | Surgery_Schwartz. anterior descending artery precludes use of a patch.Taussig–Bing Syndrome Without Pulmonary StenosisThese infants are best treated with a balloon septostomy dur-ing the neonatal period to improve mixing, followed by VSD closure baffling LV egress to the pulmonary artery and an arte-rial switch operation. The Kawashima procedure,152 in which an intraventricular tunnel is used to baffle LV egress directly to the aorta, may alternatively be used when the aorta is more posterior or when there is associated pulmonary stenosis.Taussig–Bing Syndrome With Pulmonary StenosisThis defect may be treated with a variety of techniques, depend-ing on the specific anatomic details and the expertise of the treat-ment team. A Rastelli-type repair, which involves construction of an intraventricular tunnel through the existing VSD that con-nects the LV to both great vessels, followed by division of the pulmonary artery at its origin and insertion of a valved conduit from the RV to the distal pulmonary |
Surgery_Schwartz_5225 | Surgery_Schwartz | through the existing VSD that con-nects the LV to both great vessels, followed by division of the pulmonary artery at its origin and insertion of a valved conduit from the RV to the distal pulmonary artery, can be performed.153 Alternatively, a Yasui procedure, which involves baffling the VSD to the pulmonary artery and creation of a DKS anastomo-sis between the pulmonary artery and the aorta with patch aug-mentation, can be accomplished concomitant with placement of an RV pulmonary artery conduit.154Results. The results of DORV repairs are generally favor-able, especially for the tetralogy-type DORV with subaortic VSD.150,155 However, more complex types of DORV, including noncommitted VSD and Taussig–Bing type, still carry impor-tant morbidity and mortality.150,151,155 Furthermore, repeated interventions for RVOT reconstruction or staged operations for patients triaged to single-ventricle pathways pose late hazards for patients surviving initial repair. A single-institution series | Surgery_Schwartz. through the existing VSD that con-nects the LV to both great vessels, followed by division of the pulmonary artery at its origin and insertion of a valved conduit from the RV to the distal pulmonary artery, can be performed.153 Alternatively, a Yasui procedure, which involves baffling the VSD to the pulmonary artery and creation of a DKS anastomo-sis between the pulmonary artery and the aorta with patch aug-mentation, can be accomplished concomitant with placement of an RV pulmonary artery conduit.154Results. The results of DORV repairs are generally favor-able, especially for the tetralogy-type DORV with subaortic VSD.150,155 However, more complex types of DORV, including noncommitted VSD and Taussig–Bing type, still carry impor-tant morbidity and mortality.150,151,155 Furthermore, repeated interventions for RVOT reconstruction or staged operations for patients triaged to single-ventricle pathways pose late hazards for patients surviving initial repair. A single-institution series |
Surgery_Schwartz_5226 | Surgery_Schwartz | interventions for RVOT reconstruction or staged operations for patients triaged to single-ventricle pathways pose late hazards for patients surviving initial repair. A single-institution series evaluated 393 patients with DORV.150 The authors found that the need for reintervention approached 37% at 15 years follow-ing repair. Arterial switch operation, as opposed to Rastelli-type repair, was associated with an increased risk of early postrepair mortality, but mitigated against the risk of late death. Patients with hypoplastic left-sided structures and a nonsubaortic VSD may fare better with a single-ventricle repair.Tetralogy of FallotAnatomy. The original description of tetralogy of Fallot (TOF) by Ettienne Louis Fallot,156 as the name implies, included four abnormalities: a large perimembranous VSD adjacent to the tri-cuspid valve; an overriding aorta; a variable degree of RVOT obstruction, which might include hypoplasia and dysplasia of the pulmonary valve as well as obstruction at | Surgery_Schwartz. interventions for RVOT reconstruction or staged operations for patients triaged to single-ventricle pathways pose late hazards for patients surviving initial repair. A single-institution series evaluated 393 patients with DORV.150 The authors found that the need for reintervention approached 37% at 15 years follow-ing repair. Arterial switch operation, as opposed to Rastelli-type repair, was associated with an increased risk of early postrepair mortality, but mitigated against the risk of late death. Patients with hypoplastic left-sided structures and a nonsubaortic VSD may fare better with a single-ventricle repair.Tetralogy of FallotAnatomy. The original description of tetralogy of Fallot (TOF) by Ettienne Louis Fallot,156 as the name implies, included four abnormalities: a large perimembranous VSD adjacent to the tri-cuspid valve; an overriding aorta; a variable degree of RVOT obstruction, which might include hypoplasia and dysplasia of the pulmonary valve as well as obstruction at |
Surgery_Schwartz_5227 | Surgery_Schwartz | VSD adjacent to the tri-cuspid valve; an overriding aorta; a variable degree of RVOT obstruction, which might include hypoplasia and dysplasia of the pulmonary valve as well as obstruction at the subvalvar and pulmonary artery level; and right ventricular hypertrophy. More recently, the Van Praagh et al157 pointed out that TOF could be more correctly termed monology of Fallot, since the four com-ponents are explained by the malposition of the infundibular sep-tum. When the infundibular septum is displaced anteriorly and leftward, the RVOT is narrowed and its anterior displacement results in failure of fusion of the ventricular septum between the arms of the trabeculo-septo-marginalis (Fig. 20-54).The morphology of TOF is markedly heterogeneous and includes an absent pulmonary valve, concomitant AV septal defects, and pulmonary atresia with major aortopulmonary collaterals. The present discussion will focus only on the so-called classic presentation of TOF without coexisting | Surgery_Schwartz. VSD adjacent to the tri-cuspid valve; an overriding aorta; a variable degree of RVOT obstruction, which might include hypoplasia and dysplasia of the pulmonary valve as well as obstruction at the subvalvar and pulmonary artery level; and right ventricular hypertrophy. More recently, the Van Praagh et al157 pointed out that TOF could be more correctly termed monology of Fallot, since the four com-ponents are explained by the malposition of the infundibular sep-tum. When the infundibular septum is displaced anteriorly and leftward, the RVOT is narrowed and its anterior displacement results in failure of fusion of the ventricular septum between the arms of the trabeculo-septo-marginalis (Fig. 20-54).The morphology of TOF is markedly heterogeneous and includes an absent pulmonary valve, concomitant AV septal defects, and pulmonary atresia with major aortopulmonary collaterals. The present discussion will focus only on the so-called classic presentation of TOF without coexisting |
Surgery_Schwartz_5228 | Surgery_Schwartz | concomitant AV septal defects, and pulmonary atresia with major aortopulmonary collaterals. The present discussion will focus only on the so-called classic presentation of TOF without coexisting intracardiac defects.Anomalous coronary artery patterns, related to either ori-gin or distribution, have been described in TOF.158 However, the most surgically important coronary anomaly occurs when AortaMPAVSDMultilevelpulmonary stenosisRVHFigure 20-54. Tetrology of Fallot. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 78422/02/19 2:56 PM 785CONGENITAL HEART DISEASECHAPTER 20the left anterior descending artery arises as a branch of the right coronary artery. This occurs in approximately 3% of cases of TOF and may preclude placement of a transannular patch, as the left anterior descending coronary artery crosses the RVOT at varying distances from the pulmonary valve annulus.159Pathophysiology and Clinical Presentation. The initial presentation of a child | Surgery_Schwartz. concomitant AV septal defects, and pulmonary atresia with major aortopulmonary collaterals. The present discussion will focus only on the so-called classic presentation of TOF without coexisting intracardiac defects.Anomalous coronary artery patterns, related to either ori-gin or distribution, have been described in TOF.158 However, the most surgically important coronary anomaly occurs when AortaMPAVSDMultilevelpulmonary stenosisRVHFigure 20-54. Tetrology of Fallot. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 78422/02/19 2:56 PM 785CONGENITAL HEART DISEASECHAPTER 20the left anterior descending artery arises as a branch of the right coronary artery. This occurs in approximately 3% of cases of TOF and may preclude placement of a transannular patch, as the left anterior descending coronary artery crosses the RVOT at varying distances from the pulmonary valve annulus.159Pathophysiology and Clinical Presentation. The initial presentation of a child |
Surgery_Schwartz_5229 | Surgery_Schwartz | the left anterior descending coronary artery crosses the RVOT at varying distances from the pulmonary valve annulus.159Pathophysiology and Clinical Presentation. The initial presentation of a child afflicted with TOF depends on the degree of RVOT obstruction. Children with cyanosis at birth usually have severe pulmonary annular hypoplasia with concomitant hypoplasia of the peripheral pulmonary arteries. Most children, however, present with mild cyanosis at birth, which then pro-gresses as the right ventricular hypertrophy further compromises the RVOT. Cyanosis usually becomes significant within the first 6 to 12 months of life, and the child may develop characteristic “tet” spells, which are periods of extreme hypoxemia. These spells are characterized by decreased pulmonary blood flow and an increase in systemic blood flow. They can be triggered by any stimulus that decreases systemic vascular resistance, such as fever, agitation, or vigorous physical activity. Cyanotic spells | Surgery_Schwartz. the left anterior descending coronary artery crosses the RVOT at varying distances from the pulmonary valve annulus.159Pathophysiology and Clinical Presentation. The initial presentation of a child afflicted with TOF depends on the degree of RVOT obstruction. Children with cyanosis at birth usually have severe pulmonary annular hypoplasia with concomitant hypoplasia of the peripheral pulmonary arteries. Most children, however, present with mild cyanosis at birth, which then pro-gresses as the right ventricular hypertrophy further compromises the RVOT. Cyanosis usually becomes significant within the first 6 to 12 months of life, and the child may develop characteristic “tet” spells, which are periods of extreme hypoxemia. These spells are characterized by decreased pulmonary blood flow and an increase in systemic blood flow. They can be triggered by any stimulus that decreases systemic vascular resistance, such as fever, agitation, or vigorous physical activity. Cyanotic spells |
Surgery_Schwartz_5230 | Surgery_Schwartz | and an increase in systemic blood flow. They can be triggered by any stimulus that decreases systemic vascular resistance, such as fever, agitation, or vigorous physical activity. Cyanotic spells increase in severity and frequency as the child grows, and older patients with uncorrected TOF may often squat, which increases peripheral vascular resistance and relieves the cyanosis.Evaluation in the older patient with TOF may demonstrate clubbing, polycythemia, hemoptysis, or brain abscesses. Chest radiography will demonstrate a boot-shaped heart (Fig. 20-55), and EKG will show the normal pattern of right ventricular hypertrophy. Echocardiography confirms the diagnosis because it demonstrates the position and nature of the VSD, defines the character of the RVOT obstruction, and often visualizes the branch pulmonary arteries and the proximal coronary arteries. Cardiac catheterization is rarely necessary and is actually risky in TOF since it can create spasm of the RVOT muscle and result in | Surgery_Schwartz. and an increase in systemic blood flow. They can be triggered by any stimulus that decreases systemic vascular resistance, such as fever, agitation, or vigorous physical activity. Cyanotic spells increase in severity and frequency as the child grows, and older patients with uncorrected TOF may often squat, which increases peripheral vascular resistance and relieves the cyanosis.Evaluation in the older patient with TOF may demonstrate clubbing, polycythemia, hemoptysis, or brain abscesses. Chest radiography will demonstrate a boot-shaped heart (Fig. 20-55), and EKG will show the normal pattern of right ventricular hypertrophy. Echocardiography confirms the diagnosis because it demonstrates the position and nature of the VSD, defines the character of the RVOT obstruction, and often visualizes the branch pulmonary arteries and the proximal coronary arteries. Cardiac catheterization is rarely necessary and is actually risky in TOF since it can create spasm of the RVOT muscle and result in |
Surgery_Schwartz_5231 | Surgery_Schwartz | the branch pulmonary arteries and the proximal coronary arteries. Cardiac catheterization is rarely necessary and is actually risky in TOF since it can create spasm of the RVOT muscle and result in a hypercyanotic episode (tet spell). Occasionally, aortogra-phy (Fig. 20-56) is necessary to delineate the coronary artery anatomy.Treatment. John Deanfield160 stated “…long follow-up inevi-tably means surgery in an earlier era: More recent surgery, at a younger age, with better preoperative, operative, and post-operative care, will improve long-term results. Data from the former (earlier) era will be overly pessimistic.” This statement is particularly pertinent as surgical correction of TOF has evolved from a staged approach of antecedent palliation in infancy fol-lowed by intracardiac repair to primary repair during the first few months of life without prior palliative surgery.However, systemic-to-pulmonary shunts, generally an MBTS, may still be preferred with an unstable neonate younger | Surgery_Schwartz. the branch pulmonary arteries and the proximal coronary arteries. Cardiac catheterization is rarely necessary and is actually risky in TOF since it can create spasm of the RVOT muscle and result in a hypercyanotic episode (tet spell). Occasionally, aortogra-phy (Fig. 20-56) is necessary to delineate the coronary artery anatomy.Treatment. John Deanfield160 stated “…long follow-up inevi-tably means surgery in an earlier era: More recent surgery, at a younger age, with better preoperative, operative, and post-operative care, will improve long-term results. Data from the former (earlier) era will be overly pessimistic.” This statement is particularly pertinent as surgical correction of TOF has evolved from a staged approach of antecedent palliation in infancy fol-lowed by intracardiac repair to primary repair during the first few months of life without prior palliative surgery.However, systemic-to-pulmonary shunts, generally an MBTS, may still be preferred with an unstable neonate younger |
Surgery_Schwartz_5232 | Surgery_Schwartz | primary repair during the first few months of life without prior palliative surgery.However, systemic-to-pulmonary shunts, generally an MBTS, may still be preferred with an unstable neonate younger than 3 months of age, when an extracardiac conduit is required because of an anomalous left anterior descending coronary artery, or when pulmonary atresia, significant branch pulmo-nary artery hypoplasia, or severe noncardiac anomalies coexist with TOF.Traditionally, TOF was repaired through a right ventricu-lotomy, providing excellent exposure for closure of the VSD and relief of the RVOT obstruction, but concerns that the resul-tant scar would significantly impair right ventricular function or lead to lethal arrhythmias led to the development of a transatrial approach. Transatrial repair, except in cases when the presence of diffuse RVOT hypoplasia requires insertion of a transannular patch, is now being increasingly advocated by many, although its superiority has not been conclusively | Surgery_Schwartz. primary repair during the first few months of life without prior palliative surgery.However, systemic-to-pulmonary shunts, generally an MBTS, may still be preferred with an unstable neonate younger than 3 months of age, when an extracardiac conduit is required because of an anomalous left anterior descending coronary artery, or when pulmonary atresia, significant branch pulmo-nary artery hypoplasia, or severe noncardiac anomalies coexist with TOF.Traditionally, TOF was repaired through a right ventricu-lotomy, providing excellent exposure for closure of the VSD and relief of the RVOT obstruction, but concerns that the resul-tant scar would significantly impair right ventricular function or lead to lethal arrhythmias led to the development of a transatrial approach. Transatrial repair, except in cases when the presence of diffuse RVOT hypoplasia requires insertion of a transannular patch, is now being increasingly advocated by many, although its superiority has not been conclusively |
Surgery_Schwartz_5233 | Surgery_Schwartz | in cases when the presence of diffuse RVOT hypoplasia requires insertion of a transannular patch, is now being increasingly advocated by many, although its superiority has not been conclusively demonstrated.161The operative technique involves the use of CPB. All existing systemic-to-pulmonary arterial shunts, as well as the ductus arteriosus, are ligated. A right atriotomy is then made, and the anatomy of the VSD and the RVOT are assessed by retracting the tricuspid valve. The outflow tract obstruction is relieved by resecting the offending portion of the infundibular septum as well as any muscle trabeculations. If necessary, a pul-monary valvotomy or, alternatively, a longitudinal incision in the main pulmonary artery can be performed to improve expo-sure. The diameter of the pulmonary valve annulus is assessed by inserting Hegar dilators across the outflow tract; if the pul-monary artery/aorta diameter is less than 0.5, or the estimated RV/LV pressure is greater than 0.7, or the | Surgery_Schwartz. in cases when the presence of diffuse RVOT hypoplasia requires insertion of a transannular patch, is now being increasingly advocated by many, although its superiority has not been conclusively demonstrated.161The operative technique involves the use of CPB. All existing systemic-to-pulmonary arterial shunts, as well as the ductus arteriosus, are ligated. A right atriotomy is then made, and the anatomy of the VSD and the RVOT are assessed by retracting the tricuspid valve. The outflow tract obstruction is relieved by resecting the offending portion of the infundibular septum as well as any muscle trabeculations. If necessary, a pul-monary valvotomy or, alternatively, a longitudinal incision in the main pulmonary artery can be performed to improve expo-sure. The diameter of the pulmonary valve annulus is assessed by inserting Hegar dilators across the outflow tract; if the pul-monary artery/aorta diameter is less than 0.5, or the estimated RV/LV pressure is greater than 0.7, or the |
Surgery_Schwartz_5234 | Surgery_Schwartz | valve annulus is assessed by inserting Hegar dilators across the outflow tract; if the pul-monary artery/aorta diameter is less than 0.5, or the estimated RV/LV pressure is greater than 0.7, or the size of the pulmo-nary valve is less than a Z score of −2.5, a transannular patch is inserted. Patch closure of the VSD is then accomplished, taking Figure 20-56. CT aortogram showing the large aorta often associated with conotruncal anomalies, rotated coronaries, and extremely hypoplastic main and branch pulmonary arteries in a patient with TOF.Figure 20-55. Chest x-ray showing a boot shaped heart in an infant with tetralogy of Fallot.Brunicardi_Ch20_p0751-p0800.indd 78522/02/19 2:56 PM 786SPECIFIC CONSIDERATIONSPART IIcare when placing sutures along the posteroinferior portion to avoid the conduction system.Results. Operative mortality for primary repair of TOF in infancy is less than 5% in most series.161 Previously reported risk factors such as transannular patch insertion or | Surgery_Schwartz. valve annulus is assessed by inserting Hegar dilators across the outflow tract; if the pul-monary artery/aorta diameter is less than 0.5, or the estimated RV/LV pressure is greater than 0.7, or the size of the pulmo-nary valve is less than a Z score of −2.5, a transannular patch is inserted. Patch closure of the VSD is then accomplished, taking Figure 20-56. CT aortogram showing the large aorta often associated with conotruncal anomalies, rotated coronaries, and extremely hypoplastic main and branch pulmonary arteries in a patient with TOF.Figure 20-55. Chest x-ray showing a boot shaped heart in an infant with tetralogy of Fallot.Brunicardi_Ch20_p0751-p0800.indd 78522/02/19 2:56 PM 786SPECIFIC CONSIDERATIONSPART IIcare when placing sutures along the posteroinferior portion to avoid the conduction system.Results. Operative mortality for primary repair of TOF in infancy is less than 5% in most series.161 Previously reported risk factors such as transannular patch insertion or |
Surgery_Schwartz_5235 | Surgery_Schwartz | the conduction system.Results. Operative mortality for primary repair of TOF in infancy is less than 5% in most series.161 Previously reported risk factors such as transannular patch insertion or younger age at time of repair have been eliminated secondary to improved intraoperative and postoperative care. According to the Society of Thoracic Surgeons Congenital Heart Surgery Database, dis-charge mortality from 3059 operations from 2002 to 2007 was 7.5% for initial palliation, 1.3% for primary repair, and 0.9% for staged repair, indicating similar outcomes for patients get-ting primary repair compared to staged repair.162 Nevertheless, for neonatal repair, discharge mortality increased to 6.2% with palliation and 7.8% with primary repair. This may be partly explained by a higher chance of postoperative complications in neonates.A major complication of repaired TOF is the develop-ment of pulmonary insufficiency, which subjects the RV to the adverse effects of acute and chronic volume | Surgery_Schwartz. the conduction system.Results. Operative mortality for primary repair of TOF in infancy is less than 5% in most series.161 Previously reported risk factors such as transannular patch insertion or younger age at time of repair have been eliminated secondary to improved intraoperative and postoperative care. According to the Society of Thoracic Surgeons Congenital Heart Surgery Database, dis-charge mortality from 3059 operations from 2002 to 2007 was 7.5% for initial palliation, 1.3% for primary repair, and 0.9% for staged repair, indicating similar outcomes for patients get-ting primary repair compared to staged repair.162 Nevertheless, for neonatal repair, discharge mortality increased to 6.2% with palliation and 7.8% with primary repair. This may be partly explained by a higher chance of postoperative complications in neonates.A major complication of repaired TOF is the develop-ment of pulmonary insufficiency, which subjects the RV to the adverse effects of acute and chronic volume |
Surgery_Schwartz_5236 | Surgery_Schwartz | postoperative complications in neonates.A major complication of repaired TOF is the develop-ment of pulmonary insufficiency, which subjects the RV to the adverse effects of acute and chronic volume overload. This is especially problematic if residual lesions such as a VSD or peripheral pulmonary stenosis exist. Pulmonary valve regurgita-tion after repair of TOF is relatively well tolerated in the short term, partly because the hypertrophied RV usually adapts to the altered hemodynamic load.163 The detrimental effects of chronic pulmonary valve regurgitation are, however, numerous, and include progressive right ventricular dilatation and failure, tri-cuspid valve regurgitation, exercise intolerance, arrhythmia, and sudden death. Mechanoelectrical interaction, by which a dila-tated RV provides the substrate for electrical instability, might underlie the propensity toward ventricular arrhythmia.164 In sup-port of this contention, Gatzoulis and colleagues163,164 found that the risk of | Surgery_Schwartz. postoperative complications in neonates.A major complication of repaired TOF is the develop-ment of pulmonary insufficiency, which subjects the RV to the adverse effects of acute and chronic volume overload. This is especially problematic if residual lesions such as a VSD or peripheral pulmonary stenosis exist. Pulmonary valve regurgita-tion after repair of TOF is relatively well tolerated in the short term, partly because the hypertrophied RV usually adapts to the altered hemodynamic load.163 The detrimental effects of chronic pulmonary valve regurgitation are, however, numerous, and include progressive right ventricular dilatation and failure, tri-cuspid valve regurgitation, exercise intolerance, arrhythmia, and sudden death. Mechanoelectrical interaction, by which a dila-tated RV provides the substrate for electrical instability, might underlie the propensity toward ventricular arrhythmia.164 In sup-port of this contention, Gatzoulis and colleagues163,164 found that the risk of |
Surgery_Schwartz_5237 | Surgery_Schwartz | the substrate for electrical instability, might underlie the propensity toward ventricular arrhythmia.164 In sup-port of this contention, Gatzoulis and colleagues163,164 found that the risk of symptomatic arrhythmia was high in patients with marked right ventricular enlargement and QRS prolongation on resting ECG of more than 180 ms. Karamlou et al have shown that similar structural and hemodynamic abnormalities, including a larger right atrial volume and right ventricular chamber size, are also related to atrial arrhythmias in patients following TOF repair.165 We found that prolongation of the QRS duration beyond a threshold of 160 ms increased the risk of atrial arrhythmias.165 Together, these data show that a similar mechanism could be responsible for both atrial and ventricular arrhythmias after repair in TOF patients.When significant deterioration of ventricular func-tion occurs, insertion of a pulmonary valve may be required, although this is rarely necessary in infants. | Surgery_Schwartz. the substrate for electrical instability, might underlie the propensity toward ventricular arrhythmia.164 In sup-port of this contention, Gatzoulis and colleagues163,164 found that the risk of symptomatic arrhythmia was high in patients with marked right ventricular enlargement and QRS prolongation on resting ECG of more than 180 ms. Karamlou et al have shown that similar structural and hemodynamic abnormalities, including a larger right atrial volume and right ventricular chamber size, are also related to atrial arrhythmias in patients following TOF repair.165 We found that prolongation of the QRS duration beyond a threshold of 160 ms increased the risk of atrial arrhythmias.165 Together, these data show that a similar mechanism could be responsible for both atrial and ventricular arrhythmias after repair in TOF patients.When significant deterioration of ventricular func-tion occurs, insertion of a pulmonary valve may be required, although this is rarely necessary in infants. |
Surgery_Schwartz_5238 | Surgery_Schwartz | arrhythmias after repair in TOF patients.When significant deterioration of ventricular func-tion occurs, insertion of a pulmonary valve may be required, although this is rarely necessary in infants. Unfortunately, there are no universal criteria establishing the timing of pulmonary valve replacement. The current criteria for pulmonary valve replacement are the presence of two of the following criteria: RVEDD index >160 ml/m2, RVEDI >70 ml/m2, LVEDV index >65 ml/m2, RVEF <45%, RVOT aneurysm, and clinical symp-toms or signs, including syncope or VT.166 PVR can be achieved with minimal morbidity and mortality.167The alternative to surgical PVR is percutaneous pulmo-nary valve implantation. The Melody valve system (Fig. 20-57) is the most popular of such systems. Following risk adjustment, no significant differences were observed between surgical or transcatheter PVR. However, transcatheter PVR was associated with a shorter hospitalization. Hospitalization costs are similar for both | Surgery_Schwartz. arrhythmias after repair in TOF patients.When significant deterioration of ventricular func-tion occurs, insertion of a pulmonary valve may be required, although this is rarely necessary in infants. Unfortunately, there are no universal criteria establishing the timing of pulmonary valve replacement. The current criteria for pulmonary valve replacement are the presence of two of the following criteria: RVEDD index >160 ml/m2, RVEDI >70 ml/m2, LVEDV index >65 ml/m2, RVEF <45%, RVOT aneurysm, and clinical symp-toms or signs, including syncope or VT.166 PVR can be achieved with minimal morbidity and mortality.167The alternative to surgical PVR is percutaneous pulmo-nary valve implantation. The Melody valve system (Fig. 20-57) is the most popular of such systems. Following risk adjustment, no significant differences were observed between surgical or transcatheter PVR. However, transcatheter PVR was associated with a shorter hospitalization. Hospitalization costs are similar for both |
Surgery_Schwartz_5239 | Surgery_Schwartz | no significant differences were observed between surgical or transcatheter PVR. However, transcatheter PVR was associated with a shorter hospitalization. Hospitalization costs are similar for both procedures.168Arrhythmias are potentially the most serious late complication following TOF repair. In a multicenter cohort of 793 patients studied by Gatzoulis et al,164 a steady increase was documented in the prevalence of ventricular and atrial tachyarrhythmia and sudden cardiac death in the first 5 to 10 years after intracardiac repair. Clinical events were reported in 12% of patients at 35 years after repair. Prevalence of atrial arrhythmias from other studies, however, ranges from 1% to 11%,163,164 which is a reflection of the strong time dependence of arrhythmia onset.Underlying causes of arrhythmia following repair are complex and multifactorial, resulting in poorly defined opti-mum screening and treatment algorithms. Older repair age has been associated with an increased frequency of | Surgery_Schwartz. no significant differences were observed between surgical or transcatheter PVR. However, transcatheter PVR was associated with a shorter hospitalization. Hospitalization costs are similar for both procedures.168Arrhythmias are potentially the most serious late complication following TOF repair. In a multicenter cohort of 793 patients studied by Gatzoulis et al,164 a steady increase was documented in the prevalence of ventricular and atrial tachyarrhythmia and sudden cardiac death in the first 5 to 10 years after intracardiac repair. Clinical events were reported in 12% of patients at 35 years after repair. Prevalence of atrial arrhythmias from other studies, however, ranges from 1% to 11%,163,164 which is a reflection of the strong time dependence of arrhythmia onset.Underlying causes of arrhythmia following repair are complex and multifactorial, resulting in poorly defined opti-mum screening and treatment algorithms. Older repair age has been associated with an increased frequency of |
Surgery_Schwartz_5240 | Surgery_Schwartz | following repair are complex and multifactorial, resulting in poorly defined opti-mum screening and treatment algorithms. Older repair age has been associated with an increased frequency of both atrial and ventricular arrhythmias. Impaired ventricular function second-ary to a protracted period of cyanosis before repair might con-tribute to the propensity for arrhythmia in older patients.Ventricular Septal DefectAnatomy. VSD refers to a hole between the LV and RV. These defects are common, comprising 20% to 30% of all cases of CHD, and may occur as an isolated lesion or as part of a more Figure 20-57. The Melody valve.Brunicardi_Ch20_p0751-p0800.indd 78622/02/19 2:56 PM 787CONGENITAL HEART DISEASECHAPTER 20complex malformation.169 VSDs vary in size from 3 to 4 mm to more than 3 cm and are classified into four types based on their location in the ventricular septum: perimembranous (or paramembranous, conoventricular), AV canal (inlet), outlet or supracristal, and muscular (Fig. | Surgery_Schwartz. following repair are complex and multifactorial, resulting in poorly defined opti-mum screening and treatment algorithms. Older repair age has been associated with an increased frequency of both atrial and ventricular arrhythmias. Impaired ventricular function second-ary to a protracted period of cyanosis before repair might con-tribute to the propensity for arrhythmia in older patients.Ventricular Septal DefectAnatomy. VSD refers to a hole between the LV and RV. These defects are common, comprising 20% to 30% of all cases of CHD, and may occur as an isolated lesion or as part of a more Figure 20-57. The Melody valve.Brunicardi_Ch20_p0751-p0800.indd 78622/02/19 2:56 PM 787CONGENITAL HEART DISEASECHAPTER 20complex malformation.169 VSDs vary in size from 3 to 4 mm to more than 3 cm and are classified into four types based on their location in the ventricular septum: perimembranous (or paramembranous, conoventricular), AV canal (inlet), outlet or supracristal, and muscular (Fig. |
Surgery_Schwartz_5241 | Surgery_Schwartz | and are classified into four types based on their location in the ventricular septum: perimembranous (or paramembranous, conoventricular), AV canal (inlet), outlet or supracristal, and muscular (Fig. 20-58).Perimembranous VSDs are the most common type requir-ing surgical intervention, comprising approximately 80% of cases.169 These defects involve the membranous septum and include the malalignment defects seen in tetralogy of Fallot. In rare instances, the anterior and septal leaflets of the tricus-pid valve adhere to the edges of the perimembranous defect, forming a channel between the LV and the right atrium. These defects result in a large left-to-right shunt due to the large pres-sure differential between the two chambers.AV canal defects, also known as inlet defects, occur when part or all of the septum of the AV canal is absent. The VSD lies beneath the tricuspid valve and is limited upstream by the tricuspid annulus, without intervening muscle.The supracristal or outlet VSD | Surgery_Schwartz. and are classified into four types based on their location in the ventricular septum: perimembranous (or paramembranous, conoventricular), AV canal (inlet), outlet or supracristal, and muscular (Fig. 20-58).Perimembranous VSDs are the most common type requir-ing surgical intervention, comprising approximately 80% of cases.169 These defects involve the membranous septum and include the malalignment defects seen in tetralogy of Fallot. In rare instances, the anterior and septal leaflets of the tricus-pid valve adhere to the edges of the perimembranous defect, forming a channel between the LV and the right atrium. These defects result in a large left-to-right shunt due to the large pres-sure differential between the two chambers.AV canal defects, also known as inlet defects, occur when part or all of the septum of the AV canal is absent. The VSD lies beneath the tricuspid valve and is limited upstream by the tricuspid annulus, without intervening muscle.The supracristal or outlet VSD |
Surgery_Schwartz_5242 | Surgery_Schwartz | or all of the septum of the AV canal is absent. The VSD lies beneath the tricuspid valve and is limited upstream by the tricuspid annulus, without intervening muscle.The supracristal or outlet VSD results from a defect within the conal septum. Characteristically, these defects are limited upstream by the pulmonary valve and are otherwise surrounded by the muscle of the infundibular septum.Muscular VSDs are the most common type and may lie in four locations: anterior, midventricular, posterior, or apical. These are surrounded by muscle and can occur anywhere along the trabecular portion of the septum. The rare “Swiss-cheese” type of muscular VSD consists of multiple communications between the RV and LV, complicating operative repair.Pathophysiology and Clinical Presentation. The size of the VSD determines the initial pathophysiology of the disease. Large VSDs are classified as nonrestrictive and are at least equal in diameter to the aortic annulus. These defects allow free flow of | Surgery_Schwartz. or all of the septum of the AV canal is absent. The VSD lies beneath the tricuspid valve and is limited upstream by the tricuspid annulus, without intervening muscle.The supracristal or outlet VSD results from a defect within the conal septum. Characteristically, these defects are limited upstream by the pulmonary valve and are otherwise surrounded by the muscle of the infundibular septum.Muscular VSDs are the most common type and may lie in four locations: anterior, midventricular, posterior, or apical. These are surrounded by muscle and can occur anywhere along the trabecular portion of the septum. The rare “Swiss-cheese” type of muscular VSD consists of multiple communications between the RV and LV, complicating operative repair.Pathophysiology and Clinical Presentation. The size of the VSD determines the initial pathophysiology of the disease. Large VSDs are classified as nonrestrictive and are at least equal in diameter to the aortic annulus. These defects allow free flow of |
Surgery_Schwartz_5243 | Surgery_Schwartz | the VSD determines the initial pathophysiology of the disease. Large VSDs are classified as nonrestrictive and are at least equal in diameter to the aortic annulus. These defects allow free flow of blood from the LV to the RV, elevating right ventricular pres-sures to the same level as systemic pressure.Consequently, the pulmonary-to-systemic flow ratio (Qp to Qs) is inversely dependent on the ratio of pulmonary vas-cular resistance to systemic vascular resistance. Nonrestrictive VSDs produce a large increase in pulmonary blood flow, and the afflicted infant will present with symptoms of congestive heart failure. However, if untreated, these defects will cause pulmonary hypertension with a corresponding increase in pulmonary vascular resistance. This will lead to a reversal of flow (a right-to-left shunt), which is known as Eisenmenger’s syndrome.Small restrictive VSDs offer significant resistance to the passage of blood across the defect, and therefore right ventricu-lar pressure is | Surgery_Schwartz. the VSD determines the initial pathophysiology of the disease. Large VSDs are classified as nonrestrictive and are at least equal in diameter to the aortic annulus. These defects allow free flow of blood from the LV to the RV, elevating right ventricular pres-sures to the same level as systemic pressure.Consequently, the pulmonary-to-systemic flow ratio (Qp to Qs) is inversely dependent on the ratio of pulmonary vas-cular resistance to systemic vascular resistance. Nonrestrictive VSDs produce a large increase in pulmonary blood flow, and the afflicted infant will present with symptoms of congestive heart failure. However, if untreated, these defects will cause pulmonary hypertension with a corresponding increase in pulmonary vascular resistance. This will lead to a reversal of flow (a right-to-left shunt), which is known as Eisenmenger’s syndrome.Small restrictive VSDs offer significant resistance to the passage of blood across the defect, and therefore right ventricu-lar pressure is |
Surgery_Schwartz_5244 | Surgery_Schwartz | shunt), which is known as Eisenmenger’s syndrome.Small restrictive VSDs offer significant resistance to the passage of blood across the defect, and therefore right ventricu-lar pressure is either normal or only minimally elevated and the ratio of Qp to Qs rarely exceeds 1.5. These defects are generally asymptomatic because there are few physiologic consequences. However, there is a long-term risk of endocarditis because endo-cardial damage from the jet of blood through the defect may serve as a possible nidus for colonization (Fig. 20-59A,B).Diagnosis. The child with a large VSD will present with severe congestive heart failure and frequent respiratory tract infections. Children with Eisenmenger’s syndrome may be deceptively asymptomatic until frank cyanosis develops.The chest radiograph will show cardiomegaly and pulmo-nary overcirculation, and the ECG will show signs of left ven-tricular or biventricular hypertrophy. Echocardiography provides definitive diagnosis and can estimate | Surgery_Schwartz. shunt), which is known as Eisenmenger’s syndrome.Small restrictive VSDs offer significant resistance to the passage of blood across the defect, and therefore right ventricu-lar pressure is either normal or only minimally elevated and the ratio of Qp to Qs rarely exceeds 1.5. These defects are generally asymptomatic because there are few physiologic consequences. However, there is a long-term risk of endocarditis because endo-cardial damage from the jet of blood through the defect may serve as a possible nidus for colonization (Fig. 20-59A,B).Diagnosis. The child with a large VSD will present with severe congestive heart failure and frequent respiratory tract infections. Children with Eisenmenger’s syndrome may be deceptively asymptomatic until frank cyanosis develops.The chest radiograph will show cardiomegaly and pulmo-nary overcirculation, and the ECG will show signs of left ven-tricular or biventricular hypertrophy. Echocardiography provides definitive diagnosis and can estimate |
Surgery_Schwartz_5245 | Surgery_Schwartz | will show cardiomegaly and pulmo-nary overcirculation, and the ECG will show signs of left ven-tricular or biventricular hypertrophy. Echocardiography provides definitive diagnosis and can estimate the degree of shunting as well as pulmonary arterial pressures. Cardiac catheterization has MembranousMuscularInletSupracristalTVFigure 20-58. Types of VSD. (Used with permission from Kelly Rosso MD.)ABFigure 20-59. A. Severe TV and VSD endocarditis (‘*’) in a 4 yo untreated patient. B. Echocardiographic appearance of the same patient after patch repair(‘*’) of the VSD and complete exci-sion of the tricuspid valve.Brunicardi_Ch20_p0751-p0800.indd 78722/02/19 2:56 PM 788SPECIFIC CONSIDERATIONSPART IIlargely been supplanted by echocardiography, except in older children where measurement of pulmonary resistance is neces-sary prior to recommending closure of the defect.Treatment. VSDs may close or narrow spontaneously, and the probability of closure is inversely related to the age at which | Surgery_Schwartz. will show cardiomegaly and pulmo-nary overcirculation, and the ECG will show signs of left ven-tricular or biventricular hypertrophy. Echocardiography provides definitive diagnosis and can estimate the degree of shunting as well as pulmonary arterial pressures. Cardiac catheterization has MembranousMuscularInletSupracristalTVFigure 20-58. Types of VSD. (Used with permission from Kelly Rosso MD.)ABFigure 20-59. A. Severe TV and VSD endocarditis (‘*’) in a 4 yo untreated patient. B. Echocardiographic appearance of the same patient after patch repair(‘*’) of the VSD and complete exci-sion of the tricuspid valve.Brunicardi_Ch20_p0751-p0800.indd 78722/02/19 2:56 PM 788SPECIFIC CONSIDERATIONSPART IIlargely been supplanted by echocardiography, except in older children where measurement of pulmonary resistance is neces-sary prior to recommending closure of the defect.Treatment. VSDs may close or narrow spontaneously, and the probability of closure is inversely related to the age at which |
Surgery_Schwartz_5246 | Surgery_Schwartz | resistance is neces-sary prior to recommending closure of the defect.Treatment. VSDs may close or narrow spontaneously, and the probability of closure is inversely related to the age at which the defect is observed. Thus, infants at 1 month of age have an 80% incidence of spontaneous closure, whereas a child at 12 months of age has only a 25% chance of closure.170 This has an important impact on operative decision-making because a small or moder-ate-size VSD may be observed for a period of time in the absence of symptoms. Large defects and those in severely symptomatic neonates should be repaired during infancy to relieve symptoms and because irreversible changes in pulmonary vascular resis-tance may develop during the first year of life.Repair of isolated VSDs requires the use of CPB with moderate hypothermia and cardioplegic arrest. The right atrial approach (Fig. 20-60) is preferable for most defects, except apical muscular defects, which often require a right ventricu-lotomy for | Surgery_Schwartz. resistance is neces-sary prior to recommending closure of the defect.Treatment. VSDs may close or narrow spontaneously, and the probability of closure is inversely related to the age at which the defect is observed. Thus, infants at 1 month of age have an 80% incidence of spontaneous closure, whereas a child at 12 months of age has only a 25% chance of closure.170 This has an important impact on operative decision-making because a small or moder-ate-size VSD may be observed for a period of time in the absence of symptoms. Large defects and those in severely symptomatic neonates should be repaired during infancy to relieve symptoms and because irreversible changes in pulmonary vascular resis-tance may develop during the first year of life.Repair of isolated VSDs requires the use of CPB with moderate hypothermia and cardioplegic arrest. The right atrial approach (Fig. 20-60) is preferable for most defects, except apical muscular defects, which often require a right ventricu-lotomy for |
Surgery_Schwartz_5247 | Surgery_Schwartz | moderate hypothermia and cardioplegic arrest. The right atrial approach (Fig. 20-60) is preferable for most defects, except apical muscular defects, which often require a right ventricu-lotomy for adequate exposure. Supracristal defects may alter-natively be exposed via a pulmonary arteriotomy or through an incision in the RV immediately beneath the pulmonary valve (Fig. 20-61). Regardless of the type of defect present, a right atrial approach can be used initially to inspect the anatomy, as this may be abandoned should it offer inadequate exposure for repair. After careful inspection of the heart for any associated malformations, a patch repair is employed, taking care to avoid the conduction system. Routine use of intraoperative trans-esophageal echocardiography should be used to assess for any residual defect.Successful percutaneous device closure of VSDs using the Amplatzer device has been described.152 The device has demon-strated a 100% closure rate in a small series of patients | Surgery_Schwartz. moderate hypothermia and cardioplegic arrest. The right atrial approach (Fig. 20-60) is preferable for most defects, except apical muscular defects, which often require a right ventricu-lotomy for adequate exposure. Supracristal defects may alter-natively be exposed via a pulmonary arteriotomy or through an incision in the RV immediately beneath the pulmonary valve (Fig. 20-61). Regardless of the type of defect present, a right atrial approach can be used initially to inspect the anatomy, as this may be abandoned should it offer inadequate exposure for repair. After careful inspection of the heart for any associated malformations, a patch repair is employed, taking care to avoid the conduction system. Routine use of intraoperative trans-esophageal echocardiography should be used to assess for any residual defect.Successful percutaneous device closure of VSDs using the Amplatzer device has been described.152 The device has demon-strated a 100% closure rate in a small series of patients |
Surgery_Schwartz_5248 | Surgery_Schwartz | for any residual defect.Successful percutaneous device closure of VSDs using the Amplatzer device has been described.152 The device has demon-strated a 100% closure rate in a small series of patients with iso-lated or residual VSDs, or as a collaborative treatment strategy for the VSD component in more complex congenital lesions. Proponents of device closure argue that its use can decrease the complexity of surgical repair, avoid reoperation for a small residual lesion, or avoid the need for a ventriculotomy. The use of devices to close paramembranous defects can cause heart block because the defect is in close association to the conduction system (Fig 20-62).171 The procedure can be performed percuta-neously or through the per ventricular approach. Embolization of the device is an added risk.Multiple or “Swiss-cheese” VSDs represent a special case, and many cannot be repaired during infancy. In patients in whom definitive VSD closure cannot be accomplished, tem-porary placement of a | Surgery_Schwartz. for any residual defect.Successful percutaneous device closure of VSDs using the Amplatzer device has been described.152 The device has demon-strated a 100% closure rate in a small series of patients with iso-lated or residual VSDs, or as a collaborative treatment strategy for the VSD component in more complex congenital lesions. Proponents of device closure argue that its use can decrease the complexity of surgical repair, avoid reoperation for a small residual lesion, or avoid the need for a ventriculotomy. The use of devices to close paramembranous defects can cause heart block because the defect is in close association to the conduction system (Fig 20-62).171 The procedure can be performed percuta-neously or through the per ventricular approach. Embolization of the device is an added risk.Multiple or “Swiss-cheese” VSDs represent a special case, and many cannot be repaired during infancy. In patients in whom definitive VSD closure cannot be accomplished, tem-porary placement of a |
Surgery_Schwartz_5249 | Surgery_Schwartz | risk.Multiple or “Swiss-cheese” VSDs represent a special case, and many cannot be repaired during infancy. In patients in whom definitive VSD closure cannot be accomplished, tem-porary placement of a pulmonary artery band can be employed to control pulmonary flow. This allows time for spontaneous closure of many of the smaller defects, thus simplifying surgi-cal repair.172Some centers, however, have advocated early definitive repair of the Swiss-cheese septum, by using oversize patches, fibrin glue, and combined intraoperative device closure, as well as techniques to complete the repair transatrially.173Results. Even in very small infants, closure of VSDs can be safely performed with hospital mortality near 0%. The main risk factor remains the presence of other associated lesions, espe-cially when present in symptomatic neonates with large VSDs.Figure 20-60. Intra-op picture during a VSD closure performed by interrupted suture technique with patch closure.Figure | Surgery_Schwartz. risk.Multiple or “Swiss-cheese” VSDs represent a special case, and many cannot be repaired during infancy. In patients in whom definitive VSD closure cannot be accomplished, tem-porary placement of a pulmonary artery band can be employed to control pulmonary flow. This allows time for spontaneous closure of many of the smaller defects, thus simplifying surgi-cal repair.172Some centers, however, have advocated early definitive repair of the Swiss-cheese septum, by using oversize patches, fibrin glue, and combined intraoperative device closure, as well as techniques to complete the repair transatrially.173Results. Even in very small infants, closure of VSDs can be safely performed with hospital mortality near 0%. The main risk factor remains the presence of other associated lesions, espe-cially when present in symptomatic neonates with large VSDs.Figure 20-60. Intra-op picture during a VSD closure performed by interrupted suture technique with patch closure.Figure |
Surgery_Schwartz_5250 | Surgery_Schwartz | lesions, espe-cially when present in symptomatic neonates with large VSDs.Figure 20-60. Intra-op picture during a VSD closure performed by interrupted suture technique with patch closure.Figure 20-61. Echocardiographic appearance of a supracristal VSD (arrow). Note its location just beneath the pulmonary valve (‘*’).Brunicardi_Ch20_p0751-p0800.indd 78822/02/19 2:56 PM 789CONGENITAL HEART DISEASECHAPTER 20Atrioventricular Canal DefectsAnatomy. AV canal defects result from failure of fusion of the endocardial cushions in the central portion of the heart, caus-ing a lesion that involves the atrial and the ventricular septum, as well as the anterior mitral and septal tricuspid valve leaf-lets. Defects involving primarily the atrial septum are known as partial AV canal defects and frequently occur in conjunction with a cleft anterior mitral leaflet. Complete AV canal defects have a combined deficiency of the atrial and ventricular sep-tum associated with a common AV orifice rather than | Surgery_Schwartz. lesions, espe-cially when present in symptomatic neonates with large VSDs.Figure 20-60. Intra-op picture during a VSD closure performed by interrupted suture technique with patch closure.Figure 20-61. Echocardiographic appearance of a supracristal VSD (arrow). Note its location just beneath the pulmonary valve (‘*’).Brunicardi_Ch20_p0751-p0800.indd 78822/02/19 2:56 PM 789CONGENITAL HEART DISEASECHAPTER 20Atrioventricular Canal DefectsAnatomy. AV canal defects result from failure of fusion of the endocardial cushions in the central portion of the heart, caus-ing a lesion that involves the atrial and the ventricular septum, as well as the anterior mitral and septal tricuspid valve leaf-lets. Defects involving primarily the atrial septum are known as partial AV canal defects and frequently occur in conjunction with a cleft anterior mitral leaflet. Complete AV canal defects have a combined deficiency of the atrial and ventricular sep-tum associated with a common AV orifice rather than |
Surgery_Schwartz_5251 | Surgery_Schwartz | occur in conjunction with a cleft anterior mitral leaflet. Complete AV canal defects have a combined deficiency of the atrial and ventricular sep-tum associated with a common AV orifice rather than separate tricuspid and mitral valves. The common AV valve generally has five leaflets, three lateral (free wall) and two bridging (septal) leaflets. The defect in the ventricular septum can lie either between the two bridging leaflets or beneath them. The relationship between the septal defect and the anterior bridging leaflet forms the basis of the Rastelli classification for complete AV canal defects (Fig. 20-63).174,175Pathophysiology and Diagnosis. Partial AV canal defects, in the absence of AV valvular regurgitation, frequently resemble isolated ASDs. Left-to-right shunting predominates as long as pulmonary vascular resistance remains low. However, 40% of patients with partial AV canal defects have moderate-to-severe valve incompetence, and progressive heart failure occurs early in | Surgery_Schwartz. occur in conjunction with a cleft anterior mitral leaflet. Complete AV canal defects have a combined deficiency of the atrial and ventricular sep-tum associated with a common AV orifice rather than separate tricuspid and mitral valves. The common AV valve generally has five leaflets, three lateral (free wall) and two bridging (septal) leaflets. The defect in the ventricular septum can lie either between the two bridging leaflets or beneath them. The relationship between the septal defect and the anterior bridging leaflet forms the basis of the Rastelli classification for complete AV canal defects (Fig. 20-63).174,175Pathophysiology and Diagnosis. Partial AV canal defects, in the absence of AV valvular regurgitation, frequently resemble isolated ASDs. Left-to-right shunting predominates as long as pulmonary vascular resistance remains low. However, 40% of patients with partial AV canal defects have moderate-to-severe valve incompetence, and progressive heart failure occurs early in |
Surgery_Schwartz_5252 | Surgery_Schwartz | as long as pulmonary vascular resistance remains low. However, 40% of patients with partial AV canal defects have moderate-to-severe valve incompetence, and progressive heart failure occurs early in this patient population.175 Complete AV canal defects produce more severe pathophysiologic changes because the large intra-cardiac communication and significant AV valve regurgitation contribute to ventricular volume loading and pulmonary hyper-tension. Children with complete AV canal defects develop signs of congestive heart failure within the first few months of life.Physical examination may reveal a right ventricular heave and a systolic murmur. Children may also present with endo-carditis or paradoxical emboli as a result of the intracardiac communication. Chest radiography will be consistent with con-gestive heart failure, and the EKG demonstrates right ventricu-lar hypertrophy with a prolonged PR interval and is classically associated with left axis deviation.Two-dimensional | Surgery_Schwartz. as long as pulmonary vascular resistance remains low. However, 40% of patients with partial AV canal defects have moderate-to-severe valve incompetence, and progressive heart failure occurs early in this patient population.175 Complete AV canal defects produce more severe pathophysiologic changes because the large intra-cardiac communication and significant AV valve regurgitation contribute to ventricular volume loading and pulmonary hyper-tension. Children with complete AV canal defects develop signs of congestive heart failure within the first few months of life.Physical examination may reveal a right ventricular heave and a systolic murmur. Children may also present with endo-carditis or paradoxical emboli as a result of the intracardiac communication. Chest radiography will be consistent with con-gestive heart failure, and the EKG demonstrates right ventricu-lar hypertrophy with a prolonged PR interval and is classically associated with left axis deviation.Two-dimensional |
Surgery_Schwartz_5253 | Surgery_Schwartz | consistent with con-gestive heart failure, and the EKG demonstrates right ventricu-lar hypertrophy with a prolonged PR interval and is classically associated with left axis deviation.Two-dimensional echocardiography (Fig. 20-64) with color-flow mapping is confirmatory, but cardiac catheterization can be employed to define the status of the pulmonary vascula-ture, with a pulmonary vascular resistance greater than 12 Wood units indicating inoperability.Treatment. The management of patients with AV canal defects can be especially challenging. Timing of operation is individualized. Patients with partial defects can be electively repaired between 2 and 5 years of age, whereas complete AV canal defects should be repaired within the first year of life to prevent irreversible changes in the pulmonary circulation. Complete repair in infancy should be accomplished, with palliative procedures such as pulmonary artery banding reserved for only those infants with other complex lesions or who are | Surgery_Schwartz. consistent with con-gestive heart failure, and the EKG demonstrates right ventricu-lar hypertrophy with a prolonged PR interval and is classically associated with left axis deviation.Two-dimensional echocardiography (Fig. 20-64) with color-flow mapping is confirmatory, but cardiac catheterization can be employed to define the status of the pulmonary vascula-ture, with a pulmonary vascular resistance greater than 12 Wood units indicating inoperability.Treatment. The management of patients with AV canal defects can be especially challenging. Timing of operation is individualized. Patients with partial defects can be electively repaired between 2 and 5 years of age, whereas complete AV canal defects should be repaired within the first year of life to prevent irreversible changes in the pulmonary circulation. Complete repair in infancy should be accomplished, with palliative procedures such as pulmonary artery banding reserved for only those infants with other complex lesions or who are |
Surgery_Schwartz_5254 | Surgery_Schwartz | circulation. Complete repair in infancy should be accomplished, with palliative procedures such as pulmonary artery banding reserved for only those infants with other complex lesions or who are too ill to tolerate CPB.The operative technique requires the use of either continu-ous hypothermic CPB or, for small infants, deep hypothermic circulatory arrest. The heart is initially approached through an oblique right atriotomy, and the anatomy is carefully observed. In the case of a partial AV canal, the cleft in the mitral valve is repaired with interrupted sutures and the ASD is closed with a pericardial patch. Complete AV canal defects are repaired by patch closure of the VSD, separating the common AV valve into tricuspid and mitral components and suspending the neovalves from the top of the VSD patch and closing the ASD.Results. Partial AV canal defects have an excellent outcome, with a mortality rate of 0% to 2% in most series.175 Complete AV canal defects are associated with | Surgery_Schwartz. circulation. Complete repair in infancy should be accomplished, with palliative procedures such as pulmonary artery banding reserved for only those infants with other complex lesions or who are too ill to tolerate CPB.The operative technique requires the use of either continu-ous hypothermic CPB or, for small infants, deep hypothermic circulatory arrest. The heart is initially approached through an oblique right atriotomy, and the anatomy is carefully observed. In the case of a partial AV canal, the cleft in the mitral valve is repaired with interrupted sutures and the ASD is closed with a pericardial patch. Complete AV canal defects are repaired by patch closure of the VSD, separating the common AV valve into tricuspid and mitral components and suspending the neovalves from the top of the VSD patch and closing the ASD.Results. Partial AV canal defects have an excellent outcome, with a mortality rate of 0% to 2% in most series.175 Complete AV canal defects are associated with |
Surgery_Schwartz_5255 | Surgery_Schwartz | of the VSD patch and closing the ASD.Results. Partial AV canal defects have an excellent outcome, with a mortality rate of 0% to 2% in most series.175 Complete AV canal defects are associated with anoperative mortality of 3% to 4%.176The most frequently encountered postoperative problems are complete heart block (1%–2%), right bundle-branch block (22%), arrhythmias (11%), RVOT obstruction (11%), and severe mitral regurgitation (13%–24%).175 The increasing use of intraoperative transesophageal echocardiography may positively Figure 20-62. Intraoperative picture at the time of removal of a percutaneously placed VSD device causing severe TR and complete heart block. Note the close association of the device to the tricuspid valve leaflet (arrow) and cordae.Type AType BType CFigure 20-63. Rastelli classification of complete AVSD. (Used with permission from Kelly Rosso MD.)Figure 20-64. Echo of an infant with complete AVSD. Note the prominent absence of the ‘crux’ (‘*’) of the heart in this | Surgery_Schwartz. of the VSD patch and closing the ASD.Results. Partial AV canal defects have an excellent outcome, with a mortality rate of 0% to 2% in most series.175 Complete AV canal defects are associated with anoperative mortality of 3% to 4%.176The most frequently encountered postoperative problems are complete heart block (1%–2%), right bundle-branch block (22%), arrhythmias (11%), RVOT obstruction (11%), and severe mitral regurgitation (13%–24%).175 The increasing use of intraoperative transesophageal echocardiography may positively Figure 20-62. Intraoperative picture at the time of removal of a percutaneously placed VSD device causing severe TR and complete heart block. Note the close association of the device to the tricuspid valve leaflet (arrow) and cordae.Type AType BType CFigure 20-63. Rastelli classification of complete AVSD. (Used with permission from Kelly Rosso MD.)Figure 20-64. Echo of an infant with complete AVSD. Note the prominent absence of the ‘crux’ (‘*’) of the heart in this |
Surgery_Schwartz_5256 | Surgery_Schwartz | classification of complete AVSD. (Used with permission from Kelly Rosso MD.)Figure 20-64. Echo of an infant with complete AVSD. Note the prominent absence of the ‘crux’ (‘*’) of the heart in this defect.Brunicardi_Ch20_p0751-p0800.indd 78922/02/19 2:56 PM 790SPECIFIC CONSIDERATIONSPART IIinfluence outcomes, as the adequacy of repair can be assessed and treated without need for subsequent reoperation.174-175Interrupted Aortic ArchAnatomy. Interrupted aortic arch (IAA) is a rare defect, com-prising approximately 1% of all cases of CHD.177 It is defined as an absence of luminal continuity between the ascending and descending aorta and does not occur as an isolated defect in most cases because a VSD or PDA is usually present. IAA is classified based on the location of the interruption (Fig. 20-65 to Fig. 20-67).Clinical Manifestations and Diagnosis. Infants with IAA have ductal-dependent systemic blood flow and will develop profound metabolic acidosis and hemodynamic collapse upon | Surgery_Schwartz. classification of complete AVSD. (Used with permission from Kelly Rosso MD.)Figure 20-64. Echo of an infant with complete AVSD. Note the prominent absence of the ‘crux’ (‘*’) of the heart in this defect.Brunicardi_Ch20_p0751-p0800.indd 78922/02/19 2:56 PM 790SPECIFIC CONSIDERATIONSPART IIinfluence outcomes, as the adequacy of repair can be assessed and treated without need for subsequent reoperation.174-175Interrupted Aortic ArchAnatomy. Interrupted aortic arch (IAA) is a rare defect, com-prising approximately 1% of all cases of CHD.177 It is defined as an absence of luminal continuity between the ascending and descending aorta and does not occur as an isolated defect in most cases because a VSD or PDA is usually present. IAA is classified based on the location of the interruption (Fig. 20-65 to Fig. 20-67).Clinical Manifestations and Diagnosis. Infants with IAA have ductal-dependent systemic blood flow and will develop profound metabolic acidosis and hemodynamic collapse upon |
Surgery_Schwartz_5257 | Surgery_Schwartz | (Fig. 20-65 to Fig. 20-67).Clinical Manifestations and Diagnosis. Infants with IAA have ductal-dependent systemic blood flow and will develop profound metabolic acidosis and hemodynamic collapse upon ductal closure. In the rare instance of failed ductal closure, the diagnosis may be missed during infancy, and the child will pres-ent with symptoms of congestive heart failure from a persistent left-to-right shunt.Once definitive diagnosis is made in infants, usually with echocardiography, preparations are made for operative interven-tion, and prostaglandin E1 is infused to maintain ductal patency and correct acidosis. The infant’s hemodynamic status should Figure 20-66. CT angiogram of a Type A IAA.AoAoPAType AType BType CPAPAAoFigure 20-65. Types of IAA. (Used with permission from Nicholas Clarke MD.)Figure 20-67. MRI reconstruction of a Type B IAA.be optimized with mechanical ventilation and inotropic support. An effort should be made to increase pulmonary vascular resis-tance by | Surgery_Schwartz. (Fig. 20-65 to Fig. 20-67).Clinical Manifestations and Diagnosis. Infants with IAA have ductal-dependent systemic blood flow and will develop profound metabolic acidosis and hemodynamic collapse upon ductal closure. In the rare instance of failed ductal closure, the diagnosis may be missed during infancy, and the child will pres-ent with symptoms of congestive heart failure from a persistent left-to-right shunt.Once definitive diagnosis is made in infants, usually with echocardiography, preparations are made for operative interven-tion, and prostaglandin E1 is infused to maintain ductal patency and correct acidosis. The infant’s hemodynamic status should Figure 20-66. CT angiogram of a Type A IAA.AoAoPAType AType BType CPAPAAoFigure 20-65. Types of IAA. (Used with permission from Nicholas Clarke MD.)Figure 20-67. MRI reconstruction of a Type B IAA.be optimized with mechanical ventilation and inotropic support. An effort should be made to increase pulmonary vascular resis-tance by |
Surgery_Schwartz_5258 | Surgery_Schwartz | Clarke MD.)Figure 20-67. MRI reconstruction of a Type B IAA.be optimized with mechanical ventilation and inotropic support. An effort should be made to increase pulmonary vascular resis-tance by decreasing the fractional inspired oxygen and avoiding hyperventilation because this will preferentially direct blood into the systemic circulation.Treatment. Initial strategies for the management of IAA involved palliation though a left thoracotomy by using one of the arch vessels as a conduit to restore aortic continuity. Pulmo-nary artery banding can be simultaneously performed to limit left-to-right shunting because it is not feasible to repair the VSD or other intracardiac communications with this approach.However, complete one stage surgical repair in infants with IAA is now preferable. The operative technique involves use of a median sternotomy and CPB with short periods of cir-culatory arrest. Aortic arch reconstruction can be accomplished with either direct anastomosis or patch | Surgery_Schwartz. Clarke MD.)Figure 20-67. MRI reconstruction of a Type B IAA.be optimized with mechanical ventilation and inotropic support. An effort should be made to increase pulmonary vascular resis-tance by decreasing the fractional inspired oxygen and avoiding hyperventilation because this will preferentially direct blood into the systemic circulation.Treatment. Initial strategies for the management of IAA involved palliation though a left thoracotomy by using one of the arch vessels as a conduit to restore aortic continuity. Pulmo-nary artery banding can be simultaneously performed to limit left-to-right shunting because it is not feasible to repair the VSD or other intracardiac communications with this approach.However, complete one stage surgical repair in infants with IAA is now preferable. The operative technique involves use of a median sternotomy and CPB with short periods of cir-culatory arrest. Aortic arch reconstruction can be accomplished with either direct anastomosis or patch |
Surgery_Schwartz_5259 | Surgery_Schwartz | The operative technique involves use of a median sternotomy and CPB with short periods of cir-culatory arrest. Aortic arch reconstruction can be accomplished with either direct anastomosis or patch aortoplasty followed by closure of the VSD.178In certain cases, the defect will involve hypoplasia of the left heart, precluding attempts at definitive repair. These infants should be managed with a Norwood procedure followed by a Fontan repair.Results. Outcomes in infants with IAA have improved sub-stantially over the last decades as a result of improved periop-erative care. Operative mortality is now less than 10% in most series.177,179 Some authors advocate the use of patch augmenta-tion of the aorta to ensure adequate relief of LVOT obstruction and to diminish anastomotic tension, thus reducing the subse-quent risk of restenosis and tracheobronchial compression.178Pediatric Mechanical Circulatory SupportMechanical circulatory support has become standard therapy for adults with end stage | Surgery_Schwartz. The operative technique involves use of a median sternotomy and CPB with short periods of cir-culatory arrest. Aortic arch reconstruction can be accomplished with either direct anastomosis or patch aortoplasty followed by closure of the VSD.178In certain cases, the defect will involve hypoplasia of the left heart, precluding attempts at definitive repair. These infants should be managed with a Norwood procedure followed by a Fontan repair.Results. Outcomes in infants with IAA have improved sub-stantially over the last decades as a result of improved periop-erative care. Operative mortality is now less than 10% in most series.177,179 Some authors advocate the use of patch augmenta-tion of the aorta to ensure adequate relief of LVOT obstruction and to diminish anastomotic tension, thus reducing the subse-quent risk of restenosis and tracheobronchial compression.178Pediatric Mechanical Circulatory SupportMechanical circulatory support has become standard therapy for adults with end stage |
Surgery_Schwartz_5260 | Surgery_Schwartz | the subse-quent risk of restenosis and tracheobronchial compression.178Pediatric Mechanical Circulatory SupportMechanical circulatory support has become standard therapy for adults with end stage heart failure. There has been a sig-nificant lag with development of similar devices for the pediatric population. This is probably related to the smaller mar-ket for these devices and the technical challenges associated with the anatomical constraints secondary to anatomy and size of the patients. Extracorporeal membrane oxygenation (ECMO) 8Brunicardi_Ch20_p0751-p0800.indd 79022/02/19 2:56 PM 791CONGENITAL HEART DISEASECHAPTER 20has been the mainstay of mechanical support in many centers for the pediatric population. The adaptation of other adult devices to the pediatric population las led to the slow but steady devel-opment of pediatric durable mechanical devices. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) device was approved by the FDA in 2011 in the United States as a | Surgery_Schwartz. the subse-quent risk of restenosis and tracheobronchial compression.178Pediatric Mechanical Circulatory SupportMechanical circulatory support has become standard therapy for adults with end stage heart failure. There has been a sig-nificant lag with development of similar devices for the pediatric population. This is probably related to the smaller mar-ket for these devices and the technical challenges associated with the anatomical constraints secondary to anatomy and size of the patients. Extracorporeal membrane oxygenation (ECMO) 8Brunicardi_Ch20_p0751-p0800.indd 79022/02/19 2:56 PM 791CONGENITAL HEART DISEASECHAPTER 20has been the mainstay of mechanical support in many centers for the pediatric population. The adaptation of other adult devices to the pediatric population las led to the slow but steady devel-opment of pediatric durable mechanical devices. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) device was approved by the FDA in 2011 in the United States as a |
Surgery_Schwartz_5261 | Surgery_Schwartz | to the slow but steady devel-opment of pediatric durable mechanical devices. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) device was approved by the FDA in 2011 in the United States as a paracor-poreal device that can be used as a bridge to transplantation. This device has a 73% overall survival post implant at 12 months.97 Infection, stroke and bleeding remain significant morbidities associated with it. Young age and small body surface area still remain poor prognostic factors. In 2010, the National Heart, Lung, and Blood Institute launched the Pumps for Kids, Infants, and Neonates (PumpKIN) program to promote development of new devices with the goal of clinical use.ECMO remains the most commonly used form of mechan-ical support in the pediatric population in the United States. Per the ECLS Registry report released by the Extracorporeal Life Support Organization, as of January 2017, there were a total of 16,531 ECMO runs performed for cardiac causes, internation-ally.180 | Surgery_Schwartz. to the slow but steady devel-opment of pediatric durable mechanical devices. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) device was approved by the FDA in 2011 in the United States as a paracor-poreal device that can be used as a bridge to transplantation. This device has a 73% overall survival post implant at 12 months.97 Infection, stroke and bleeding remain significant morbidities associated with it. Young age and small body surface area still remain poor prognostic factors. In 2010, the National Heart, Lung, and Blood Institute launched the Pumps for Kids, Infants, and Neonates (PumpKIN) program to promote development of new devices with the goal of clinical use.ECMO remains the most commonly used form of mechan-ical support in the pediatric population in the United States. Per the ECLS Registry report released by the Extracorporeal Life Support Organization, as of January 2017, there were a total of 16,531 ECMO runs performed for cardiac causes, internation-ally.180 |
Surgery_Schwartz_5262 | Surgery_Schwartz | Per the ECLS Registry report released by the Extracorporeal Life Support Organization, as of January 2017, there were a total of 16,531 ECMO runs performed for cardiac causes, internation-ally.180 The survival to discharge is about 40% in the neona-tal population as opposed to 50% in the pediatric population. ECMO remains the only means of salvage for newborns and infants in many institutions. The biggest limitation remains the short duration it can be used. It is often used as a bridge to recovery and sometimes as a bridge to transplantation. The abil-ity to place small infants on ECMO with peripheral cannulation continues to make it a very attractive first line option.Ventricular assist devices can be either of the pulsatile or continuous types. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) remains a classic example of a pulsa-tile device. The Impella 2.5 (Abiomed) (Fig. 20-68) has been used in the pediatric population as a temporary support device for recovering | Surgery_Schwartz. Per the ECLS Registry report released by the Extracorporeal Life Support Organization, as of January 2017, there were a total of 16,531 ECMO runs performed for cardiac causes, internation-ally.180 The survival to discharge is about 40% in the neona-tal population as opposed to 50% in the pediatric population. ECMO remains the only means of salvage for newborns and infants in many institutions. The biggest limitation remains the short duration it can be used. It is often used as a bridge to recovery and sometimes as a bridge to transplantation. The abil-ity to place small infants on ECMO with peripheral cannulation continues to make it a very attractive first line option.Ventricular assist devices can be either of the pulsatile or continuous types. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) remains a classic example of a pulsa-tile device. The Impella 2.5 (Abiomed) (Fig. 20-68) has been used in the pediatric population as a temporary support device for recovering |
Surgery_Schwartz_5263 | Surgery_Schwartz | AG, Berlin, Germany) remains a classic example of a pulsa-tile device. The Impella 2.5 (Abiomed) (Fig. 20-68) has been used in the pediatric population as a temporary support device for recovering myocarditis, during treatment of acute rejection after heart transplantation and high-risk interventions in frag-ile patients with marginal function.181,182 Other continuous flow devices available for the pediatric patient include the Heartmate II Figure 20-68. Impella 2.5 (Abiomed). (Reproduced with permis-sion from Abiomed. Danvers, MA.)Figure 20-69. Heartware HVAD. (HeartWare® HVAD (Heart-Ware Inc., Miami Lakes, FL.)and Heartmate III devices (Thoratec, Pleasanton, CA), DeBakey VAD Child (MicroMed Technology, Houston, TX), PediMag (Thoratec, Pleasanton, CA), Jarvik2015 and HeartWare HVAD (Fig 20-69) (HeartWare international Inc, Framingham, MA).183 The total artificial heart (SynCardia Systems Inc, Tuscon, Az, USA) is an implantable biventricular device that replaces both ventricles. With | Surgery_Schwartz. AG, Berlin, Germany) remains a classic example of a pulsa-tile device. The Impella 2.5 (Abiomed) (Fig. 20-68) has been used in the pediatric population as a temporary support device for recovering myocarditis, during treatment of acute rejection after heart transplantation and high-risk interventions in frag-ile patients with marginal function.181,182 Other continuous flow devices available for the pediatric patient include the Heartmate II Figure 20-68. Impella 2.5 (Abiomed). (Reproduced with permis-sion from Abiomed. Danvers, MA.)Figure 20-69. Heartware HVAD. (HeartWare® HVAD (Heart-Ware Inc., Miami Lakes, FL.)and Heartmate III devices (Thoratec, Pleasanton, CA), DeBakey VAD Child (MicroMed Technology, Houston, TX), PediMag (Thoratec, Pleasanton, CA), Jarvik2015 and HeartWare HVAD (Fig 20-69) (HeartWare international Inc, Framingham, MA).183 The total artificial heart (SynCardia Systems Inc, Tuscon, Az, USA) is an implantable biventricular device that replaces both ventricles. With |
Surgery_Schwartz_5264 | Surgery_Schwartz | 20-69) (HeartWare international Inc, Framingham, MA).183 The total artificial heart (SynCardia Systems Inc, Tuscon, Az, USA) is an implantable biventricular device that replaces both ventricles. With the new introduction of the 50 ml pump, its popularity in the pediatric population has risen.Posttransplant survival of patients bridged with and with-out mechanical circulatory support (ventricular assist device or total artificial heart) at 5 years post transplant remains the same. However, patients bridged to transplant with ECMO have a sig-nificantly worse survival.184 All in all, the field of pediatric heart surgery is very exciting and rapidly expanding.Pediatric Heart TransplantationHeart transplantation is currently an accepted mode of therapy in infants and children. Annually, about 600 pediatric heart transplants are performed worldwide,184 about 400 of which are performed in the United States.185 The common indications for heart transplant in the pediatric population are | Surgery_Schwartz. 20-69) (HeartWare international Inc, Framingham, MA).183 The total artificial heart (SynCardia Systems Inc, Tuscon, Az, USA) is an implantable biventricular device that replaces both ventricles. With the new introduction of the 50 ml pump, its popularity in the pediatric population has risen.Posttransplant survival of patients bridged with and with-out mechanical circulatory support (ventricular assist device or total artificial heart) at 5 years post transplant remains the same. However, patients bridged to transplant with ECMO have a sig-nificantly worse survival.184 All in all, the field of pediatric heart surgery is very exciting and rapidly expanding.Pediatric Heart TransplantationHeart transplantation is currently an accepted mode of therapy in infants and children. Annually, about 600 pediatric heart transplants are performed worldwide,184 about 400 of which are performed in the United States.185 The common indications for heart transplant in the pediatric population are |
Surgery_Schwartz_5265 | Surgery_Schwartz | about 600 pediatric heart transplants are performed worldwide,184 about 400 of which are performed in the United States.185 The common indications for heart transplant in the pediatric population are congenital heart disease, dilated cardiomyopathy, retransplantation, and other rare indications (e.g., arrhythmogenic right ventricular dysplasia, cancer, muscular dystrophy, and restrictive cardio-myopathy). The most common congenital heart defect requir-ing transplantation remains hypoplastic left heart syndrome. Although in the past some centers have advocated primary heart transplantation for this lesion, the improved outcomes with surgical palliation have eliminated this as an option. The first year post transplant remains the greatest risk for mortality. The overall median survival is 20.7 years for infants, 18.2 years for children age 1 to 5 years, 14 years for age 6 to 10 years, and 12.7 years for those age 11 to 17 years.184 Males seem to have a modestly superior overall survival | Surgery_Schwartz. about 600 pediatric heart transplants are performed worldwide,184 about 400 of which are performed in the United States.185 The common indications for heart transplant in the pediatric population are congenital heart disease, dilated cardiomyopathy, retransplantation, and other rare indications (e.g., arrhythmogenic right ventricular dysplasia, cancer, muscular dystrophy, and restrictive cardio-myopathy). The most common congenital heart defect requir-ing transplantation remains hypoplastic left heart syndrome. Although in the past some centers have advocated primary heart transplantation for this lesion, the improved outcomes with surgical palliation have eliminated this as an option. The first year post transplant remains the greatest risk for mortality. The overall median survival is 20.7 years for infants, 18.2 years for children age 1 to 5 years, 14 years for age 6 to 10 years, and 12.7 years for those age 11 to 17 years.184 Males seem to have a modestly superior overall survival |
Surgery_Schwartz_5266 | Surgery_Schwartz | years for infants, 18.2 years for children age 1 to 5 years, 14 years for age 6 to 10 years, and 12.7 years for those age 11 to 17 years.184 Males seem to have a modestly superior overall survival compared with females. The causes of mortality include cardiac allograft vasculopathy, acute Brunicardi_Ch20_p0751-p0800.indd 79122/02/19 2:57 PM 792SPECIFIC CONSIDERATIONSPART IIrejection, infections, and graft failures. In the current era, the expected 1-year survival rate is 80% to 90%, the 2-year survival rate is 80% to 85%, and the 5-year survival rate is approximately 70% to 80% in experienced centers.186 Interestingly, infants who undergo transplantation in the first month of life appear to have a survival advantage over infants who undergo transplantation during the remainder of the first year of life.The two main techniques for performing the implant of the heart are the right atrial technique developed by Lower and Shumway and the bicaval-left atrial technique described by | Surgery_Schwartz. years for infants, 18.2 years for children age 1 to 5 years, 14 years for age 6 to 10 years, and 12.7 years for those age 11 to 17 years.184 Males seem to have a modestly superior overall survival compared with females. The causes of mortality include cardiac allograft vasculopathy, acute Brunicardi_Ch20_p0751-p0800.indd 79122/02/19 2:57 PM 792SPECIFIC CONSIDERATIONSPART IIrejection, infections, and graft failures. In the current era, the expected 1-year survival rate is 80% to 90%, the 2-year survival rate is 80% to 85%, and the 5-year survival rate is approximately 70% to 80% in experienced centers.186 Interestingly, infants who undergo transplantation in the first month of life appear to have a survival advantage over infants who undergo transplantation during the remainder of the first year of life.The two main techniques for performing the implant of the heart are the right atrial technique developed by Lower and Shumway and the bicaval-left atrial technique described by |
Surgery_Schwartz_5267 | Surgery_Schwartz | the first year of life.The two main techniques for performing the implant of the heart are the right atrial technique developed by Lower and Shumway and the bicaval-left atrial technique described by Sievers and associates.187 In the latter technique, implantation consists of five anastamoses performed using a running prolene suture. These include the left atrial cuff, aorta, pulmonary artery, and the superior and inferior vena cave. One of the cornerstones of postoperative management remains immunosuppression. The triple drug regimen remains popular, corticosteroids, calcineurin inhibitor (cyclosporine or tacrolimus), and an antiproliferative agent (azathioprine or mycophenolate mofetil). Endomyocardial biopsy and coronary angiography are performed at regular inter-vals to monitor rejection. The field of pediatric heart transplan-tation has made huge strides since the days of “Baby Fae.”188,189Public Reporting and the STS Database in Congenital Heart SurgeryThere has been a recent | Surgery_Schwartz. the first year of life.The two main techniques for performing the implant of the heart are the right atrial technique developed by Lower and Shumway and the bicaval-left atrial technique described by Sievers and associates.187 In the latter technique, implantation consists of five anastamoses performed using a running prolene suture. These include the left atrial cuff, aorta, pulmonary artery, and the superior and inferior vena cave. One of the cornerstones of postoperative management remains immunosuppression. The triple drug regimen remains popular, corticosteroids, calcineurin inhibitor (cyclosporine or tacrolimus), and an antiproliferative agent (azathioprine or mycophenolate mofetil). Endomyocardial biopsy and coronary angiography are performed at regular inter-vals to monitor rejection. The field of pediatric heart transplan-tation has made huge strides since the days of “Baby Fae.”188,189Public Reporting and the STS Database in Congenital Heart SurgeryThere has been a recent |
Surgery_Schwartz_5268 | Surgery_Schwartz | The field of pediatric heart transplan-tation has made huge strides since the days of “Baby Fae.”188,189Public Reporting and the STS Database in Congenital Heart SurgeryThere has been a recent impetus in the filed of congenital and pediatric cardiac surgery toward public reporting of out-comes. The advantages of this include promoting patient autonomy, shows a commitment to quality improvement, and also serves as a free marketing tool. The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD), is the largest clinical database in the world for congenital and pedi-atric cardiac surgery. It was founded in 1994. It contains data of about 394,980 operations as of September 9, 2016.192 These data are the foundation for assessment of performance by benchmark and comparison of individual programmatic outcomes to national aggregate data, development and subsequent applica-tion of sophisticated risk adjustment models, quality improve-ment initiatives, research, voluntary | Surgery_Schwartz. The field of pediatric heart transplan-tation has made huge strides since the days of “Baby Fae.”188,189Public Reporting and the STS Database in Congenital Heart SurgeryThere has been a recent impetus in the filed of congenital and pediatric cardiac surgery toward public reporting of out-comes. The advantages of this include promoting patient autonomy, shows a commitment to quality improvement, and also serves as a free marketing tool. The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD), is the largest clinical database in the world for congenital and pedi-atric cardiac surgery. It was founded in 1994. It contains data of about 394,980 operations as of September 9, 2016.192 These data are the foundation for assessment of performance by benchmark and comparison of individual programmatic outcomes to national aggregate data, development and subsequent applica-tion of sophisticated risk adjustment models, quality improve-ment initiatives, research, voluntary |
Surgery_Schwartz_5269 | Surgery_Schwartz | of individual programmatic outcomes to national aggregate data, development and subsequent applica-tion of sophisticated risk adjustment models, quality improve-ment initiatives, research, voluntary public reporting, development of reimbursement strategies, and governmental and regulatory collaborations.190 The database is currently in its 25th overall data harvest and records and represents data from 120 participants and 392 surgeons. Thus, this database has greater than 95% penetrance. STS CHSD public reporting started in January 2015, and participation is voluntary. Report-ing is restricted to the hospital level and involves a rolling 4-year analytic window of data. Public reporting is based on the STS CHSD Operative Mortality Risk Model. Developed in 2014, this risk model calculates the operative mortality rate of hospitals performing such surgery, adjusting for procedural and patient level factors. The overall mortality rate over a 4-year period and the operative mortality rate | Surgery_Schwartz. of individual programmatic outcomes to national aggregate data, development and subsequent applica-tion of sophisticated risk adjustment models, quality improve-ment initiatives, research, voluntary public reporting, development of reimbursement strategies, and governmental and regulatory collaborations.190 The database is currently in its 25th overall data harvest and records and represents data from 120 participants and 392 surgeons. Thus, this database has greater than 95% penetrance. STS CHSD public reporting started in January 2015, and participation is voluntary. Report-ing is restricted to the hospital level and involves a rolling 4-year analytic window of data. Public reporting is based on the STS CHSD Operative Mortality Risk Model. Developed in 2014, this risk model calculates the operative mortality rate of hospitals performing such surgery, adjusting for procedural and patient level factors. The overall mortality rate over a 4-year period and the operative mortality rate |
Surgery_Schwartz_5270 | Surgery_Schwartz | the operative mortality rate of hospitals performing such surgery, adjusting for procedural and patient level factors. The overall mortality rate over a 4-year period and the operative mortality rate for each of the five STAT (Society of Thoracic Surgeons—European Association for Cardio-Thoracic Surgery) categories is reported. The STAT categories are a multi-institutional, validated complexity stratification tool. They range from a score of 1 to 5, and the risk of mortality increases with each category.190 In addition, the STS star rating system was introduced, and every institution is rated as one, two, or three stars. This system is based on the confidence limits of the O/E (observed to expected) overall mortality for the institu-tion (Fig. 20-70). One star equals higher than expected operative 9Rady Children’s Hospital San DiegoRady Children’s Hospital San Diego SurgeonsEnc Devaney, MDDaniel DiBardino, MDJohn Lambert, MDPeter Pastuszko, MDOverall Star RatingPopulation: | Surgery_Schwartz. the operative mortality rate of hospitals performing such surgery, adjusting for procedural and patient level factors. The overall mortality rate over a 4-year period and the operative mortality rate for each of the five STAT (Society of Thoracic Surgeons—European Association for Cardio-Thoracic Surgery) categories is reported. The STAT categories are a multi-institutional, validated complexity stratification tool. They range from a score of 1 to 5, and the risk of mortality increases with each category.190 In addition, the STS star rating system was introduced, and every institution is rated as one, two, or three stars. This system is based on the confidence limits of the O/E (observed to expected) overall mortality for the institu-tion (Fig. 20-70). One star equals higher than expected operative 9Rady Children’s Hospital San DiegoRady Children’s Hospital San Diego SurgeonsEnc Devaney, MDDaniel DiBardino, MDJohn Lambert, MDPeter Pastuszko, MDOverall Star RatingPopulation: |
Surgery_Schwartz_5271 | Surgery_Schwartz | expected operative 9Rady Children’s Hospital San DiegoRady Children’s Hospital San Diego SurgeonsEnc Devaney, MDDaniel DiBardino, MDJohn Lambert, MDPeter Pastuszko, MDOverall Star RatingPopulation: Neonates,Infants, Children & AdultsOvarallSTAT Mortality Category 1STAT Mortality Category 2STAT Mortality Category 3STAT Mortality Category 4STAT Mortality Category 5#/Eligible28/11650/3067/3991/12817/2953/37Observed2.4%0.0%1.8%0.8%5.8%8.1%Expected3.0%0.6%1.5%2.1%6.7%14.7%OE (95% CI)0.79 (0.53, 1.14)0.00 (0.00, 2.16)1.20 (0.48, 2.45)0.37 (0.01, 2.05)0.87 (0.51, 1.36)0.55 (0.12, 1.49)Adj. Rate (95% CI)2.5 (1.6, 3.5)0.0 (0.0, 1.1)2.0 (0.8, 4.1)1.0 (0.0, 5.3)6.0 (3.5, 9.4)8.7 (1.8, 23.6)San DiegoCAWebsite: http://www.rchsd.org/programs-services/cardiologyOperative and Adjusted Operative Mortality, Last 4 Years (January 2012–December 2015)Figure 20-70. Program performance as currently reported by the STS-CHSD.Brunicardi_Ch20_p0751-p0800.indd 79222/02/19 2:57 PM 793CONGENITAL HEART | Surgery_Schwartz. expected operative 9Rady Children’s Hospital San DiegoRady Children’s Hospital San Diego SurgeonsEnc Devaney, MDDaniel DiBardino, MDJohn Lambert, MDPeter Pastuszko, MDOverall Star RatingPopulation: Neonates,Infants, Children & AdultsOvarallSTAT Mortality Category 1STAT Mortality Category 2STAT Mortality Category 3STAT Mortality Category 4STAT Mortality Category 5#/Eligible28/11650/3067/3991/12817/2953/37Observed2.4%0.0%1.8%0.8%5.8%8.1%Expected3.0%0.6%1.5%2.1%6.7%14.7%OE (95% CI)0.79 (0.53, 1.14)0.00 (0.00, 2.16)1.20 (0.48, 2.45)0.37 (0.01, 2.05)0.87 (0.51, 1.36)0.55 (0.12, 1.49)Adj. Rate (95% CI)2.5 (1.6, 3.5)0.0 (0.0, 1.1)2.0 (0.8, 4.1)1.0 (0.0, 5.3)6.0 (3.5, 9.4)8.7 (1.8, 23.6)San DiegoCAWebsite: http://www.rchsd.org/programs-services/cardiologyOperative and Adjusted Operative Mortality, Last 4 Years (January 2012–December 2015)Figure 20-70. Program performance as currently reported by the STS-CHSD.Brunicardi_Ch20_p0751-p0800.indd 79222/02/19 2:57 PM 793CONGENITAL HEART |
Surgery_Schwartz_5272 | Surgery_Schwartz | Last 4 Years (January 2012–December 2015)Figure 20-70. Program performance as currently reported by the STS-CHSD.Brunicardi_Ch20_p0751-p0800.indd 79222/02/19 2:57 PM 793CONGENITAL HEART DISEASECHAPTER 20mortality (the 95% confidence interval for their risk-adjusted O/E mortality ratio was entirely above the number 1), two stars equals the same as expected operative mortality (the 95% con-fidence interval for their risk-adjusted O/E mortality ratio over-lapped with the number 1), and three stars equals lower than expected operative mortality (the 95% confidence interval for their risk-adjusted O/E mortality ratio was entirely below the number 1). The Spring 2016 STS CHSD Feedback Report includes data from 117 participants in the STS-CHSD, including 14 one-star programs, 83 two-star programs, and 8 three-star programs. Twelve participants did not receive a star rating due to incomplete data.191 Public reporting increased from 23% to 57.6% (all three-star programs, 50 two-star and | Surgery_Schwartz. Last 4 Years (January 2012–December 2015)Figure 20-70. Program performance as currently reported by the STS-CHSD.Brunicardi_Ch20_p0751-p0800.indd 79222/02/19 2:57 PM 793CONGENITAL HEART DISEASECHAPTER 20mortality (the 95% confidence interval for their risk-adjusted O/E mortality ratio was entirely above the number 1), two stars equals the same as expected operative mortality (the 95% con-fidence interval for their risk-adjusted O/E mortality ratio over-lapped with the number 1), and three stars equals lower than expected operative mortality (the 95% confidence interval for their risk-adjusted O/E mortality ratio was entirely below the number 1). The Spring 2016 STS CHSD Feedback Report includes data from 117 participants in the STS-CHSD, including 14 one-star programs, 83 two-star programs, and 8 three-star programs. Twelve participants did not receive a star rating due to incomplete data.191 Public reporting increased from 23% to 57.6% (all three-star programs, 50 two-star and |
Surgery_Schwartz_5273 | Surgery_Schwartz | and 8 three-star programs. Twelve participants did not receive a star rating due to incomplete data.191 Public reporting increased from 23% to 57.6% (all three-star programs, 50 two-star and three one-star programs). The online public reporting portal can be accessed at www.sts.org/congenital-public-reporting-module-search.There are several criticisms to the current methodology used for reporting. Important limitations of current publicly reported data (including the STS star rating system) will need to be addressed in future initiatives in order to completely engage parents of children with CHD and reassure providers that risk-adjustment models are optimized. There are four spe-cific areas that should be considered when making decisions how to improve this methodology: (a) While the mortality risk-adjustment model on which the star rating system is based is mature now, there are not comparable models that provide risk-adjusted morbidity (complication) rates. The assessment of the | Surgery_Schwartz. and 8 three-star programs. Twelve participants did not receive a star rating due to incomplete data.191 Public reporting increased from 23% to 57.6% (all three-star programs, 50 two-star and three one-star programs). The online public reporting portal can be accessed at www.sts.org/congenital-public-reporting-module-search.There are several criticisms to the current methodology used for reporting. Important limitations of current publicly reported data (including the STS star rating system) will need to be addressed in future initiatives in order to completely engage parents of children with CHD and reassure providers that risk-adjustment models are optimized. There are four spe-cific areas that should be considered when making decisions how to improve this methodology: (a) While the mortality risk-adjustment model on which the star rating system is based is mature now, there are not comparable models that provide risk-adjusted morbidity (complication) rates. The assessment of the |
Surgery_Schwartz_5274 | Surgery_Schwartz | risk-adjustment model on which the star rating system is based is mature now, there are not comparable models that provide risk-adjusted morbidity (complication) rates. The assessment of the quality of congenital heart disease care at different centers should include complication metrics and incorporate failure-to-rescue as an important discriminator; (b) the star rating system does not provide risk-adjusted outcomes for specific procedures or, more importantly, for specific diagnoses. This is mainly because of the exceptionally wide spectrum of diagnoses and procedures in pediatric cardiac surgery that preclude sufficiently large numbers in most procedure-specific categories; (c) the star rating system, although the “best” we have at present, may not be understood equally by all families. It will be critical to provide equivalent information to the large numbers of under-resourced and non–English-speaking families; (d) finally, the current adjusted mortality rate reported by the STS | Surgery_Schwartz. risk-adjustment model on which the star rating system is based is mature now, there are not comparable models that provide risk-adjusted morbidity (complication) rates. The assessment of the quality of congenital heart disease care at different centers should include complication metrics and incorporate failure-to-rescue as an important discriminator; (b) the star rating system does not provide risk-adjusted outcomes for specific procedures or, more importantly, for specific diagnoses. This is mainly because of the exceptionally wide spectrum of diagnoses and procedures in pediatric cardiac surgery that preclude sufficiently large numbers in most procedure-specific categories; (c) the star rating system, although the “best” we have at present, may not be understood equally by all families. It will be critical to provide equivalent information to the large numbers of under-resourced and non–English-speaking families; (d) finally, the current adjusted mortality rate reported by the STS |
Surgery_Schwartz_5275 | Surgery_Schwartz | It will be critical to provide equivalent information to the large numbers of under-resourced and non–English-speaking families; (d) finally, the current adjusted mortality rate reported by the STS is calculated from a statistical formula and refers to what the hospital’s mor-tality rate would be if the measured performance (in this case the mortality rate) were extrapolated to the overall case-mix or make-up of patients within the entire STS database. This is a critical point because a hospital’s case-mix is highly variable, and discrimination based on mortality is mostly related to out-comes of more complex procedures. In other words, if hospital A has excellent survival for less complex procedures and therefore performs very few highly complex procedures (i.e., choosing a case-mix consistent with its expertise), the application of an extrapolated mortality rate may not reflect the actual quality of care for that particular hospital. This issue is evident because the majority of | Surgery_Schwartz. It will be critical to provide equivalent information to the large numbers of under-resourced and non–English-speaking families; (d) finally, the current adjusted mortality rate reported by the STS is calculated from a statistical formula and refers to what the hospital’s mor-tality rate would be if the measured performance (in this case the mortality rate) were extrapolated to the overall case-mix or make-up of patients within the entire STS database. This is a critical point because a hospital’s case-mix is highly variable, and discrimination based on mortality is mostly related to out-comes of more complex procedures. In other words, if hospital A has excellent survival for less complex procedures and therefore performs very few highly complex procedures (i.e., choosing a case-mix consistent with its expertise), the application of an extrapolated mortality rate may not reflect the actual quality of care for that particular hospital. This issue is evident because the majority of |
Surgery_Schwartz_5276 | Surgery_Schwartz | with its expertise), the application of an extrapolated mortality rate may not reflect the actual quality of care for that particular hospital. This issue is evident because the majority of experienced centers with arguably the highest complexity received a “middle star” rating of 2. This rating may reflect calibration issues with the current rating system, whereby centers are potentially penalized for high-complexity predominance.Fortunately, there are efforts to correct these deficien-cies. In 2016, the STS CHSD Task Force and STS Quality Measurement Task Force began to collaborate on an initiative to refine risk adjustment for chromosomal abnormalities, syn-dromes, and noncardiac congenital anatomic abnormalities and to then enhance the STS CHSD Mortality Risk Model with this additional information. Upon completion of this project, STS CHSD Task Force plans to collaborate with the STS Quality Measurement Task Force to study the relationship between vol-ume (programmatic volume and | Surgery_Schwartz. with its expertise), the application of an extrapolated mortality rate may not reflect the actual quality of care for that particular hospital. This issue is evident because the majority of experienced centers with arguably the highest complexity received a “middle star” rating of 2. This rating may reflect calibration issues with the current rating system, whereby centers are potentially penalized for high-complexity predominance.Fortunately, there are efforts to correct these deficien-cies. In 2016, the STS CHSD Task Force and STS Quality Measurement Task Force began to collaborate on an initiative to refine risk adjustment for chromosomal abnormalities, syn-dromes, and noncardiac congenital anatomic abnormalities and to then enhance the STS CHSD Mortality Risk Model with this additional information. Upon completion of this project, STS CHSD Task Force plans to collaborate with the STS Quality Measurement Task Force to study the relationship between vol-ume (programmatic volume and |
Surgery_Schwartz_5277 | Surgery_Schwartz | information. Upon completion of this project, STS CHSD Task Force plans to collaborate with the STS Quality Measurement Task Force to study the relationship between vol-ume (programmatic volume and surgeon volume) and outcome using this enhanced STS CHSD Mortality Risk Model.192 Also, currently under development is a multidomain quality metric that incorporates mortality, morbidity, postoperative length of stay, and the occurrence of complications. As the largest con-genital and pediatric cardiac surgical clinical data registry in the world, containing data about nearly all pediatric cardiac operations performed in the United States, STS CHSD contains a truly representative sample of national aggregate data that is useful for multiple purposes.192Future DirectionsThe future of congenital heart surgery remains very bright and exciting. The development of novel technologies such as four-dimensional MRI flow studies (Fig. 20-71) and three-dimen-sional printing have offered this field | Surgery_Schwartz. information. Upon completion of this project, STS CHSD Task Force plans to collaborate with the STS Quality Measurement Task Force to study the relationship between vol-ume (programmatic volume and surgeon volume) and outcome using this enhanced STS CHSD Mortality Risk Model.192 Also, currently under development is a multidomain quality metric that incorporates mortality, morbidity, postoperative length of stay, and the occurrence of complications. As the largest con-genital and pediatric cardiac surgical clinical data registry in the world, containing data about nearly all pediatric cardiac operations performed in the United States, STS CHSD contains a truly representative sample of national aggregate data that is useful for multiple purposes.192Future DirectionsThe future of congenital heart surgery remains very bright and exciting. The development of novel technologies such as four-dimensional MRI flow studies (Fig. 20-71) and three-dimen-sional printing have offered this field |
Surgery_Schwartz_5278 | Surgery_Schwartz | heart surgery remains very bright and exciting. The development of novel technologies such as four-dimensional MRI flow studies (Fig. 20-71) and three-dimen-sional printing have offered this field several new tools to help understand complex anatomy and pathophysiology. Three-dimensional printing of complex congenital heart defects has helped surgeons in preoperative planning by allowing transla-tion of two-dimensional cross-sectional imaging studies into a tangible and easily visualized model.193 The hollow nature of the human heart and the direct correlation of structure to disease in the congenital population allows this technology to be used in abundance in this field. Its utilization to train young surgeons is very appealing (Figs. 20-72 and 20-73).194,196 Current research in the field of genetics, device bioengineering and miniaturization, stem cell therapy, and fusion imaging technology is expected to further improve patient outcome.195,198 The improved outcomes and survival of | Surgery_Schwartz. heart surgery remains very bright and exciting. The development of novel technologies such as four-dimensional MRI flow studies (Fig. 20-71) and three-dimen-sional printing have offered this field several new tools to help understand complex anatomy and pathophysiology. Three-dimensional printing of complex congenital heart defects has helped surgeons in preoperative planning by allowing transla-tion of two-dimensional cross-sectional imaging studies into a tangible and easily visualized model.193 The hollow nature of the human heart and the direct correlation of structure to disease in the congenital population allows this technology to be used in abundance in this field. Its utilization to train young surgeons is very appealing (Figs. 20-72 and 20-73).194,196 Current research in the field of genetics, device bioengineering and miniaturization, stem cell therapy, and fusion imaging technology is expected to further improve patient outcome.195,198 The improved outcomes and survival of |
Surgery_Schwartz_5279 | Surgery_Schwartz | of genetics, device bioengineering and miniaturization, stem cell therapy, and fusion imaging technology is expected to further improve patient outcome.195,198 The improved outcomes and survival of these young and fragile patients with congeni-tal heart disease has led to the development of a complex new field termed adult congenital heart disease. The field of con-genital heart surgery is young and offers brilliant, motivated, and upcoming surgeons a very daunting challenge to better the future of these babies.Figure 20-71. 4D MRI flow study obtained in a complex single ventricle patient for the evaluation of persistent hypoxia.Brunicardi_Ch20_p0751-p0800.indd 79322/02/19 2:57 PM 794SPECIFIC CONSIDERATIONSPART IIFigure 20-72. 3D printed models of complex heart defects which were very helpful for preoperative surgical planning and patient education.ADEFBCMAPCAPulmonaryArteryAortaFigure 20-73. Example of Pre-Interventional Planning Using 3D Printed Models. Transthoracic | Surgery_Schwartz. of genetics, device bioengineering and miniaturization, stem cell therapy, and fusion imaging technology is expected to further improve patient outcome.195,198 The improved outcomes and survival of these young and fragile patients with congeni-tal heart disease has led to the development of a complex new field termed adult congenital heart disease. The field of con-genital heart surgery is young and offers brilliant, motivated, and upcoming surgeons a very daunting challenge to better the future of these babies.Figure 20-71. 4D MRI flow study obtained in a complex single ventricle patient for the evaluation of persistent hypoxia.Brunicardi_Ch20_p0751-p0800.indd 79322/02/19 2:57 PM 794SPECIFIC CONSIDERATIONSPART IIFigure 20-72. 3D printed models of complex heart defects which were very helpful for preoperative surgical planning and patient education.ADEFBCMAPCAPulmonaryArteryAortaFigure 20-73. Example of Pre-Interventional Planning Using 3D Printed Models. Transthoracic |
Surgery_Schwartz_5280 | Surgery_Schwartz | were very helpful for preoperative surgical planning and patient education.ADEFBCMAPCAPulmonaryArteryAortaFigure 20-73. Example of Pre-Interventional Planning Using 3D Printed Models. Transthoracic echocardiogram (A) confirms tetralogy of Fallot/pulmonary atresia/multiple aortopulmonary collateral arteries (MAPCAs) diagnosis. Three-dimensional (3D) reconstruction (B and C) illustrates spatial relationship of patient-specific geometry such as true pulmonary arteries (blue), aorta (red), and MAPCAs (green and yellow) for central aortopulmonary shunt placement and coil planning. Three-dimensional printing (D) provides absolute scaling for planning purposes, as well as patient/family education. Angiography (E and F) captured after central shunt and prior to placement of MAPCA embolization coils. (Reproduced with permission from Ryan JR, Moe TG, Richardson R, et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary | Surgery_Schwartz. were very helpful for preoperative surgical planning and patient education.ADEFBCMAPCAPulmonaryArteryAortaFigure 20-73. Example of Pre-Interventional Planning Using 3D Printed Models. Transthoracic echocardiogram (A) confirms tetralogy of Fallot/pulmonary atresia/multiple aortopulmonary collateral arteries (MAPCAs) diagnosis. Three-dimensional (3D) reconstruction (B and C) illustrates spatial relationship of patient-specific geometry such as true pulmonary arteries (blue), aorta (red), and MAPCAs (green and yellow) for central aortopulmonary shunt placement and coil planning. Three-dimensional printing (D) provides absolute scaling for planning purposes, as well as patient/family education. Angiography (E and F) captured after central shunt and prior to placement of MAPCA embolization coils. (Reproduced with permission from Ryan JR, Moe TG, Richardson R, et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary |
Surgery_Schwartz_5281 | Surgery_Schwartz | coils. (Reproduced with permission from Ryan JR, Moe TG, Richardson R, et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals, JACC Cardiovasc Imaging. 2015 Jan;8(1):103-104.)Brunicardi_Ch20_p0751-p0800.indd 79422/02/19 2:57 PM 795CONGENITAL HEART DISEASECHAPTER 20REFERENCESEntries highlighted in bright blue are key references. 1. American Heart Association. About congenital heart defects. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/Congenital-Heart-Defects_UCM_001090_SubHomePage.jsp. Accessed May 18, 2018. 2. Congenital Heart Public Health Consortium. FAQ fact sheet. Available at: https://www.aap.org/en-us/Documents/chphc/chd_fact_sheet_long.pdf. Accessed May 18, 2018. 3. Society for Thoracic Surgeons. Congenital heart surgery pub-lic reporting. Available at: https://www.sts.org/congenital-public-reporting-module-search. Accessed May 18, 2018. 4. Kouchoukos NT, | Surgery_Schwartz. coils. (Reproduced with permission from Ryan JR, Moe TG, Richardson R, et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals, JACC Cardiovasc Imaging. 2015 Jan;8(1):103-104.)Brunicardi_Ch20_p0751-p0800.indd 79422/02/19 2:57 PM 795CONGENITAL HEART DISEASECHAPTER 20REFERENCESEntries highlighted in bright blue are key references. 1. American Heart Association. About congenital heart defects. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/Congenital-Heart-Defects_UCM_001090_SubHomePage.jsp. Accessed May 18, 2018. 2. Congenital Heart Public Health Consortium. FAQ fact sheet. Available at: https://www.aap.org/en-us/Documents/chphc/chd_fact_sheet_long.pdf. Accessed May 18, 2018. 3. Society for Thoracic Surgeons. Congenital heart surgery pub-lic reporting. Available at: https://www.sts.org/congenital-public-reporting-module-search. Accessed May 18, 2018. 4. Kouchoukos NT, |
Surgery_Schwartz_5282 | Surgery_Schwartz | for Thoracic Surgeons. Congenital heart surgery pub-lic reporting. Available at: https://www.sts.org/congenital-public-reporting-module-search. Accessed May 18, 2018. 4. Kouchoukos NT, Blackstone EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:716. 5. Kirklin JW, Pacifico AD, Kirklin JK. The surgical treat-ment of atrioventricular canal defects. In: Arciniegas E, ed. Pediatric Cardiac Surgery. Chicago: Yearbook Medical; 1985:2398. 6. Peterson GE, Brickner ME, Reimold SC. Transesophageal echocardiography: clinical indications and applications. Circulation. 2003;107:2398-2402. 7. Kouchoukos NT, Blackstone EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. | Surgery_Schwartz. for Thoracic Surgeons. Congenital heart surgery pub-lic reporting. Available at: https://www.sts.org/congenital-public-reporting-module-search. Accessed May 18, 2018. 4. Kouchoukos NT, Blackstone EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:716. 5. Kirklin JW, Pacifico AD, Kirklin JK. The surgical treat-ment of atrioventricular canal defects. In: Arciniegas E, ed. Pediatric Cardiac Surgery. Chicago: Yearbook Medical; 1985:2398. 6. Peterson GE, Brickner ME, Reimold SC. Transesophageal echocardiography: clinical indications and applications. Circulation. 2003;107:2398-2402. 7. Kouchoukos NT, Blackstone EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. |
Surgery_Schwartz_5283 | Surgery_Schwartz | EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:740. 8. Reddy VM. Cardiac surgery for premature and low birth weight neonates. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2001;4:271-276. Congenital heart defects in low-birth-weight infants are typically managed with sup-portive therapy or palliative surgery, and definitive repair is delayed. This paper describes the outcomes in 116 neonates and infants under 2500 g who underwent complete repair of simple and complex cardiac defects using cardiopulmo-nary bypass. 9. Thompson JD, Abuwari EH, Watterson KG, et al. Surgi-cal and transcatheter (Amplatzer) closure of atrial septal defect: a prospective comparison of results and cost. Heart. 2002;87:466-469. 10. Du ZD, Hijazi ZM, Kleinman CS, et al. Comparison between transcatheter and surgical | Surgery_Schwartz. EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:740. 8. Reddy VM. Cardiac surgery for premature and low birth weight neonates. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2001;4:271-276. Congenital heart defects in low-birth-weight infants are typically managed with sup-portive therapy or palliative surgery, and definitive repair is delayed. This paper describes the outcomes in 116 neonates and infants under 2500 g who underwent complete repair of simple and complex cardiac defects using cardiopulmo-nary bypass. 9. Thompson JD, Abuwari EH, Watterson KG, et al. Surgi-cal and transcatheter (Amplatzer) closure of atrial septal defect: a prospective comparison of results and cost. Heart. 2002;87:466-469. 10. Du ZD, Hijazi ZM, Kleinman CS, et al. Comparison between transcatheter and surgical |
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Surgery_Schwartz_5291 | Surgery_Schwartz | Surg. 1991;51:1031-1039. 37. McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, Hanley FL. Issues and outcomes in the management of supravalvular aortic stenosis. Ann Thorac Surg. 2000;69(2): 562-567. 38. Clyman RI, Mauray F, Roman C, Rudolph AM, Heymann MA. Circulating PGE2 concentration and patent ductus arteriosus in fetal and neonatal lambs. J Pediatr. 1982;97(3):455-463. 39. McMurphy DM, Heymann MA, Rudolph AM, Melmon KL. Developmental change in constriction of the ductus arteriosus: response to oxygen and vasoactive substances in the isolated duc-tus arteriosus of the fetal lamb. Pediatr Res. 1972;6(4):231-238. 40. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circu-lation. 1971;43(3):323-332. 40. Campbell M. Natural history of persistent ductus arteriosus. Br Heart J. 1968;30(1):4-13. 41. Itabashi K, Ohno T, Nishida H. Indomethacin responsive-ness of patent ductus arteriosus and renal abnormalities in | Surgery_Schwartz. Surg. 1991;51:1031-1039. 37. McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, Hanley FL. Issues and outcomes in the management of supravalvular aortic stenosis. Ann Thorac Surg. 2000;69(2): 562-567. 38. Clyman RI, Mauray F, Roman C, Rudolph AM, Heymann MA. Circulating PGE2 concentration and patent ductus arteriosus in fetal and neonatal lambs. J Pediatr. 1982;97(3):455-463. 39. McMurphy DM, Heymann MA, Rudolph AM, Melmon KL. Developmental change in constriction of the ductus arteriosus: response to oxygen and vasoactive substances in the isolated duc-tus arteriosus of the fetal lamb. Pediatr Res. 1972;6(4):231-238. 40. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circu-lation. 1971;43(3):323-332. 40. Campbell M. Natural history of persistent ductus arteriosus. Br Heart J. 1968;30(1):4-13. 41. Itabashi K, Ohno T, Nishida H. Indomethacin responsive-ness of patent ductus arteriosus and renal abnormalities in |
Surgery_Schwartz_5292 | Surgery_Schwartz | M. Natural history of persistent ductus arteriosus. Br Heart J. 1968;30(1):4-13. 41. Itabashi K, Ohno T, Nishida H. Indomethacin responsive-ness of patent ductus arteriosus and renal abnormalities in preterm infants treated with indomethacin. J Pediatr. 2003;143(2):203-207. 42. Rashkind WJ, Cuaso CC. Transcatheter closure of patent duc-tus arteriosus. Pediatr Cardiol. 1979;1(1):3-7. 43. Moore JW, Schneider DJ, Dimeglio D. The duct-occlud device: design, clinical results, and future directions. J Interv Cardiol. 2001;14(2):231-237. 44. Zahn EM, Peck D, Phillips A, et al. Transcatheter closure of patent ductus arteriosus in extremely premature newborns: early results and midterm follow-up. JACC Cardiovasc Interv. 2016;9(23):2429-2437. This article shows that percutaneous closure of PDAs even in extremely small babies is possible. 45. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilation of congenital aor-tic stenosis: predictors of success. J Am | Surgery_Schwartz. M. Natural history of persistent ductus arteriosus. Br Heart J. 1968;30(1):4-13. 41. Itabashi K, Ohno T, Nishida H. Indomethacin responsive-ness of patent ductus arteriosus and renal abnormalities in preterm infants treated with indomethacin. J Pediatr. 2003;143(2):203-207. 42. Rashkind WJ, Cuaso CC. Transcatheter closure of patent duc-tus arteriosus. Pediatr Cardiol. 1979;1(1):3-7. 43. Moore JW, Schneider DJ, Dimeglio D. The duct-occlud device: design, clinical results, and future directions. J Interv Cardiol. 2001;14(2):231-237. 44. Zahn EM, Peck D, Phillips A, et al. Transcatheter closure of patent ductus arteriosus in extremely premature newborns: early results and midterm follow-up. JACC Cardiovasc Interv. 2016;9(23):2429-2437. This article shows that percutaneous closure of PDAs even in extremely small babies is possible. 45. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilation of congenital aor-tic stenosis: predictors of success. J Am |
Surgery_Schwartz_5293 | Surgery_Schwartz | in extremely small babies is possible. 45. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilation of congenital aor-tic stenosis: predictors of success. J Am Coll Cardiol. 1996;27(5):1257-1263. 46. Mavroudis C, Backer CL, Gevitz M. Forty-six years of pat-ent ductus arteriosus division at Children’s Memorial Hospital of Chicago. Standards for comparison. Ann Thorac Surg. 1994;220(3):402-409. 47. Elzenga NJ, Gittenberger-de Groot AC, Oppenheimer-Dekker A. Coarctation and other obstructive arch anoma-lies: their relationship to the ductus arteriosus. Int J Cardiol. 1986;13(3):289-308. 48. Locher JP, Kron IL. Coarctation of the aorta. In: Mavroudis C, Backer CL, eds. Pediatric Cardiac Surgery. St. Louis: Mosby; 1994:167. 49. Presbitero P, Demaie D, Villani M, et al. Long-term results (15–30 years) of surgical repair of coarctation. Br Heart J. 1987;57(5):462-467. 50. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta: | Surgery_Schwartz. in extremely small babies is possible. 45. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilation of congenital aor-tic stenosis: predictors of success. J Am Coll Cardiol. 1996;27(5):1257-1263. 46. Mavroudis C, Backer CL, Gevitz M. Forty-six years of pat-ent ductus arteriosus division at Children’s Memorial Hospital of Chicago. Standards for comparison. Ann Thorac Surg. 1994;220(3):402-409. 47. Elzenga NJ, Gittenberger-de Groot AC, Oppenheimer-Dekker A. Coarctation and other obstructive arch anoma-lies: their relationship to the ductus arteriosus. Int J Cardiol. 1986;13(3):289-308. 48. Locher JP, Kron IL. Coarctation of the aorta. In: Mavroudis C, Backer CL, eds. Pediatric Cardiac Surgery. St. Louis: Mosby; 1994:167. 49. Presbitero P, Demaie D, Villani M, et al. Long-term results (15–30 years) of surgical repair of coarctation. Br Heart J. 1987;57(5):462-467. 50. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta: |
Surgery_Schwartz_5294 | Surgery_Schwartz | Villani M, et al. Long-term results (15–30 years) of surgical repair of coarctation. Br Heart J. 1987;57(5):462-467. 50. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta: long-term follow-up and predic-tion of outcome after surgical correction. Circulation. 1989;80(4):840-845. 51. Hornung TS, Benson LN, McLaughlin PR. Interventions for aortic coarctation. Cardiol Rev. 2002;10(3):139-148. 52. Waldhausen JA, Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg. 1966;51(4):532-533. 53. Karamlou T, Bernasconi A, Jaeggi E, et al. Factors associated with arch reintervention and growth of the aortic arch after coarctation repair in neonates weighing less than 2.5 kg. J Thorac Cardiovasc Surg. 2009;137:1163-1167. 54. van Heum LW, Wong CM, Speigelhalter DJ, et al. Surgi-cal treatment of aortic coarctation in infants younger than 3 months: 1985-1990. Success of extended end-to-end arch aor-toplasty. J Thorac Cardiovasc | Surgery_Schwartz. Villani M, et al. Long-term results (15–30 years) of surgical repair of coarctation. Br Heart J. 1987;57(5):462-467. 50. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta: long-term follow-up and predic-tion of outcome after surgical correction. Circulation. 1989;80(4):840-845. 51. Hornung TS, Benson LN, McLaughlin PR. Interventions for aortic coarctation. Cardiol Rev. 2002;10(3):139-148. 52. Waldhausen JA, Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg. 1966;51(4):532-533. 53. Karamlou T, Bernasconi A, Jaeggi E, et al. Factors associated with arch reintervention and growth of the aortic arch after coarctation repair in neonates weighing less than 2.5 kg. J Thorac Cardiovasc Surg. 2009;137:1163-1167. 54. van Heum LW, Wong CM, Speigelhalter DJ, et al. Surgi-cal treatment of aortic coarctation in infants younger than 3 months: 1985-1990. Success of extended end-to-end arch aor-toplasty. J Thorac Cardiovasc |
Surgery_Schwartz_5295 | Surgery_Schwartz | Heum LW, Wong CM, Speigelhalter DJ, et al. Surgi-cal treatment of aortic coarctation in infants younger than 3 months: 1985-1990. Success of extended end-to-end arch aor-toplasty. J Thorac Cardiovasc Surg. 1994;107:74-85. 55. Knyshov GV, Sitar LL, Glagola MD, Atamanyuk MY. Aortic aneurysms at the site of the repair of coarctation of the aorta: a review of 48 patients. Ann Thorac Surg. 1996;61(3):935-939. 56. Bouchart F, Dubar A, Tabley A, et al. Coarctation of the aorta in adults: surgical results and long-term follow-up. Ann Thorac Surg. 2000;70(5):1483-1489. 57. Bhat MA, Neelakhandran KS, Unnikriahnan M, Rathore RS, Mohan Singh MP, Lone GN. Fate of hypertension after repair of coarctation of the aorta in adults. Br J Surg. 2001;88(4):536-538. 58. Acher C, Wynn M. Paraplegia after thoracoabdominal aortic surgery: not just assisted circulation, hypothermic arrest, clamp and sew, or TEVAR. Ann Cardiothorac Surg. 2012;1(3):365-372. 59. McCrindle BW, Jones TK, Morrow WR, et al. Acute | Surgery_Schwartz. Heum LW, Wong CM, Speigelhalter DJ, et al. Surgi-cal treatment of aortic coarctation in infants younger than 3 months: 1985-1990. Success of extended end-to-end arch aor-toplasty. J Thorac Cardiovasc Surg. 1994;107:74-85. 55. Knyshov GV, Sitar LL, Glagola MD, Atamanyuk MY. Aortic aneurysms at the site of the repair of coarctation of the aorta: a review of 48 patients. Ann Thorac Surg. 1996;61(3):935-939. 56. Bouchart F, Dubar A, Tabley A, et al. Coarctation of the aorta in adults: surgical results and long-term follow-up. Ann Thorac Surg. 2000;70(5):1483-1489. 57. Bhat MA, Neelakhandran KS, Unnikriahnan M, Rathore RS, Mohan Singh MP, Lone GN. Fate of hypertension after repair of coarctation of the aorta in adults. Br J Surg. 2001;88(4):536-538. 58. Acher C, Wynn M. Paraplegia after thoracoabdominal aortic surgery: not just assisted circulation, hypothermic arrest, clamp and sew, or TEVAR. Ann Cardiothorac Surg. 2012;1(3):365-372. 59. McCrindle BW, Jones TK, Morrow WR, et al. Acute |
Surgery_Schwartz_5296 | Surgery_Schwartz | aortic surgery: not just assisted circulation, hypothermic arrest, clamp and sew, or TEVAR. Ann Cardiothorac Surg. 2012;1(3):365-372. 59. McCrindle BW, Jones TK, Morrow WR, et al. Acute results of balloon angioplasty of native coarctation versus recurrent aor-tic obstruction are equivalent. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators. J Am Coll Cardiol. 1996;28(7):1810-1817. 60. Egbe A, Uppu S, Lee S, Ho D, Srivastava S. Changing preva-lence of severe congenital heart disease: a population-based study. Pediatr Cardiol. 2014;35(7):1232-1238. 61. Collett RW, Edwards JE. Persistent truncus arteriosus: a clas-sification according to anatomic subtypes. Surg Clin North Am. 1949;29(4):1245-1270. 62. Van Praagh R, Van Praagh S. The anatomy of common aor-ticopulmonary trunk (truncus arteriosus communis) and its embryologic implications: a study of 57 necroscopy cases. Am J Cardiol. 1965;16(3):406-425. 63. De la Cruz MV, Pio da Rocha J. An ontogenic | Surgery_Schwartz. aortic surgery: not just assisted circulation, hypothermic arrest, clamp and sew, or TEVAR. Ann Cardiothorac Surg. 2012;1(3):365-372. 59. McCrindle BW, Jones TK, Morrow WR, et al. Acute results of balloon angioplasty of native coarctation versus recurrent aor-tic obstruction are equivalent. Valvuloplasty and Angioplasty of Congenital Anomalies (VACA) Registry Investigators. J Am Coll Cardiol. 1996;28(7):1810-1817. 60. Egbe A, Uppu S, Lee S, Ho D, Srivastava S. Changing preva-lence of severe congenital heart disease: a population-based study. Pediatr Cardiol. 2014;35(7):1232-1238. 61. Collett RW, Edwards JE. Persistent truncus arteriosus: a clas-sification according to anatomic subtypes. Surg Clin North Am. 1949;29(4):1245-1270. 62. Van Praagh R, Van Praagh S. The anatomy of common aor-ticopulmonary trunk (truncus arteriosus communis) and its embryologic implications: a study of 57 necroscopy cases. Am J Cardiol. 1965;16(3):406-425. 63. De la Cruz MV, Pio da Rocha J. An ontogenic |
Surgery_Schwartz_5297 | Surgery_Schwartz | trunk (truncus arteriosus communis) and its embryologic implications: a study of 57 necroscopy cases. Am J Cardiol. 1965;16(3):406-425. 63. De la Cruz MV, Pio da Rocha J. An ontogenic theory for the explanation of congenital malformations involving the truncus and conus. Am Heart J. 1976;51(5):782-805. 64. Manner J. Cardiac looping in the chick embryo: a morpho-logic review with special reference to terminological and biomechanical aspects of the looping process. Anat Rec. 2000;259(3):242-262. 65. Hutson MR, Kirby ML. Neural crest and cardiovascular development: a 20-year perspective. Birth Defects Res Part C Embryo Today. 2003;69(1):2-13. 66. Ziolkowska L, Kawalec W, Turska-Kmiec A, et al. Chromo-some 22q11.2 microdeletion in children with conotruncal heart defects: frequency, associated cardiovascular anoma-lies, and outcome following cardiac surgery. Eur J Pediatr. 2008;167(10):1135-1140. 67. Anderson KR, McGoon DC, Lie JT. Surgical significance of the coronary arterial anatomy in | Surgery_Schwartz. trunk (truncus arteriosus communis) and its embryologic implications: a study of 57 necroscopy cases. Am J Cardiol. 1965;16(3):406-425. 63. De la Cruz MV, Pio da Rocha J. An ontogenic theory for the explanation of congenital malformations involving the truncus and conus. Am Heart J. 1976;51(5):782-805. 64. Manner J. Cardiac looping in the chick embryo: a morpho-logic review with special reference to terminological and biomechanical aspects of the looping process. Anat Rec. 2000;259(3):242-262. 65. Hutson MR, Kirby ML. Neural crest and cardiovascular development: a 20-year perspective. Birth Defects Res Part C Embryo Today. 2003;69(1):2-13. 66. Ziolkowska L, Kawalec W, Turska-Kmiec A, et al. Chromo-some 22q11.2 microdeletion in children with conotruncal heart defects: frequency, associated cardiovascular anoma-lies, and outcome following cardiac surgery. Eur J Pediatr. 2008;167(10):1135-1140. 67. Anderson KR, McGoon DC, Lie JT. Surgical significance of the coronary arterial anatomy in |
Surgery_Schwartz_5298 | Surgery_Schwartz | cardiovascular anoma-lies, and outcome following cardiac surgery. Eur J Pediatr. 2008;167(10):1135-1140. 67. Anderson KR, McGoon DC, Lie JT. Surgical significance of the coronary arterial anatomy in truncus arteriosus communis. Am J Cardiol. 1978;41(1):76-81. 68. Chiu IS, Wu SJ, Chen MR, Chen SJ, Wang JK. Anatomic rela-tionship of the coronary orifice and truncal valve in truncus arteriosus and their surgical implication. J Thorac Cardiovasc Surg. 2002;123(2):350-352. 69. Armer RM, De Oliveira PF, Lurie PR. True truncus arteriosus. Review of 17 cases and report of surgery in 7 patients. Circu-lation. 1961;24:878-890. 70. McGoon DC, Rastelli GC, Ongley PA. An operation for the correction of truncus arteriosus. JAMA. 1968;205(2): 69-73. 71. Ebert PA. Truncus arteriosus. In: Glenn WWL, Baue AE, Geha AS, eds. Thoracic and Cardiovascular Surgery. 4th ed. Norwalk: Appleton-Century-Crofts; 1983:731.Brunicardi_Ch20_p0751-p0800.indd 79622/02/19 2:57 PM 797CONGENITAL HEART DISEASECHAPTER | Surgery_Schwartz. cardiovascular anoma-lies, and outcome following cardiac surgery. Eur J Pediatr. 2008;167(10):1135-1140. 67. Anderson KR, McGoon DC, Lie JT. Surgical significance of the coronary arterial anatomy in truncus arteriosus communis. Am J Cardiol. 1978;41(1):76-81. 68. Chiu IS, Wu SJ, Chen MR, Chen SJ, Wang JK. Anatomic rela-tionship of the coronary orifice and truncal valve in truncus arteriosus and their surgical implication. J Thorac Cardiovasc Surg. 2002;123(2):350-352. 69. Armer RM, De Oliveira PF, Lurie PR. True truncus arteriosus. Review of 17 cases and report of surgery in 7 patients. Circu-lation. 1961;24:878-890. 70. McGoon DC, Rastelli GC, Ongley PA. An operation for the correction of truncus arteriosus. JAMA. 1968;205(2): 69-73. 71. Ebert PA. Truncus arteriosus. In: Glenn WWL, Baue AE, Geha AS, eds. Thoracic and Cardiovascular Surgery. 4th ed. Norwalk: Appleton-Century-Crofts; 1983:731.Brunicardi_Ch20_p0751-p0800.indd 79622/02/19 2:57 PM 797CONGENITAL HEART DISEASECHAPTER |
Surgery_Schwartz_5299 | Surgery_Schwartz | AE, Geha AS, eds. Thoracic and Cardiovascular Surgery. 4th ed. Norwalk: Appleton-Century-Crofts; 1983:731.Brunicardi_Ch20_p0751-p0800.indd 79622/02/19 2:57 PM 797CONGENITAL HEART DISEASECHAPTER 20 72. Forbess JM, Shah AS, St Louis JD, Jaggers JJ, Ungerleider RM. Cryopreserved homografts in the pulmonary position: determinants of durability. Ann Thorac Surg. 2001;71:54-59. 73. Aupecle B, Serraf A, Belli E, et al. Intermediate follow-up of a composite stentless porcine valved conduit of bovine pericardium in the pulmonary circulation. Ann Thorac Surg. 2002;74(1):127-132. 74. Correa-Villaseñor A, Ferencz C, Boughman JA, Neill CA. Total anomalous pulmonary venous return: familial and envi-ronmental factors. The Baltimore-Washington Infant Study Group. Teratology. 1991;44(4):415-428. 75. Darling RC, Rothney WB, Craij JM. Total pulmonary venous drainage into the right side of the heart. Lab Invest. 1957;6(1):44-64. 76. Delisle G, Ando M, Calder AL, et al. Total anomalous pul-monary | Surgery_Schwartz. AE, Geha AS, eds. Thoracic and Cardiovascular Surgery. 4th ed. Norwalk: Appleton-Century-Crofts; 1983:731.Brunicardi_Ch20_p0751-p0800.indd 79622/02/19 2:57 PM 797CONGENITAL HEART DISEASECHAPTER 20 72. Forbess JM, Shah AS, St Louis JD, Jaggers JJ, Ungerleider RM. Cryopreserved homografts in the pulmonary position: determinants of durability. Ann Thorac Surg. 2001;71:54-59. 73. Aupecle B, Serraf A, Belli E, et al. Intermediate follow-up of a composite stentless porcine valved conduit of bovine pericardium in the pulmonary circulation. Ann Thorac Surg. 2002;74(1):127-132. 74. Correa-Villaseñor A, Ferencz C, Boughman JA, Neill CA. Total anomalous pulmonary venous return: familial and envi-ronmental factors. The Baltimore-Washington Infant Study Group. Teratology. 1991;44(4):415-428. 75. Darling RC, Rothney WB, Craij JM. Total pulmonary venous drainage into the right side of the heart. Lab Invest. 1957;6(1):44-64. 76. Delisle G, Ando M, Calder AL, et al. Total anomalous pul-monary |
Surgery_Schwartz_5300 | Surgery_Schwartz | RC, Rothney WB, Craij JM. Total pulmonary venous drainage into the right side of the heart. Lab Invest. 1957;6(1):44-64. 76. Delisle G, Ando M, Calder AL, et al. Total anomalous pul-monary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J. 1976;91(1):99-122. 77. Michielon G, Di Donato RM, Pasquini L, et al. Total anoma-lous pulmonary venous connection: long-term appraisal with evolving technical solutions. Eur J Cardiothorac Surg. 2002;22(2):184-191. 78. Jonas RA, Smolinsky A, Mayer JE, Castaneda AR. Obstructed pulmonary venous drainage with total anomalous pulmo-nary venous connection to the coronary sinus. Am J Cardiol. 1987;59(5):431-435. 79. Austin EH. Disorders of pulmonary venous return. In: Sabis-ton DC, Lyerly HK, eds. Textbook of Surgery: The Biologi-cal Basis of Modern Surgical Practice. 15th ed. Philadelphia: W.B. Saunders; 1997:2001. 80. Lacour-Gayet F, Rey C, Planche C. Pulmonary vein steno-sis. Description | Surgery_Schwartz. RC, Rothney WB, Craij JM. Total pulmonary venous drainage into the right side of the heart. Lab Invest. 1957;6(1):44-64. 76. Delisle G, Ando M, Calder AL, et al. Total anomalous pul-monary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am Heart J. 1976;91(1):99-122. 77. Michielon G, Di Donato RM, Pasquini L, et al. Total anoma-lous pulmonary venous connection: long-term appraisal with evolving technical solutions. Eur J Cardiothorac Surg. 2002;22(2):184-191. 78. Jonas RA, Smolinsky A, Mayer JE, Castaneda AR. Obstructed pulmonary venous drainage with total anomalous pulmo-nary venous connection to the coronary sinus. Am J Cardiol. 1987;59(5):431-435. 79. Austin EH. Disorders of pulmonary venous return. In: Sabis-ton DC, Lyerly HK, eds. Textbook of Surgery: The Biologi-cal Basis of Modern Surgical Practice. 15th ed. Philadelphia: W.B. Saunders; 1997:2001. 80. Lacour-Gayet F, Rey C, Planche C. Pulmonary vein steno-sis. Description |
Surgery_Schwartz_5301 | Surgery_Schwartz | Textbook of Surgery: The Biologi-cal Basis of Modern Surgical Practice. 15th ed. Philadelphia: W.B. Saunders; 1997:2001. 80. Lacour-Gayet F, Rey C, Planche C. Pulmonary vein steno-sis. Description of a sutureless surgical procedure using the pericardium in situ (in French). Arch Mal Coeur Vaiss. 1996;89(5):633-636. 81. Najm HK, Caldarone CA, Smallhorn J, Coles JG. A suture-less technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg. 1998;115(2):468-470. 82. Hyde JAJ, Stumper O, Barth MJ, et al. Total anomalous pul-monary venous connection: outcome of surgical correction and management of recurrent venous obstruction. Eur J Car-diothorac Surg. 1999;15(6):735-740. 83. Korbmacher B, Buttgen S, Schulte HD, et al. Long-term results after repair of total anomalous pulmonary venous con-nection. Thorac Cardiovasc Surg. 2001;49(2):101-106. 84. Bando K, Turrentine MW, Ensing GJ, et al. Surgical man-agement of total anomalous pulmonary | Surgery_Schwartz. Textbook of Surgery: The Biologi-cal Basis of Modern Surgical Practice. 15th ed. Philadelphia: W.B. Saunders; 1997:2001. 80. Lacour-Gayet F, Rey C, Planche C. Pulmonary vein steno-sis. Description of a sutureless surgical procedure using the pericardium in situ (in French). Arch Mal Coeur Vaiss. 1996;89(5):633-636. 81. Najm HK, Caldarone CA, Smallhorn J, Coles JG. A suture-less technique for the relief of pulmonary vein stenosis with the use of in situ pericardium. J Thorac Cardiovasc Surg. 1998;115(2):468-470. 82. Hyde JAJ, Stumper O, Barth MJ, et al. Total anomalous pul-monary venous connection: outcome of surgical correction and management of recurrent venous obstruction. Eur J Car-diothorac Surg. 1999;15(6):735-740. 83. Korbmacher B, Buttgen S, Schulte HD, et al. Long-term results after repair of total anomalous pulmonary venous con-nection. Thorac Cardiovasc Surg. 2001;49(2):101-106. 84. Bando K, Turrentine MW, Ensing GJ, et al. Surgical man-agement of total anomalous pulmonary |
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