id
stringlengths 14
28
| title
stringclasses 18
values | content
stringlengths 2
999
| contents
stringlengths 19
1.02k
|
|---|---|---|---|
Surgery_Schwartz_5102 | Surgery_Schwartz | associated anomalies, such as ventricular septal defect, PDA, and ASD, may be seen with COA, but the most common is that of a bicuspid aortic valve, which can be demon-strated in 25% to 42% of cases.48Pathophysiology. Infants with COA develop symptoms con-sistent with left ventricular outflow obstruction, including pulmo-nary overcirculation and, later, biventricular failure. In addition, proximal systemic hypertension develops as a result of mechanical obstruction to ventricular ejection, as well as hypoperfusion-induced activation of the renin-angiotensin-aldosterone system. PAAoBrunicardi_Ch20_p0751-p0800.indd 76122/02/19 2:54 PM 762SPECIFIC CONSIDERATIONSPART IIFigure 20-9. Reformatted images obtained from CT angiography of a baby showing a descrete coarctation of the aorta (‘*’ points to the coarctation).ABFigure 20-10. A. Reformatted images obtained from a CT angio-gram of a child with discrete coarctation of the aorta (‘*’ points to the coarctation). B. Aortogram performed | Surgery_Schwartz. associated anomalies, such as ventricular septal defect, PDA, and ASD, may be seen with COA, but the most common is that of a bicuspid aortic valve, which can be demon-strated in 25% to 42% of cases.48Pathophysiology. Infants with COA develop symptoms con-sistent with left ventricular outflow obstruction, including pulmo-nary overcirculation and, later, biventricular failure. In addition, proximal systemic hypertension develops as a result of mechanical obstruction to ventricular ejection, as well as hypoperfusion-induced activation of the renin-angiotensin-aldosterone system. PAAoBrunicardi_Ch20_p0751-p0800.indd 76122/02/19 2:54 PM 762SPECIFIC CONSIDERATIONSPART IIFigure 20-9. Reformatted images obtained from CT angiography of a baby showing a descrete coarctation of the aorta (‘*’ points to the coarctation).ABFigure 20-10. A. Reformatted images obtained from a CT angio-gram of a child with discrete coarctation of the aorta (‘*’ points to the coarctation). B. Aortogram performed |
Surgery_Schwartz_5103 | Surgery_Schwartz | to the coarctation).ABFigure 20-10. A. Reformatted images obtained from a CT angio-gram of a child with discrete coarctation of the aorta (‘*’ points to the coarctation). B. Aortogram performed in the cardiac catheteriza-tion lab after stenting the coarctation (‘*’ points to the stent).Interestingly, hypertension is often persistent after surgical correction despite complete amelioration of the mechanical obstruction and pressure gradient.49 It has been shown that early surgical correction may prevent the development of long-term hypertension, which undoubtedly contributes to many of the adverse sequelae of COA, including the development of circle of Willis aneurysms, aortic dissection and rupture, and an increased incidence of coronary arteriopathy with resulting myocardial infarction.50Diagnosis. COA is likely to become symptomatic either in the newborn period if other anomalies are present or in the late ado-lescent period with the onset of left ventricular failure.Physical | Surgery_Schwartz. to the coarctation).ABFigure 20-10. A. Reformatted images obtained from a CT angio-gram of a child with discrete coarctation of the aorta (‘*’ points to the coarctation). B. Aortogram performed in the cardiac catheteriza-tion lab after stenting the coarctation (‘*’ points to the stent).Interestingly, hypertension is often persistent after surgical correction despite complete amelioration of the mechanical obstruction and pressure gradient.49 It has been shown that early surgical correction may prevent the development of long-term hypertension, which undoubtedly contributes to many of the adverse sequelae of COA, including the development of circle of Willis aneurysms, aortic dissection and rupture, and an increased incidence of coronary arteriopathy with resulting myocardial infarction.50Diagnosis. COA is likely to become symptomatic either in the newborn period if other anomalies are present or in the late ado-lescent period with the onset of left ventricular failure.Physical |
Surgery_Schwartz_5104 | Surgery_Schwartz | is likely to become symptomatic either in the newborn period if other anomalies are present or in the late ado-lescent period with the onset of left ventricular failure.Physical examination will demonstrate a hyperdynamic precordium with a harsh murmur localized to the left chest and back. Femoral pulses will be dramatically decreased when com-pared to upper extremity pulses, and differential cyanosis may be apparent until ductal closure.Echocardiography will reliably demonstrate the narrowed aortic segment, as well as define the pressure gradient across the stenotic segment. In addition, detailed information regarding other associated anomalies can be gleaned. Aortography (Fig. 20-9) is reserved for those cases in which the echocardiographic findings are equivocal. Cross-sectional imaging with computed tomogra-phy (CT) scan or MRI is also increasing to facilitate definition of arch anatomy (i.e., transverse arch hypoplasia), assess intracardiac volumes, and associated | Surgery_Schwartz. is likely to become symptomatic either in the newborn period if other anomalies are present or in the late ado-lescent period with the onset of left ventricular failure.Physical examination will demonstrate a hyperdynamic precordium with a harsh murmur localized to the left chest and back. Femoral pulses will be dramatically decreased when com-pared to upper extremity pulses, and differential cyanosis may be apparent until ductal closure.Echocardiography will reliably demonstrate the narrowed aortic segment, as well as define the pressure gradient across the stenotic segment. In addition, detailed information regarding other associated anomalies can be gleaned. Aortography (Fig. 20-9) is reserved for those cases in which the echocardiographic findings are equivocal. Cross-sectional imaging with computed tomogra-phy (CT) scan or MRI is also increasing to facilitate definition of arch anatomy (i.e., transverse arch hypoplasia), assess intracardiac volumes, and associated |
Surgery_Schwartz_5105 | Surgery_Schwartz | imaging with computed tomogra-phy (CT) scan or MRI is also increasing to facilitate definition of arch anatomy (i.e., transverse arch hypoplasia), assess intracardiac volumes, and associated defects.Therapy. The routine management of hemodynamically sig-nificant COA in all age groups has traditionally been surgical. Transcatheter repairs (Fig. 20-10) are used with increasing frequency in older patients and those with recoarctation fol-lowing surgical repair. Balloon dilatation of native coarctation in neonates generally is avoided because of the high recoarc-tation rate. However, in infants who present with severely depressed LV function and a closed ductus arteriosus, initial decompression with balloon dilation of the COA followed by later surgical intervention may be useful. The most common surgical techniques in current use are resection with end-to-end anastomosis or extended end-to-end anastomosis, taking care to remove all residual ductal tissue.51,52 Extended end-to-end | Surgery_Schwartz. imaging with computed tomogra-phy (CT) scan or MRI is also increasing to facilitate definition of arch anatomy (i.e., transverse arch hypoplasia), assess intracardiac volumes, and associated defects.Therapy. The routine management of hemodynamically sig-nificant COA in all age groups has traditionally been surgical. Transcatheter repairs (Fig. 20-10) are used with increasing frequency in older patients and those with recoarctation fol-lowing surgical repair. Balloon dilatation of native coarctation in neonates generally is avoided because of the high recoarc-tation rate. However, in infants who present with severely depressed LV function and a closed ductus arteriosus, initial decompression with balloon dilation of the COA followed by later surgical intervention may be useful. The most common surgical techniques in current use are resection with end-to-end anastomosis or extended end-to-end anastomosis, taking care to remove all residual ductal tissue.51,52 Extended end-to-end |
Surgery_Schwartz_5106 | Surgery_Schwartz | most common surgical techniques in current use are resection with end-to-end anastomosis or extended end-to-end anastomosis, taking care to remove all residual ductal tissue.51,52 Extended end-to-end anastomosis (Fig. 20-11) may also allow the surgeon to treat transverse arch hypoplasia, which is commonly encoun-tered in infants with aortic coarctation.53,54 The subclavian flap Brunicardi_Ch20_p0751-p0800.indd 76222/02/19 2:55 PM 763CONGENITAL HEART DISEASECHAPTER 20aortoplasty is another repair, although it is used less frequently in the modern era because of the risk of late aneurysm formation and possible underdevelopment of the left upper extremity isch-emia.52 In this method, the left subclavian artery is transected and brought down over the coarcted segment as a vascular-ized patch. The main benefit of these techniques is that they do not involve the use of prosthetic materials, and evidence sug-gests that extended end-to-end anastomosis may promote arch growth, especially | Surgery_Schwartz. most common surgical techniques in current use are resection with end-to-end anastomosis or extended end-to-end anastomosis, taking care to remove all residual ductal tissue.51,52 Extended end-to-end anastomosis (Fig. 20-11) may also allow the surgeon to treat transverse arch hypoplasia, which is commonly encoun-tered in infants with aortic coarctation.53,54 The subclavian flap Brunicardi_Ch20_p0751-p0800.indd 76222/02/19 2:55 PM 763CONGENITAL HEART DISEASECHAPTER 20aortoplasty is another repair, although it is used less frequently in the modern era because of the risk of late aneurysm formation and possible underdevelopment of the left upper extremity isch-emia.52 In this method, the left subclavian artery is transected and brought down over the coarcted segment as a vascular-ized patch. The main benefit of these techniques is that they do not involve the use of prosthetic materials, and evidence sug-gests that extended end-to-end anastomosis may promote arch growth, especially |
Surgery_Schwartz_5107 | Surgery_Schwartz | The main benefit of these techniques is that they do not involve the use of prosthetic materials, and evidence sug-gests that extended end-to-end anastomosis may promote arch growth, especially in infants with the smallest initial aortic arch diameters.53Despite the benefits, however, extended end-to-end anas-tomosis may not be feasible when there is a long segment of coarctation or in the presence of previous surgery because suf-ficient mobilization of the aorta above and below the lesion may not be possible. In this instance, prosthetic materials, such as a patch aortoplasty, in which a prosthetic patch is used to enlarge the coarcted segment, or an interposition tube graft must be employed. One of the most important decisions in infants and neonates with COA and some degree of transverse arch hypoplasia is whether the lesion should be approached with a sternotomy or a thoracotomy. Cross-sectional imaging with CT scan can be extremely helpful in assessing the adequacy of the | Surgery_Schwartz. The main benefit of these techniques is that they do not involve the use of prosthetic materials, and evidence sug-gests that extended end-to-end anastomosis may promote arch growth, especially in infants with the smallest initial aortic arch diameters.53Despite the benefits, however, extended end-to-end anas-tomosis may not be feasible when there is a long segment of coarctation or in the presence of previous surgery because suf-ficient mobilization of the aorta above and below the lesion may not be possible. In this instance, prosthetic materials, such as a patch aortoplasty, in which a prosthetic patch is used to enlarge the coarcted segment, or an interposition tube graft must be employed. One of the most important decisions in infants and neonates with COA and some degree of transverse arch hypoplasia is whether the lesion should be approached with a sternotomy or a thoracotomy. Cross-sectional imaging with CT scan can be extremely helpful in assessing the adequacy of the |
Surgery_Schwartz_5108 | Surgery_Schwartz | arch hypoplasia is whether the lesion should be approached with a sternotomy or a thoracotomy. Cross-sectional imaging with CT scan can be extremely helpful in assessing the adequacy of the transverse arch and any associated abnormalities with branching that may complicate repair from the side.The most common complications after COA repair are late restenosis (Fig. 20-12) and aneurysm formation at the repair site.55-57 Aneurysm formation is particularly common after patch aortoplasty when using Dacron material. In a large series of 891 patients, aneurysms occurred in 5.4% of the total, with 89% occurring in the group who received Dacron-patch aortoplasty and only 8% occurring in those who received resection with primary end-to-end anastomosis.55 A further complication, although uncommon, is lower-body paralysis resulting from ischemic spinal cord injury during the repair. This dreaded outcome complicates 0.5% of all surgical repairs, but its incidence can be lessened with the use of | Surgery_Schwartz. arch hypoplasia is whether the lesion should be approached with a sternotomy or a thoracotomy. Cross-sectional imaging with CT scan can be extremely helpful in assessing the adequacy of the transverse arch and any associated abnormalities with branching that may complicate repair from the side.The most common complications after COA repair are late restenosis (Fig. 20-12) and aneurysm formation at the repair site.55-57 Aneurysm formation is particularly common after patch aortoplasty when using Dacron material. In a large series of 891 patients, aneurysms occurred in 5.4% of the total, with 89% occurring in the group who received Dacron-patch aortoplasty and only 8% occurring in those who received resection with primary end-to-end anastomosis.55 A further complication, although uncommon, is lower-body paralysis resulting from ischemic spinal cord injury during the repair. This dreaded outcome complicates 0.5% of all surgical repairs, but its incidence can be lessened with the use of |
Surgery_Schwartz_5109 | Surgery_Schwartz | is lower-body paralysis resulting from ischemic spinal cord injury during the repair. This dreaded outcome complicates 0.5% of all surgical repairs, but its incidence can be lessened with the use of some form of distal perfusion, preferably left heart bypass with the use of femoral arterial or distal thoracic aorta for arterial inflow and Figure 20-11. Appearance of the aorta after resection of the seg-ment of coarctation and reconstruction with an extended end-to-end anastomosis. (Used with permission from Kelly Rosso MD.)Figure 20-12. Reformatted images obtained from a CT angiogram after recurrent coarctation repaired by an extra anatomic bypass (‘*’ points to the bypass graft).the femoral vein or left atrium for venous return.51 These tech-niques are generally reserved for older patients with complex coarctations that may need prolonged aortic cross clamp times for repair, often in the setting of large collateral vessels and/or previous surgery.58Hypertension is also well | Surgery_Schwartz. is lower-body paralysis resulting from ischemic spinal cord injury during the repair. This dreaded outcome complicates 0.5% of all surgical repairs, but its incidence can be lessened with the use of some form of distal perfusion, preferably left heart bypass with the use of femoral arterial or distal thoracic aorta for arterial inflow and Figure 20-11. Appearance of the aorta after resection of the seg-ment of coarctation and reconstruction with an extended end-to-end anastomosis. (Used with permission from Kelly Rosso MD.)Figure 20-12. Reformatted images obtained from a CT angiogram after recurrent coarctation repaired by an extra anatomic bypass (‘*’ points to the bypass graft).the femoral vein or left atrium for venous return.51 These tech-niques are generally reserved for older patients with complex coarctations that may need prolonged aortic cross clamp times for repair, often in the setting of large collateral vessels and/or previous surgery.58Hypertension is also well |
Surgery_Schwartz_5110 | Surgery_Schwartz | patients with complex coarctations that may need prolonged aortic cross clamp times for repair, often in the setting of large collateral vessels and/or previous surgery.58Hypertension is also well recognized following repair of COA. Bouchart and colleagues reported that in a cohort of 35 hypertensive adults (mean age, 28 years) undergoing repair, despite a satisfactory anatomic outcome, only 23 patients were normotensive at a mean follow-up period of 165 months.56 Like-wise, Bhat and associates reported that in a series of 84 patients (mean age at repair, 29 years), 31% remained hypertensive at a mean follow-up of 5 years following surgery.57Although operative repair is still the gold standard, treat-ment of COA by catheter-based intervention has become more widespread for older children and adults. Both balloon dilata-tion and primary stent implantation have been used successfully. The most extensive study of the results of balloon angioplasty reported on 970 procedures: 422 native | Surgery_Schwartz. patients with complex coarctations that may need prolonged aortic cross clamp times for repair, often in the setting of large collateral vessels and/or previous surgery.58Hypertension is also well recognized following repair of COA. Bouchart and colleagues reported that in a cohort of 35 hypertensive adults (mean age, 28 years) undergoing repair, despite a satisfactory anatomic outcome, only 23 patients were normotensive at a mean follow-up period of 165 months.56 Like-wise, Bhat and associates reported that in a series of 84 patients (mean age at repair, 29 years), 31% remained hypertensive at a mean follow-up of 5 years following surgery.57Although operative repair is still the gold standard, treat-ment of COA by catheter-based intervention has become more widespread for older children and adults. Both balloon dilata-tion and primary stent implantation have been used successfully. The most extensive study of the results of balloon angioplasty reported on 970 procedures: 422 native |
Surgery_Schwartz_5111 | Surgery_Schwartz | and adults. Both balloon dilata-tion and primary stent implantation have been used successfully. The most extensive study of the results of balloon angioplasty reported on 970 procedures: 422 native and 548 recurrent COAs. Mean gradient reduction was 74% ± 24% for native and 70% ± 31% for recurrent COA.59 This demonstrated that catheter-based therapy could produce equally effective results both in recurrent and in primary COA, a finding with far-reaching implications in the new paradigm of multidisciplinary treatment algorithms for CHD. In the Valvuloplasty and Angioplasty of Congeni-tal Anomalies (VACA) report, higher preangioplasty gradient, earlier procedure date, older patient age, and the presence of recurrent COA were independent risk factors for suboptimal procedural outcome.5The gradient after balloon dilatation in most series is gen-erally acceptable. However, there is a significant minority of patients (0%–26%) for whom the procedural outcome is sub-optimal, with a | Surgery_Schwartz. and adults. Both balloon dilata-tion and primary stent implantation have been used successfully. The most extensive study of the results of balloon angioplasty reported on 970 procedures: 422 native and 548 recurrent COAs. Mean gradient reduction was 74% ± 24% for native and 70% ± 31% for recurrent COA.59 This demonstrated that catheter-based therapy could produce equally effective results both in recurrent and in primary COA, a finding with far-reaching implications in the new paradigm of multidisciplinary treatment algorithms for CHD. In the Valvuloplasty and Angioplasty of Congeni-tal Anomalies (VACA) report, higher preangioplasty gradient, earlier procedure date, older patient age, and the presence of recurrent COA were independent risk factors for suboptimal procedural outcome.5The gradient after balloon dilatation in most series is gen-erally acceptable. However, there is a significant minority of patients (0%–26%) for whom the procedural outcome is sub-optimal, with a |
Surgery_Schwartz_5112 | Surgery_Schwartz | gradient after balloon dilatation in most series is gen-erally acceptable. However, there is a significant minority of patients (0%–26%) for whom the procedural outcome is sub-optimal, with a postprocedure gradient of 20 mmHg or greater. These patients may be ideal candidates for primary stent place-ment. Deaths from the procedure also are infrequent (<1% of cases), and the main major complication is aneurysm formation, PAAoBrunicardi_Ch20_p0751-p0800.indd 76322/02/19 2:55 PM 764SPECIFIC CONSIDERATIONSPART IIwhich occurs in 7% of patients.51 With stent implantation, many authors have demonstrated improved resolution of stenosis compared with balloon dilatation alone, yet the long-term com-plications on vessel wall compliance remain largely unknown because only mid-term data are widely available.In summary, children younger than age 6 months with native COA should be treated with surgical repair, while those requiring intervention at later ages may be ideal candidates for balloon | Surgery_Schwartz. gradient after balloon dilatation in most series is gen-erally acceptable. However, there is a significant minority of patients (0%–26%) for whom the procedural outcome is sub-optimal, with a postprocedure gradient of 20 mmHg or greater. These patients may be ideal candidates for primary stent place-ment. Deaths from the procedure also are infrequent (<1% of cases), and the main major complication is aneurysm formation, PAAoBrunicardi_Ch20_p0751-p0800.indd 76322/02/19 2:55 PM 764SPECIFIC CONSIDERATIONSPART IIwhich occurs in 7% of patients.51 With stent implantation, many authors have demonstrated improved resolution of stenosis compared with balloon dilatation alone, yet the long-term com-plications on vessel wall compliance remain largely unknown because only mid-term data are widely available.In summary, children younger than age 6 months with native COA should be treated with surgical repair, while those requiring intervention at later ages may be ideal candidates for balloon |
Surgery_Schwartz_5113 | Surgery_Schwartz | available.In summary, children younger than age 6 months with native COA should be treated with surgical repair, while those requiring intervention at later ages may be ideal candidates for balloon dilatation or primary stent implantation.51 Additionally, catheter-based therapy should be employed for those cases of restenosis following either surgical or primary endovascular management.Truncus ArteriosusAnatomy. Truncus arteriosus is a rare anomaly, compris-ing between 1% and 2% of all live born cases of CHD.60 It is characterized by a single great artery that arises from the heart, overrides the ventricular septum, and supplies the pulmonary, systemic, and coronary circulations.The two major classification systems are those of Collett and Edwards, described in 1949, and Van Praagh, described in 1965 (Fig. 20-13).61,62 The Collett and Edwards classification focuses mainly on the origin of the pulmonary arteries from the common arterial trunk, whereas the Van Praagh system is based on | Surgery_Schwartz. available.In summary, children younger than age 6 months with native COA should be treated with surgical repair, while those requiring intervention at later ages may be ideal candidates for balloon dilatation or primary stent implantation.51 Additionally, catheter-based therapy should be employed for those cases of restenosis following either surgical or primary endovascular management.Truncus ArteriosusAnatomy. Truncus arteriosus is a rare anomaly, compris-ing between 1% and 2% of all live born cases of CHD.60 It is characterized by a single great artery that arises from the heart, overrides the ventricular septum, and supplies the pulmonary, systemic, and coronary circulations.The two major classification systems are those of Collett and Edwards, described in 1949, and Van Praagh, described in 1965 (Fig. 20-13).61,62 The Collett and Edwards classification focuses mainly on the origin of the pulmonary arteries from the common arterial trunk, whereas the Van Praagh system is based on |
Surgery_Schwartz_5114 | Surgery_Schwartz | in 1965 (Fig. 20-13).61,62 The Collett and Edwards classification focuses mainly on the origin of the pulmonary arteries from the common arterial trunk, whereas the Van Praagh system is based on the presence or absence of a VSD, the degree of formation of the aorticopulmonary septum, and the status of the aortic arch.During embryonic life, the truncus arteriosus normally begins to separate and spiral into a distinguishable anterior pul-monary artery and posterior aorta. Persistent truncus, therefore, represents an arrest in embryologic development at this stage.63 Other implicated events include twisting of the dividing trun-cus because of ventricular looping, subinfundibular atresia, and abnormal location of the semilunar valve anlages.64The neural crest may also play a crucial role in the normal formation of the great vessels, as experimental studies in chick embryos have shown that ablation of the neural crest results in persistent truncus arteriosus.65 The neural crest also | Surgery_Schwartz. in 1965 (Fig. 20-13).61,62 The Collett and Edwards classification focuses mainly on the origin of the pulmonary arteries from the common arterial trunk, whereas the Van Praagh system is based on the presence or absence of a VSD, the degree of formation of the aorticopulmonary septum, and the status of the aortic arch.During embryonic life, the truncus arteriosus normally begins to separate and spiral into a distinguishable anterior pul-monary artery and posterior aorta. Persistent truncus, therefore, represents an arrest in embryologic development at this stage.63 Other implicated events include twisting of the dividing trun-cus because of ventricular looping, subinfundibular atresia, and abnormal location of the semilunar valve anlages.64The neural crest may also play a crucial role in the normal formation of the great vessels, as experimental studies in chick embryos have shown that ablation of the neural crest results in persistent truncus arteriosus.65 The neural crest also |
Surgery_Schwartz_5115 | Surgery_Schwartz | in the normal formation of the great vessels, as experimental studies in chick embryos have shown that ablation of the neural crest results in persistent truncus arteriosus.65 The neural crest also develops into the pharyngeal pouches that give rise to the thymus and parathyroids, which likely explains the prevalent association of truncus arteriosus and DiGeorge’s syndrome.66The annulus of the truncal valve usually straddles the ventricular septum in a “balanced” fashion; however, it is not unusual for it to be positioned predominantly over the RV, which increases the potential for LVOT obstruction following surgical repair. In the great majority of cases, the leaflets are thickened and deformed, which leads to valvular insufficiency. There are usually three leaflets (60%), but occasionally a bicus-pid (5%) or even a quadricuspid valve (25%) is present.61In truncus arteriosus, the pulmonary trunk bifurcates, with the left and right pulmonary arteries forming posteriorly and to the | Surgery_Schwartz. in the normal formation of the great vessels, as experimental studies in chick embryos have shown that ablation of the neural crest results in persistent truncus arteriosus.65 The neural crest also develops into the pharyngeal pouches that give rise to the thymus and parathyroids, which likely explains the prevalent association of truncus arteriosus and DiGeorge’s syndrome.66The annulus of the truncal valve usually straddles the ventricular septum in a “balanced” fashion; however, it is not unusual for it to be positioned predominantly over the RV, which increases the potential for LVOT obstruction following surgical repair. In the great majority of cases, the leaflets are thickened and deformed, which leads to valvular insufficiency. There are usually three leaflets (60%), but occasionally a bicus-pid (5%) or even a quadricuspid valve (25%) is present.61In truncus arteriosus, the pulmonary trunk bifurcates, with the left and right pulmonary arteries forming posteriorly and to the |
Surgery_Schwartz_5116 | Surgery_Schwartz | a bicus-pid (5%) or even a quadricuspid valve (25%) is present.61In truncus arteriosus, the pulmonary trunk bifurcates, with the left and right pulmonary arteries forming posteriorly and to the left in most cases. The caliber of the pulmonary arterial branches is usually normal, with stenosis or diffuse hypoplasia occurring in rare instances.The coronary arteries may be normal; however, anomalies are not unusual and occur in 50% of cases.67 Many of these are relatively minor, although two variations are of particular importance because they have implications in the conduct of operative repair. The first is that the left coronary ostium may arise high in the sinus of Valsalva or even from the truncal tis-sue at the margin of the pulmonary artery tissue. This coronary artery can be injured during repair when the pulmonary arteries are removed from the trunk or when the resulting truncal defect is closed. The second is that the right coronary artery can give rise to an important | Surgery_Schwartz. a bicus-pid (5%) or even a quadricuspid valve (25%) is present.61In truncus arteriosus, the pulmonary trunk bifurcates, with the left and right pulmonary arteries forming posteriorly and to the left in most cases. The caliber of the pulmonary arterial branches is usually normal, with stenosis or diffuse hypoplasia occurring in rare instances.The coronary arteries may be normal; however, anomalies are not unusual and occur in 50% of cases.67 Many of these are relatively minor, although two variations are of particular importance because they have implications in the conduct of operative repair. The first is that the left coronary ostium may arise high in the sinus of Valsalva or even from the truncal tis-sue at the margin of the pulmonary artery tissue. This coronary artery can be injured during repair when the pulmonary arteries are removed from the trunk or when the resulting truncal defect is closed. The second is that the right coronary artery can give rise to an important |
Surgery_Schwartz_5117 | Surgery_Schwartz | injured during repair when the pulmonary arteries are removed from the trunk or when the resulting truncal defect is closed. The second is that the right coronary artery can give rise to an important accessory anterior descending artery, which often passes across the RV in the exact location where the right ventriculotomy is commonly performed during repair.68Physiology and Diagnosis. The main pathophysiologic con-sequences of truncus arteriosus are (a) the obligatory mixing of systemic and pulmonary venous blood at the level of the ven-tricular septal defect (VSD) and truncal valve, which leads to arterial saturations near 85% and (b) the presence of a nonre-strictive left-to-right shunt, which occurs during both systole and diastole, the volume of which is determined by the relative resistances of the pulmonary and systemic circulations. Addi-tionally, truncal valve stenosis or regurgitation, the presence of important LVOT obstruction, and stenosis of pulmonary artery branches can | Surgery_Schwartz. injured during repair when the pulmonary arteries are removed from the trunk or when the resulting truncal defect is closed. The second is that the right coronary artery can give rise to an important accessory anterior descending artery, which often passes across the RV in the exact location where the right ventriculotomy is commonly performed during repair.68Physiology and Diagnosis. The main pathophysiologic con-sequences of truncus arteriosus are (a) the obligatory mixing of systemic and pulmonary venous blood at the level of the ven-tricular septal defect (VSD) and truncal valve, which leads to arterial saturations near 85% and (b) the presence of a nonre-strictive left-to-right shunt, which occurs during both systole and diastole, the volume of which is determined by the relative resistances of the pulmonary and systemic circulations. Addi-tionally, truncal valve stenosis or regurgitation, the presence of important LVOT obstruction, and stenosis of pulmonary artery branches can |
Surgery_Schwartz_5118 | Surgery_Schwartz | of the pulmonary and systemic circulations. Addi-tionally, truncal valve stenosis or regurgitation, the presence of important LVOT obstruction, and stenosis of pulmonary artery branches can further contribute to both pressure and volume-loading of the ventricles. The presence of these lesions often results in severe heart failure and cardiovascular instability early in life. Pulmonary vascular resistance may develop as early as 6 months of age, leading to poor results with late surgical correction.Patients with truncus arteriosus usually present in the neo-natal period, with signs and symptoms of congestive heart fail-ure and mild to moderate cyanosis. A pansystolic murmur may be noted at the left sternal border, and occasionally a diastolic murmur may be heard in the presence of truncal regurgitation.Chest radiography will be consistent with pulmonary over-circulation, and a right aortic arch can be appreciated 35% of the time. The thymus is prominent by its absence if associated | Surgery_Schwartz. of the pulmonary and systemic circulations. Addi-tionally, truncal valve stenosis or regurgitation, the presence of important LVOT obstruction, and stenosis of pulmonary artery branches can further contribute to both pressure and volume-loading of the ventricles. The presence of these lesions often results in severe heart failure and cardiovascular instability early in life. Pulmonary vascular resistance may develop as early as 6 months of age, leading to poor results with late surgical correction.Patients with truncus arteriosus usually present in the neo-natal period, with signs and symptoms of congestive heart fail-ure and mild to moderate cyanosis. A pansystolic murmur may be noted at the left sternal border, and occasionally a diastolic murmur may be heard in the presence of truncal regurgitation.Chest radiography will be consistent with pulmonary over-circulation, and a right aortic arch can be appreciated 35% of the time. The thymus is prominent by its absence if associated |
Surgery_Schwartz_5119 | Surgery_Schwartz | regurgitation.Chest radiography will be consistent with pulmonary over-circulation, and a right aortic arch can be appreciated 35% of the time. The thymus is prominent by its absence if associated with DiGeorge syndrome (Fig. 20-14). The ECG is usually non-specific, demonstrating normal sinus rhythm with biventricular hypertrophy.Echocardiography with Doppler color-flow or pulsed Doppler is diagnostic and usually provides sufficient informa-tion to determine the type of truncus arteriosus, the origin of the Figure 20-13. Collett & Edwards classification for Truncus arteriosus. (Used with permission from Kelly Rosso MD.)RPARPARPAType 1Type 2Type 3LPALPALPABrunicardi_Ch20_p0751-p0800.indd 76422/02/19 2:55 PM 765CONGENITAL HEART DISEASECHAPTER 20coronary arteries and their proximity to the pulmonary trunk, the character of the truncal valves, and the extent of truncal insuffi-ciency (Fig. 20-15). CT scan helps define the pulmonary arteries and the coronary anatomy (Fig. 20-16). | Surgery_Schwartz. regurgitation.Chest radiography will be consistent with pulmonary over-circulation, and a right aortic arch can be appreciated 35% of the time. The thymus is prominent by its absence if associated with DiGeorge syndrome (Fig. 20-14). The ECG is usually non-specific, demonstrating normal sinus rhythm with biventricular hypertrophy.Echocardiography with Doppler color-flow or pulsed Doppler is diagnostic and usually provides sufficient informa-tion to determine the type of truncus arteriosus, the origin of the Figure 20-13. Collett & Edwards classification for Truncus arteriosus. (Used with permission from Kelly Rosso MD.)RPARPARPAType 1Type 2Type 3LPALPALPABrunicardi_Ch20_p0751-p0800.indd 76422/02/19 2:55 PM 765CONGENITAL HEART DISEASECHAPTER 20coronary arteries and their proximity to the pulmonary trunk, the character of the truncal valves, and the extent of truncal insuffi-ciency (Fig. 20-15). CT scan helps define the pulmonary arteries and the coronary anatomy (Fig. 20-16). |
Surgery_Schwartz_5120 | Surgery_Schwartz | to the pulmonary trunk, the character of the truncal valves, and the extent of truncal insuffi-ciency (Fig. 20-15). CT scan helps define the pulmonary arteries and the coronary anatomy (Fig. 20-16). Cardiac catheterization can be helpful in cases where pulmonary hypertension is sus-pected or to further delineate coronary artery anomalies prior to repair.The presence of truncus is an indication for surgery. Repair should be undertaken in the neonatal period or as soon as the diagnosis is established.Repair. Truncus arteriosus was first managed with pulmonary artery banding as described by Armer and colleagues in 1961.69 However, this technique led to only marginal improvements in 1-year survival rates because ventricular failure inevitably occurred. In 1967, however, complete repair was accomplished by McGoon and his associates based on the experimental work of Rastelli, who introduced the idea that an extracardiac valved conduit could be used to restore ventricular-to-pulmonary artery | Surgery_Schwartz. to the pulmonary trunk, the character of the truncal valves, and the extent of truncal insuffi-ciency (Fig. 20-15). CT scan helps define the pulmonary arteries and the coronary anatomy (Fig. 20-16). Cardiac catheterization can be helpful in cases where pulmonary hypertension is sus-pected or to further delineate coronary artery anomalies prior to repair.The presence of truncus is an indication for surgery. Repair should be undertaken in the neonatal period or as soon as the diagnosis is established.Repair. Truncus arteriosus was first managed with pulmonary artery banding as described by Armer and colleagues in 1961.69 However, this technique led to only marginal improvements in 1-year survival rates because ventricular failure inevitably occurred. In 1967, however, complete repair was accomplished by McGoon and his associates based on the experimental work of Rastelli, who introduced the idea that an extracardiac valved conduit could be used to restore ventricular-to-pulmonary artery |
Surgery_Schwartz_5121 | Surgery_Schwartz | by McGoon and his associates based on the experimental work of Rastelli, who introduced the idea that an extracardiac valved conduit could be used to restore ventricular-to-pulmonary artery continuity.70 Over the next 20 years, improved survival rates led to uniform adoption of complete repair even in the youngest and smallest infants.71Surgical correction entails the use of CPB. Repair is completed by separation of the pulmonary arteries from the aorta, closure of the aortic defect (occasionally with a patch) to minimize coronary flow complications, placement of a valved cryopreserved allograft or jugular venous valved conduit (Con-tegra) to reconstruct the RVOT, and VSD closure. Important branch pulmonary arterial stenosis should be repaired at the time of complete repair and can usually be accomplished with longitudinal allograft patch arterioplasty. Severe truncal valve insufficiency occasionally requires truncal valve repair or even replacement, which can be accomplished with a | Surgery_Schwartz. by McGoon and his associates based on the experimental work of Rastelli, who introduced the idea that an extracardiac valved conduit could be used to restore ventricular-to-pulmonary artery continuity.70 Over the next 20 years, improved survival rates led to uniform adoption of complete repair even in the youngest and smallest infants.71Surgical correction entails the use of CPB. Repair is completed by separation of the pulmonary arteries from the aorta, closure of the aortic defect (occasionally with a patch) to minimize coronary flow complications, placement of a valved cryopreserved allograft or jugular venous valved conduit (Con-tegra) to reconstruct the RVOT, and VSD closure. Important branch pulmonary arterial stenosis should be repaired at the time of complete repair and can usually be accomplished with longitudinal allograft patch arterioplasty. Severe truncal valve insufficiency occasionally requires truncal valve repair or even replacement, which can be accomplished with a |
Surgery_Schwartz_5122 | Surgery_Schwartz | be accomplished with longitudinal allograft patch arterioplasty. Severe truncal valve insufficiency occasionally requires truncal valve repair or even replacement, which can be accomplished with a cryopreserved allograft.72Results. The results of complete repair of truncus have steadily improved. Ebert reported a 91% survival rate in his series of 77 patients who were younger than 6 months of age; later reports by others confirmed these findings and demonstrated that excel-lent results could be achieved in even smaller infants with com-plex-associated defects.71Newer extracardiac conduits also have been developed and used with success, which has widened the repertoire of the modern congenital heart surgeon and improved outcomes.72,73 Severe truncal regurgitation, interrupted aortic arch, coexistent coronary anomalies, chromosomal or genetic anomalies, and age younger than 100 days are risk factors associated with peri-operative death and poor outcome.Total Anomalous Pulmonary Venous | Surgery_Schwartz. be accomplished with longitudinal allograft patch arterioplasty. Severe truncal valve insufficiency occasionally requires truncal valve repair or even replacement, which can be accomplished with a cryopreserved allograft.72Results. The results of complete repair of truncus have steadily improved. Ebert reported a 91% survival rate in his series of 77 patients who were younger than 6 months of age; later reports by others confirmed these findings and demonstrated that excel-lent results could be achieved in even smaller infants with com-plex-associated defects.71Newer extracardiac conduits also have been developed and used with success, which has widened the repertoire of the modern congenital heart surgeon and improved outcomes.72,73 Severe truncal regurgitation, interrupted aortic arch, coexistent coronary anomalies, chromosomal or genetic anomalies, and age younger than 100 days are risk factors associated with peri-operative death and poor outcome.Total Anomalous Pulmonary Venous |
Surgery_Schwartz_5123 | Surgery_Schwartz | coexistent coronary anomalies, chromosomal or genetic anomalies, and age younger than 100 days are risk factors associated with peri-operative death and poor outcome.Total Anomalous Pulmonary Venous ConnectionTotal anomalous pulmonary venous connection (TAPVC) occurs in 1% to 2% of all cardiac malformations and is char-acterized by abnormal drainage of the pulmonary veins into the right heart, whether through connections into the right atrium or into its tributaries.74 Accordingly, the only mechanism by which oxygenated blood can return to the left heart is through an ASD, which is almost uniformly present with TAPVC.Figure 20-14. Chest x-ray of a baby with DiGeorge syndrome and truncus arteriosus. Note the absence of the thymic shadow in the superior mediastinum (‘*’).Figure 20-15. Echo appearance of a baby with Truncus Ateriosus. The ‘*’ represents the VSD, and the arrow points to the truncal valve.Figure 20-16. CT scan of a baby with Truncus Arteriosus Type 2. The ‘*’ mark the RPA | Surgery_Schwartz. coexistent coronary anomalies, chromosomal or genetic anomalies, and age younger than 100 days are risk factors associated with peri-operative death and poor outcome.Total Anomalous Pulmonary Venous ConnectionTotal anomalous pulmonary venous connection (TAPVC) occurs in 1% to 2% of all cardiac malformations and is char-acterized by abnormal drainage of the pulmonary veins into the right heart, whether through connections into the right atrium or into its tributaries.74 Accordingly, the only mechanism by which oxygenated blood can return to the left heart is through an ASD, which is almost uniformly present with TAPVC.Figure 20-14. Chest x-ray of a baby with DiGeorge syndrome and truncus arteriosus. Note the absence of the thymic shadow in the superior mediastinum (‘*’).Figure 20-15. Echo appearance of a baby with Truncus Ateriosus. The ‘*’ represents the VSD, and the arrow points to the truncal valve.Figure 20-16. CT scan of a baby with Truncus Arteriosus Type 2. The ‘*’ mark the RPA |
Surgery_Schwartz_5124 | Surgery_Schwartz | appearance of a baby with Truncus Ateriosus. The ‘*’ represents the VSD, and the arrow points to the truncal valve.Figure 20-16. CT scan of a baby with Truncus Arteriosus Type 2. The ‘*’ mark the RPA and the LPA. Note the stenosis at the origin of the LPA.Brunicardi_Ch20_p0751-p0800.indd 76522/02/19 2:55 PM 766SPECIFIC CONSIDERATIONSPART IIUnique to this lesion is the absence of a definitive form of palliation. Thus, TAPVC with concomitant obstruction (Fig. 20-17) represents one of the only true surgical emergen-cies across the entire spectrum of congenital heart surgery.Anatomy and Embryology. The lungs develop from an out-pouching of the foregut, and their venous plexus arises as part of the splanchnic venous system. TAPVC arises when the pul-monary vein evagination from the posterior surface of the left atrium fails to fuse with the pulmonary venous plexus surround-ing the lung buds. In place of the usual connection to the left atrium, at least one connection of the pulmonary | Surgery_Schwartz. appearance of a baby with Truncus Ateriosus. The ‘*’ represents the VSD, and the arrow points to the truncal valve.Figure 20-16. CT scan of a baby with Truncus Arteriosus Type 2. The ‘*’ mark the RPA and the LPA. Note the stenosis at the origin of the LPA.Brunicardi_Ch20_p0751-p0800.indd 76522/02/19 2:55 PM 766SPECIFIC CONSIDERATIONSPART IIUnique to this lesion is the absence of a definitive form of palliation. Thus, TAPVC with concomitant obstruction (Fig. 20-17) represents one of the only true surgical emergen-cies across the entire spectrum of congenital heart surgery.Anatomy and Embryology. The lungs develop from an out-pouching of the foregut, and their venous plexus arises as part of the splanchnic venous system. TAPVC arises when the pul-monary vein evagination from the posterior surface of the left atrium fails to fuse with the pulmonary venous plexus surround-ing the lung buds. In place of the usual connection to the left atrium, at least one connection of the pulmonary |
Surgery_Schwartz_5125 | Surgery_Schwartz | surface of the left atrium fails to fuse with the pulmonary venous plexus surround-ing the lung buds. In place of the usual connection to the left atrium, at least one connection of the pulmonary plexus to the splanchnic plexus persists. Accordingly, the pulmonary veins drain to the heart through a systemic vein.Darling and colleagues classified TAPVC (Fig. 20-18) according to the site or level of connection of the pulmonary veins to the systemic venous system75: type I (45%), anomalous connection at the supracardiac level; type II (25%), anomalous connection at the cardiac level; type III (25%), anomalous con-nection at the infracardiac level; and type IV (5%), anomalous connection at multiple levels.76 Within each category, further subdivisions can be implemented, depending on whether pul-monary venous obstruction exists. Obstruction to pulmonary venous drainage is a powerful predictor of adverse natural out-come and occurs most frequently with the infracardiac type, especially when | Surgery_Schwartz. surface of the left atrium fails to fuse with the pulmonary venous plexus surround-ing the lung buds. In place of the usual connection to the left atrium, at least one connection of the pulmonary plexus to the splanchnic plexus persists. Accordingly, the pulmonary veins drain to the heart through a systemic vein.Darling and colleagues classified TAPVC (Fig. 20-18) according to the site or level of connection of the pulmonary veins to the systemic venous system75: type I (45%), anomalous connection at the supracardiac level; type II (25%), anomalous connection at the cardiac level; type III (25%), anomalous con-nection at the infracardiac level; and type IV (5%), anomalous connection at multiple levels.76 Within each category, further subdivisions can be implemented, depending on whether pul-monary venous obstruction exists. Obstruction to pulmonary venous drainage is a powerful predictor of adverse natural out-come and occurs most frequently with the infracardiac type, especially when |
Surgery_Schwartz_5126 | Surgery_Schwartz | venous obstruction exists. Obstruction to pulmonary venous drainage is a powerful predictor of adverse natural out-come and occurs most frequently with the infracardiac type, especially when the pattern of infracardiac connection prevents the ductus venosus from bypassing the liver.77Pathophysiology and Diagnosis. Because both pulmonary and systemic venous blood returns to the right atrium in all forms of TAPVC, a right-to-left intracardiac shunt must be present in order for the afflicted infant to survive. This invariably occurs via a nonrestrictive patent foramen ovale. Because of this obliga-tory mixing, cyanosis is usually present, and its degree depends on the ratio of pulmonary to systemic blood flow. Decreased Figure 20-17. Infracardiac type of TAPVR. Note the stenosis (‘*’) of the descending vertical vein as it drains into the portal system.Figure 20-18. The various types of TAPVC as described by Darling and colleagues. (Used with permission from Nicholas Clarke MD.)pulmonary | Surgery_Schwartz. venous obstruction exists. Obstruction to pulmonary venous drainage is a powerful predictor of adverse natural out-come and occurs most frequently with the infracardiac type, especially when the pattern of infracardiac connection prevents the ductus venosus from bypassing the liver.77Pathophysiology and Diagnosis. Because both pulmonary and systemic venous blood returns to the right atrium in all forms of TAPVC, a right-to-left intracardiac shunt must be present in order for the afflicted infant to survive. This invariably occurs via a nonrestrictive patent foramen ovale. Because of this obliga-tory mixing, cyanosis is usually present, and its degree depends on the ratio of pulmonary to systemic blood flow. Decreased Figure 20-17. Infracardiac type of TAPVR. Note the stenosis (‘*’) of the descending vertical vein as it drains into the portal system.Figure 20-18. The various types of TAPVC as described by Darling and colleagues. (Used with permission from Nicholas Clarke MD.)pulmonary |
Surgery_Schwartz_5127 | Surgery_Schwartz | descending vertical vein as it drains into the portal system.Figure 20-18. The various types of TAPVC as described by Darling and colleagues. (Used with permission from Nicholas Clarke MD.)pulmonary blood flow is a consequence of pulmonary venous obstruction, the presence of which is unlikely if the right ven-tricular pressure is less than 85% of systemic pressure.78The child with TAPVC may present with severe cyanosis and respiratory distress, necessitating urgent surgical interven-tion if a severe degree of pulmonary venous obstruction is pres-ent. However, in cases where there is no obstructive component, the clinical picture is usually one of pulmonary overcircula-tion, hepatomegaly, tachycardia, and tachypnea with feeding. In a child with serious obstruction, arterial blood gas analysis reveals severe hypoxemia (partial pressure of oxygen [Po2] < 20 mmHg), with metabolic acidosis.79Chest radiography (Fig. 20-19) will show normal heart size with generalized pulmonary edema. | Surgery_Schwartz. descending vertical vein as it drains into the portal system.Figure 20-18. The various types of TAPVC as described by Darling and colleagues. (Used with permission from Nicholas Clarke MD.)pulmonary blood flow is a consequence of pulmonary venous obstruction, the presence of which is unlikely if the right ven-tricular pressure is less than 85% of systemic pressure.78The child with TAPVC may present with severe cyanosis and respiratory distress, necessitating urgent surgical interven-tion if a severe degree of pulmonary venous obstruction is pres-ent. However, in cases where there is no obstructive component, the clinical picture is usually one of pulmonary overcircula-tion, hepatomegaly, tachycardia, and tachypnea with feeding. In a child with serious obstruction, arterial blood gas analysis reveals severe hypoxemia (partial pressure of oxygen [Po2] < 20 mmHg), with metabolic acidosis.79Chest radiography (Fig. 20-19) will show normal heart size with generalized pulmonary edema. |
Surgery_Schwartz_5128 | Surgery_Schwartz | analysis reveals severe hypoxemia (partial pressure of oxygen [Po2] < 20 mmHg), with metabolic acidosis.79Chest radiography (Fig. 20-19) will show normal heart size with generalized pulmonary edema. Two-dimensional echocardiography is very useful in establishing the diagnosis and also can assess ventricular septal position, which may be leftward secondary to small left ventricular volumes, as well as estimate the right ventricular pressure based on the height of the tricuspid regurgitant jet. Echocardiography can usually identify the pulmonary venous connections (types I to IV), and it is rarely necessary to perform other diagnostic tests.Cardiac catheterization is not recommended in these patients because the osmotic load from the intravenous contrast can exacerbate the degree of pulmonary edema.80 When cardiac catheterization is performed, equalization of oxygen saturations in all four heart chambers is a hallmark finding in this disease because the mixed blood returned to the right | Surgery_Schwartz. analysis reveals severe hypoxemia (partial pressure of oxygen [Po2] < 20 mmHg), with metabolic acidosis.79Chest radiography (Fig. 20-19) will show normal heart size with generalized pulmonary edema. Two-dimensional echocardiography is very useful in establishing the diagnosis and also can assess ventricular septal position, which may be leftward secondary to small left ventricular volumes, as well as estimate the right ventricular pressure based on the height of the tricuspid regurgitant jet. Echocardiography can usually identify the pulmonary venous connections (types I to IV), and it is rarely necessary to perform other diagnostic tests.Cardiac catheterization is not recommended in these patients because the osmotic load from the intravenous contrast can exacerbate the degree of pulmonary edema.80 When cardiac catheterization is performed, equalization of oxygen saturations in all four heart chambers is a hallmark finding in this disease because the mixed blood returned to the right |
Surgery_Schwartz_5129 | Surgery_Schwartz | edema.80 When cardiac catheterization is performed, equalization of oxygen saturations in all four heart chambers is a hallmark finding in this disease because the mixed blood returned to the right atrium gets dis-tributed throughout the heart.Therapy. Operative correction of TAPVC requires anastomo-sis of the common pulmonary venous channel to the left atrium, obliteration of the anomalous venous connection, and closure of the ASD.79,81IIIAIVCIVCSVCLPVLARARPVIVCRVLVRARVDVVVPVLVLASVCCPVIVCIIBIIISVCVVLPVCPVSVCRPRALARVLVCPVLALVRARVBrunicardi_Ch20_p0751-p0800.indd 76622/02/19 2:55 PM 767CONGENITAL HEART DISEASECHAPTER 20All types of TAPVC are approached through a median ster-notomy, and many surgeons use deep hypothermic circulatory arrest in order to achieve an accurate and widely patent anastomo-sis. The technique for supracardiac TAPVC includes early division of the vertical vein, retraction of the aorta and the superior vena cava laterally to expose the posterior aspect of the | Surgery_Schwartz. edema.80 When cardiac catheterization is performed, equalization of oxygen saturations in all four heart chambers is a hallmark finding in this disease because the mixed blood returned to the right atrium gets dis-tributed throughout the heart.Therapy. Operative correction of TAPVC requires anastomo-sis of the common pulmonary venous channel to the left atrium, obliteration of the anomalous venous connection, and closure of the ASD.79,81IIIAIVCIVCSVCLPVLARARPVIVCRVLVRARVDVVVPVLVLASVCCPVIVCIIBIIISVCVVLPVCPVSVCRPRALARVLVCPVLALVRARVBrunicardi_Ch20_p0751-p0800.indd 76622/02/19 2:55 PM 767CONGENITAL HEART DISEASECHAPTER 20All types of TAPVC are approached through a median ster-notomy, and many surgeons use deep hypothermic circulatory arrest in order to achieve an accurate and widely patent anastomo-sis. The technique for supracardiac TAPVC includes early division of the vertical vein, retraction of the aorta and the superior vena cava laterally to expose the posterior aspect of the |
Surgery_Schwartz_5130 | Surgery_Schwartz | anastomo-sis. The technique for supracardiac TAPVC includes early division of the vertical vein, retraction of the aorta and the superior vena cava laterally to expose the posterior aspect of the left atrium and the pulmonary venous confluence, and a side-to-side anastomosis between a long, horizontal biatrial incision and a longitudinal inci-sion within the pulmonary venous confluence. The ASD can then be closed with an autologous pericardial or synthetic patch.In patients with TAPVC to the coronary sinus without obstruction, a simple unroofing of the coronary sinus can be performed through a single right atriotomy with concomitant closure of the ASD. If pulmonary venous obstruction is pres-ent, the repair should include generous resection of roof of the coronary sinus.79Repair of infracardiac TAPVC entails ligation of the verti-cal vein at the diaphragm, followed by construction of a proximal, patulous longitudinal venotomy. This repair is usually performed by “rolling” the heart | Surgery_Schwartz. anastomo-sis. The technique for supracardiac TAPVC includes early division of the vertical vein, retraction of the aorta and the superior vena cava laterally to expose the posterior aspect of the left atrium and the pulmonary venous confluence, and a side-to-side anastomosis between a long, horizontal biatrial incision and a longitudinal inci-sion within the pulmonary venous confluence. The ASD can then be closed with an autologous pericardial or synthetic patch.In patients with TAPVC to the coronary sinus without obstruction, a simple unroofing of the coronary sinus can be performed through a single right atriotomy with concomitant closure of the ASD. If pulmonary venous obstruction is pres-ent, the repair should include generous resection of roof of the coronary sinus.79Repair of infracardiac TAPVC entails ligation of the verti-cal vein at the diaphragm, followed by construction of a proximal, patulous longitudinal venotomy. This repair is usually performed by “rolling” the heart |
Surgery_Schwartz_5131 | Surgery_Schwartz | TAPVC entails ligation of the verti-cal vein at the diaphragm, followed by construction of a proximal, patulous longitudinal venotomy. This repair is usually performed by “rolling” the heart toward the left, thus exposing the left atrium where it usually overlies the descending vertical vein.As originally described by Lacour-Gayet and colleagues at the Marie-Lannelongue Hospital, Paris, and Coles and col-leagues at The Hospital for Sick Children, Toronto, the suture-less technique was developed for patients with anastomotic stenosis occurring after TAPVC repair.80,81 After determining that favorable outcomes were possible using this technique, it is currently used in selected patients upon initial presentation of TAPVC.81 Incisions are made in the venous confluence. Based on the surgeon’s discretion, the incisions are extended into both upper and lower pulmonary veins separately if judged to be important for an unobstructed pathway. An atriopericardial anastomosis is created using the | Surgery_Schwartz. TAPVC entails ligation of the verti-cal vein at the diaphragm, followed by construction of a proximal, patulous longitudinal venotomy. This repair is usually performed by “rolling” the heart toward the left, thus exposing the left atrium where it usually overlies the descending vertical vein.As originally described by Lacour-Gayet and colleagues at the Marie-Lannelongue Hospital, Paris, and Coles and col-leagues at The Hospital for Sick Children, Toronto, the suture-less technique was developed for patients with anastomotic stenosis occurring after TAPVC repair.80,81 After determining that favorable outcomes were possible using this technique, it is currently used in selected patients upon initial presentation of TAPVC.81 Incisions are made in the venous confluence. Based on the surgeon’s discretion, the incisions are extended into both upper and lower pulmonary veins separately if judged to be important for an unobstructed pathway. An atriopericardial anastomosis is created using the |
Surgery_Schwartz_5132 | Surgery_Schwartz | discretion, the incisions are extended into both upper and lower pulmonary veins separately if judged to be important for an unobstructed pathway. An atriopericardial anastomosis is created using the pericardium adjacent to where the pulmonary veins enter the pericardium (Fig. 20-20). This anastomosis avoids direct contact with the incision site in the wall of the pulmonary veins and allows the free egress of blood from the lungs to the left atrium.The perioperative care of these infants is crucial because episodes of pulmonary hypertension can occur within the first 48 hours, which contribute significantly to mortality following repair. Muscle relaxants and narcotics should be administered during this period to maintain a constant state of anesthesia. Arterial partial pressure of carbon dioxide (Pco2) should be maintained at 30 mmHg with use of a volume ventilator, and the fraction of inspired oxygen (Fio2) should be increased to keep the pulmonary arterial pressure at less than | Surgery_Schwartz. discretion, the incisions are extended into both upper and lower pulmonary veins separately if judged to be important for an unobstructed pathway. An atriopericardial anastomosis is created using the pericardium adjacent to where the pulmonary veins enter the pericardium (Fig. 20-20). This anastomosis avoids direct contact with the incision site in the wall of the pulmonary veins and allows the free egress of blood from the lungs to the left atrium.The perioperative care of these infants is crucial because episodes of pulmonary hypertension can occur within the first 48 hours, which contribute significantly to mortality following repair. Muscle relaxants and narcotics should be administered during this period to maintain a constant state of anesthesia. Arterial partial pressure of carbon dioxide (Pco2) should be maintained at 30 mmHg with use of a volume ventilator, and the fraction of inspired oxygen (Fio2) should be increased to keep the pulmonary arterial pressure at less than |
Surgery_Schwartz_5133 | Surgery_Schwartz | dioxide (Pco2) should be maintained at 30 mmHg with use of a volume ventilator, and the fraction of inspired oxygen (Fio2) should be increased to keep the pulmonary arterial pressure at less than two-thirds of the systemic pressure.Results. Results of TAPVC in infancy have markedly improved in recent years, with an operative mortality of 5% or less in some series.79-82 This improvement is probably multifac-torial, mainly as a consequence of early noninvasive diagnosis and aggressive perioperative management. The routine use of echocardiography; improvements in myocardial protection with specific attention to the RV; creation of a large, tension-free anastomosis with maximal use of the venous confluence and atrial tissue; use of a sutureless technique in selected cases; and prevention of pulmonary hypertensive events have likely played a major role in reducing operative mortality. The importance of risk factors for early mortality, such as venous obstruction at presentation, urgency of | Surgery_Schwartz. dioxide (Pco2) should be maintained at 30 mmHg with use of a volume ventilator, and the fraction of inspired oxygen (Fio2) should be increased to keep the pulmonary arterial pressure at less than two-thirds of the systemic pressure.Results. Results of TAPVC in infancy have markedly improved in recent years, with an operative mortality of 5% or less in some series.79-82 This improvement is probably multifac-torial, mainly as a consequence of early noninvasive diagnosis and aggressive perioperative management. The routine use of echocardiography; improvements in myocardial protection with specific attention to the RV; creation of a large, tension-free anastomosis with maximal use of the venous confluence and atrial tissue; use of a sutureless technique in selected cases; and prevention of pulmonary hypertensive events have likely played a major role in reducing operative mortality. The importance of risk factors for early mortality, such as venous obstruction at presentation, urgency of |
Surgery_Schwartz_5134 | Surgery_Schwartz | hypertensive events have likely played a major role in reducing operative mortality. The importance of risk factors for early mortality, such as venous obstruction at presentation, urgency of operative repair, and infradiaphrag-matic anatomic type, has been debated.81,83Bando and colleagues84 made the controversial statement that both preoperative pulmonary venous obstruction and ana-tomic type had been neutralized as potential risk factors beyond calendar year 1991. Hyde et al82 similarly reported that connec-tion type was not related to outcome. However, a large single-institution report of 377 children with TAPVC by the author from the Hospital for Sick Children in Toronto85 found that, although outcomes had improved over time, patient anatomic factors were still important determinants of both survival and the need for subsequent reoperation. Risk factors for postrepair death were earlier operation year, younger age at repair, cardiac connection type, and postoperative pulmonary | Surgery_Schwartz. hypertensive events have likely played a major role in reducing operative mortality. The importance of risk factors for early mortality, such as venous obstruction at presentation, urgency of operative repair, and infradiaphrag-matic anatomic type, has been debated.81,83Bando and colleagues84 made the controversial statement that both preoperative pulmonary venous obstruction and ana-tomic type had been neutralized as potential risk factors beyond calendar year 1991. Hyde et al82 similarly reported that connec-tion type was not related to outcome. However, a large single-institution report of 377 children with TAPVC by the author from the Hospital for Sick Children in Toronto85 found that, although outcomes had improved over time, patient anatomic factors were still important determinants of both survival and the need for subsequent reoperation. Risk factors for postrepair death were earlier operation year, younger age at repair, cardiac connection type, and postoperative pulmonary |
Surgery_Schwartz_5135 | Surgery_Schwartz | of both survival and the need for subsequent reoperation. Risk factors for postrepair death were earlier operation year, younger age at repair, cardiac connection type, and postoperative pulmonary venous obstruc-tion. Risk-adjusted estimated 1-year survival for a patient repaired at birth with unfavorable morphology in 2006 was 37% (95% confidence interval [CI], 8%–80%) compared with 96% (95% CI, 91%–99%) for a patient with favorable morphology repaired at age 1 year. Freedom from reoperation was 82% ± 6% 4Figure 20-19. Chest x-ray of a newborn with obstructed infracar-diac type of TAPVR rescued by ECMO. Note the ECMO cannulas in the right neck (‘*’).InfracardiacTAPVCConventionalRepairSuturelessRepairFigure 20-20. Differences between conventional repair of total anomalous pulmonary venous connection (TAPVC) and sutureless repair of TAPVC. In the sutureless techniques, there are no sutures placed in the fragile veins themselves. Rather, the pericardial flaps are used to create a “well” | Surgery_Schwartz. of both survival and the need for subsequent reoperation. Risk factors for postrepair death were earlier operation year, younger age at repair, cardiac connection type, and postoperative pulmonary venous obstruc-tion. Risk-adjusted estimated 1-year survival for a patient repaired at birth with unfavorable morphology in 2006 was 37% (95% confidence interval [CI], 8%–80%) compared with 96% (95% CI, 91%–99%) for a patient with favorable morphology repaired at age 1 year. Freedom from reoperation was 82% ± 6% 4Figure 20-19. Chest x-ray of a newborn with obstructed infracar-diac type of TAPVR rescued by ECMO. Note the ECMO cannulas in the right neck (‘*’).InfracardiacTAPVCConventionalRepairSuturelessRepairFigure 20-20. Differences between conventional repair of total anomalous pulmonary venous connection (TAPVC) and sutureless repair of TAPVC. In the sutureless techniques, there are no sutures placed in the fragile veins themselves. Rather, the pericardial flaps are used to create a “well” |
Surgery_Schwartz_5136 | Surgery_Schwartz | connection (TAPVC) and sutureless repair of TAPVC. In the sutureless techniques, there are no sutures placed in the fragile veins themselves. Rather, the pericardial flaps are used to create a “well” for the pulmonary venous return (bottom inset). Early and late extrinsic stenosis are thought to be reduced using this latter technique.Brunicardi_Ch20_p0751-p0800.indd 76722/02/19 2:55 PM 768SPECIFIC CONSIDERATIONSPART IIat 11 years after repair, with increased risk associated with mixed connection and postoperative pulmonary venous obstruction. A study from the Hospital for Sick Children, Toronto, showed a lower incidence of reoperation in the sutureless technique com-pared to conventional pulmonary venous confluence–left atrial anastomosis.86 However, there was no statistically significant difference suggesting similar results between the strategies. Although the sutureless technique appears to have favorable outcomes at primary repair for TAPVC, long-term follow-up is necessary to | Surgery_Schwartz. connection (TAPVC) and sutureless repair of TAPVC. In the sutureless techniques, there are no sutures placed in the fragile veins themselves. Rather, the pericardial flaps are used to create a “well” for the pulmonary venous return (bottom inset). Early and late extrinsic stenosis are thought to be reduced using this latter technique.Brunicardi_Ch20_p0751-p0800.indd 76722/02/19 2:55 PM 768SPECIFIC CONSIDERATIONSPART IIat 11 years after repair, with increased risk associated with mixed connection and postoperative pulmonary venous obstruction. A study from the Hospital for Sick Children, Toronto, showed a lower incidence of reoperation in the sutureless technique com-pared to conventional pulmonary venous confluence–left atrial anastomosis.86 However, there was no statistically significant difference suggesting similar results between the strategies. Although the sutureless technique appears to have favorable outcomes at primary repair for TAPVC, long-term follow-up is necessary to |
Surgery_Schwartz_5137 | Surgery_Schwartz | difference suggesting similar results between the strategies. Although the sutureless technique appears to have favorable outcomes at primary repair for TAPVC, long-term follow-up is necessary to evaluate the occurrence of arrhythmias, such as complete heart block and atrial tachycardia, since an incision on the atrial septum and atrial wall is more invasive compared to the conventional technique.The most significant postoperative complication of TAPVC repair is pulmonary venous obstruction (Figure 20-21), which occurs 9% to 11% of the time, regardless of the surgi-cal technique employed. Mortality varies between 30% and 45%, and alternative catheter interventions do not offer defini-tive solutions.80 Recurrent pulmonary venous obstruction can be localized at the site of the pulmonary venous anastomosis (extrinsic), which usually can be cured with patch enlargement or balloon dilatation, or it may be secondary to endocardial thickening of the pulmonary venous ostia frequently | Surgery_Schwartz. difference suggesting similar results between the strategies. Although the sutureless technique appears to have favorable outcomes at primary repair for TAPVC, long-term follow-up is necessary to evaluate the occurrence of arrhythmias, such as complete heart block and atrial tachycardia, since an incision on the atrial septum and atrial wall is more invasive compared to the conventional technique.The most significant postoperative complication of TAPVC repair is pulmonary venous obstruction (Figure 20-21), which occurs 9% to 11% of the time, regardless of the surgi-cal technique employed. Mortality varies between 30% and 45%, and alternative catheter interventions do not offer defini-tive solutions.80 Recurrent pulmonary venous obstruction can be localized at the site of the pulmonary venous anastomosis (extrinsic), which usually can be cured with patch enlargement or balloon dilatation, or it may be secondary to endocardial thickening of the pulmonary venous ostia frequently |
Surgery_Schwartz_5138 | Surgery_Schwartz | venous anastomosis (extrinsic), which usually can be cured with patch enlargement or balloon dilatation, or it may be secondary to endocardial thickening of the pulmonary venous ostia frequently resulting in diffuse pulmonary venous sclerosis (intrinsic), which car-ries a 66% mortality rate because few good solutions exist.77 More commonly, postrepair left ventricular dysfunction can occur as the noncompliant LV suddenly is required to handle an increased volume load from redirected pulmonary venous return. This can manifest as an increase in pulmonary artery pressure but is distinguishable from primary pulmonary hyper-tension (another possible postoperative complication following repair of TAPVC) from the elevated left atrial pressure and LV dysfunction along with echocardiographic evidence of poor LV contractility. In pulmonary hypertension, the left atrial pressure may be low, the LV may appear “underfilled” (by echocardiog-raphy), and the RV may appear dilated. In either case, | Surgery_Schwartz. venous anastomosis (extrinsic), which usually can be cured with patch enlargement or balloon dilatation, or it may be secondary to endocardial thickening of the pulmonary venous ostia frequently resulting in diffuse pulmonary venous sclerosis (intrinsic), which car-ries a 66% mortality rate because few good solutions exist.77 More commonly, postrepair left ventricular dysfunction can occur as the noncompliant LV suddenly is required to handle an increased volume load from redirected pulmonary venous return. This can manifest as an increase in pulmonary artery pressure but is distinguishable from primary pulmonary hyper-tension (another possible postoperative complication following repair of TAPVC) from the elevated left atrial pressure and LV dysfunction along with echocardiographic evidence of poor LV contractility. In pulmonary hypertension, the left atrial pressure may be low, the LV may appear “underfilled” (by echocardiog-raphy), and the RV may appear dilated. In either case, |
Surgery_Schwartz_5139 | Surgery_Schwartz | of poor LV contractility. In pulmonary hypertension, the left atrial pressure may be low, the LV may appear “underfilled” (by echocardiog-raphy), and the RV may appear dilated. In either case, postop-erative support for a few days with extracorporeal membrane oxygenation may be lifesaving, and TAPVC should be repaired in centers that have this capacity.Some investigators have speculated that preoperative pul-monary venous obstruction is associated with increased medial thickness within the pulmonary vasculature, which may predis-pose these infants to intrinsic pulmonary venous stenosis despite adequate pulmonary venous decompression.82 The majority of studies demonstrating that preoperative pulmonary venous obstruction is a predictor of subsequent need for reoperation to correct recurrent pulmonary venous obstruction lend credence to this notion.Cor TriatriatumAnatomy. Cor triatriatum is a rare congenital heart defect char-acterized by the presence of a fibromuscular diaphragm that | Surgery_Schwartz. of poor LV contractility. In pulmonary hypertension, the left atrial pressure may be low, the LV may appear “underfilled” (by echocardiog-raphy), and the RV may appear dilated. In either case, postop-erative support for a few days with extracorporeal membrane oxygenation may be lifesaving, and TAPVC should be repaired in centers that have this capacity.Some investigators have speculated that preoperative pul-monary venous obstruction is associated with increased medial thickness within the pulmonary vasculature, which may predis-pose these infants to intrinsic pulmonary venous stenosis despite adequate pulmonary venous decompression.82 The majority of studies demonstrating that preoperative pulmonary venous obstruction is a predictor of subsequent need for reoperation to correct recurrent pulmonary venous obstruction lend credence to this notion.Cor TriatriatumAnatomy. Cor triatriatum is a rare congenital heart defect char-acterized by the presence of a fibromuscular diaphragm that |
Surgery_Schwartz_5140 | Surgery_Schwartz | pulmonary venous obstruction lend credence to this notion.Cor TriatriatumAnatomy. Cor triatriatum is a rare congenital heart defect char-acterized by the presence of a fibromuscular diaphragm that par-titions the left atrium into two chambers: a superior chamber that receives drainage from the pulmonary veins, and an inferior chamber that communicates with the mitral valve and the LV (Fig. 20-22). An ASD frequently exists between the superior chamber and the right atrium, or, more rarely, between the right atrium and the inferior chamber.Pathophysiology and Diagnosis. Cor triatriatum results in obstruction of pulmonary venous return to the left atrium. The degree of obstruction is variable and depends on the size of fen-estrations present in the left atrial membrane, the size of the ASD, and the existence of other associated anomalies. If the communication between the superior and inferior chambers is <3 mm, patients usually are symptomatic during the first year of life. The afflicted | Surgery_Schwartz. pulmonary venous obstruction lend credence to this notion.Cor TriatriatumAnatomy. Cor triatriatum is a rare congenital heart defect char-acterized by the presence of a fibromuscular diaphragm that par-titions the left atrium into two chambers: a superior chamber that receives drainage from the pulmonary veins, and an inferior chamber that communicates with the mitral valve and the LV (Fig. 20-22). An ASD frequently exists between the superior chamber and the right atrium, or, more rarely, between the right atrium and the inferior chamber.Pathophysiology and Diagnosis. Cor triatriatum results in obstruction of pulmonary venous return to the left atrium. The degree of obstruction is variable and depends on the size of fen-estrations present in the left atrial membrane, the size of the ASD, and the existence of other associated anomalies. If the communication between the superior and inferior chambers is <3 mm, patients usually are symptomatic during the first year of life. The afflicted |
Surgery_Schwartz_5141 | Surgery_Schwartz | the existence of other associated anomalies. If the communication between the superior and inferior chambers is <3 mm, patients usually are symptomatic during the first year of life. The afflicted infant will present with the stigmata of low cardiac output and pulmonary venous hypertension, as well as congestive heart failure and poor feeding.Physical examination may demonstrate a loud pulmonary S2 sound and a right ventricular heave, as well as jugular venous distention and hepatomegaly. Chest radiography will show car-diomegaly and pulmonary vascular prominence, and the ECG will suggest right ventricular hypertrophy. Two-dimensional echocardiography provides a definitive diagnosis in most cases, with catheterization necessary only when echocardiographic evaluation is equivocal.Therapy. Operative treatment for cor triatriatum is fairly simple. CPB and cardioplegic arrest are used. A right atriotomy usually Figure 20-21. Angiogram showing the discrete stenosis (‘*’) of the right-sided | Surgery_Schwartz. the existence of other associated anomalies. If the communication between the superior and inferior chambers is <3 mm, patients usually are symptomatic during the first year of life. The afflicted infant will present with the stigmata of low cardiac output and pulmonary venous hypertension, as well as congestive heart failure and poor feeding.Physical examination may demonstrate a loud pulmonary S2 sound and a right ventricular heave, as well as jugular venous distention and hepatomegaly. Chest radiography will show car-diomegaly and pulmonary vascular prominence, and the ECG will suggest right ventricular hypertrophy. Two-dimensional echocardiography provides a definitive diagnosis in most cases, with catheterization necessary only when echocardiographic evaluation is equivocal.Therapy. Operative treatment for cor triatriatum is fairly simple. CPB and cardioplegic arrest are used. A right atriotomy usually Figure 20-21. Angiogram showing the discrete stenosis (‘*’) of the right-sided |
Surgery_Schwartz_5142 | Surgery_Schwartz | treatment for cor triatriatum is fairly simple. CPB and cardioplegic arrest are used. A right atriotomy usually Figure 20-21. Angiogram showing the discrete stenosis (‘*’) of the right-sided pulmonary veins after conventional repair for supra-cardiac type TAPVC.Figure 20-22. Echocardiogram (apical 4 chamber view) showing the discrete membrane (‘*’) in a patient with Cor triatritum.Brunicardi_Ch20_p0751-p0800.indd 76822/02/19 2:55 PM 769CONGENITAL HEART DISEASECHAPTER 20allows access to the left atrial membrane through the existing ASD because it is dilated secondary to communication with the pulmonary venous chamber. The membrane is then excised, tak-ing care not to injure the mitral valve or the interatrial septum, and the ASD is closed with a patch. Alternatively, if the right atrium is small, the membrane can be exposed through an inci-sion directly into the superior left atrial chamber, just anterior to the right pulmonary veins. Surgical results are uniformly excel-lent for | Surgery_Schwartz. treatment for cor triatriatum is fairly simple. CPB and cardioplegic arrest are used. A right atriotomy usually Figure 20-21. Angiogram showing the discrete stenosis (‘*’) of the right-sided pulmonary veins after conventional repair for supra-cardiac type TAPVC.Figure 20-22. Echocardiogram (apical 4 chamber view) showing the discrete membrane (‘*’) in a patient with Cor triatritum.Brunicardi_Ch20_p0751-p0800.indd 76822/02/19 2:55 PM 769CONGENITAL HEART DISEASECHAPTER 20allows access to the left atrial membrane through the existing ASD because it is dilated secondary to communication with the pulmonary venous chamber. The membrane is then excised, tak-ing care not to injure the mitral valve or the interatrial septum, and the ASD is closed with a patch. Alternatively, if the right atrium is small, the membrane can be exposed through an inci-sion directly into the superior left atrial chamber, just anterior to the right pulmonary veins. Surgical results are uniformly excel-lent for |
Surgery_Schwartz_5143 | Surgery_Schwartz | is small, the membrane can be exposed through an inci-sion directly into the superior left atrial chamber, just anterior to the right pulmonary veins. Surgical results are uniformly excel-lent for this defect, with survival approaching 100%.The utility of catheter-based intervention for this diagnosis remains controversial, although there have been some reports of successful balloon dilatation.87Aortopulmonary WindowEmbryology and Anatomy. Aortopulmonary window (APW) is a rare congenital lesion, occurring in about 0.2% of patients, characterized by incomplete development of the septum that normally divides the truncus into the aorta and the pulmonary artery88In the vast majority of cases, APW occurs as a single defect of minimal length, which begins a few millimeters above the semilunar valves on the left lateral wall of the aorta (Fig. 20-23). Coronary artery anomalies, such as aberrant origin of the right or left coronary artery from the main pulmonary artery, are occa-sionally | Surgery_Schwartz. is small, the membrane can be exposed through an inci-sion directly into the superior left atrial chamber, just anterior to the right pulmonary veins. Surgical results are uniformly excel-lent for this defect, with survival approaching 100%.The utility of catheter-based intervention for this diagnosis remains controversial, although there have been some reports of successful balloon dilatation.87Aortopulmonary WindowEmbryology and Anatomy. Aortopulmonary window (APW) is a rare congenital lesion, occurring in about 0.2% of patients, characterized by incomplete development of the septum that normally divides the truncus into the aorta and the pulmonary artery88In the vast majority of cases, APW occurs as a single defect of minimal length, which begins a few millimeters above the semilunar valves on the left lateral wall of the aorta (Fig. 20-23). Coronary artery anomalies, such as aberrant origin of the right or left coronary artery from the main pulmonary artery, are occa-sionally |
Surgery_Schwartz_5144 | Surgery_Schwartz | valves on the left lateral wall of the aorta (Fig. 20-23). Coronary artery anomalies, such as aberrant origin of the right or left coronary artery from the main pulmonary artery, are occa-sionally present.Pathophysiology and Diagnosis. The dominant pathophysi-ology of APW is that of a large left-to-right shunt with increased pulmonary flow and the early development of congestive heart failure. Like other lesions with left-to-right flow, the magnitude of the shunt is determined by both the size of the defect and the pulmonary vascular resistance.Infants with APW present with frequent respiratory tract infections, tachypnea with feeding, and failure to thrive. Cya-nosis usually is absent because these infants deteriorate prior to the onset of significant pulmonary hypertension. The rapid decline with this defect occurs because shunt flow continues during both phases of the cardiac cycle, which limits systemic perfusion and increases ventricular work.89The diagnosis of APW begins with | Surgery_Schwartz. valves on the left lateral wall of the aorta (Fig. 20-23). Coronary artery anomalies, such as aberrant origin of the right or left coronary artery from the main pulmonary artery, are occa-sionally present.Pathophysiology and Diagnosis. The dominant pathophysi-ology of APW is that of a large left-to-right shunt with increased pulmonary flow and the early development of congestive heart failure. Like other lesions with left-to-right flow, the magnitude of the shunt is determined by both the size of the defect and the pulmonary vascular resistance.Infants with APW present with frequent respiratory tract infections, tachypnea with feeding, and failure to thrive. Cya-nosis usually is absent because these infants deteriorate prior to the onset of significant pulmonary hypertension. The rapid decline with this defect occurs because shunt flow continues during both phases of the cardiac cycle, which limits systemic perfusion and increases ventricular work.89The diagnosis of APW begins with |
Surgery_Schwartz_5145 | Surgery_Schwartz | decline with this defect occurs because shunt flow continues during both phases of the cardiac cycle, which limits systemic perfusion and increases ventricular work.89The diagnosis of APW begins with the physical exami-nation, which may demonstrate a systolic flow murmur, a hyperdynamic precordium, and bounding peripheral pulses. The chest radiograph will show pulmonary overcirculation and cardiomegaly, and the ECG will usually demonstrate either left ventricular hypertrophy or biventricular hypertrophy. Echocar-diography (Fig. 20-24) can detect the defect and also provide information about associated anomalies. Retrograde aortogra-phy will confirm the diagnosis but is rarely necessary.Therapy. All infants with APW require surgical correction once the diagnosis is made. Repair is undertaken through a median sternotomy and the use of CPB. The pulmonary arteries are occluded once the distal aorta is cannulated, and a transaor-tic repair using a prosthetic patch for pulmonary artery | Surgery_Schwartz. decline with this defect occurs because shunt flow continues during both phases of the cardiac cycle, which limits systemic perfusion and increases ventricular work.89The diagnosis of APW begins with the physical exami-nation, which may demonstrate a systolic flow murmur, a hyperdynamic precordium, and bounding peripheral pulses. The chest radiograph will show pulmonary overcirculation and cardiomegaly, and the ECG will usually demonstrate either left ventricular hypertrophy or biventricular hypertrophy. Echocar-diography (Fig. 20-24) can detect the defect and also provide information about associated anomalies. Retrograde aortogra-phy will confirm the diagnosis but is rarely necessary.Therapy. All infants with APW require surgical correction once the diagnosis is made. Repair is undertaken through a median sternotomy and the use of CPB. The pulmonary arteries are occluded once the distal aorta is cannulated, and a transaor-tic repair using a prosthetic patch for pulmonary artery |
Surgery_Schwartz_5146 | Surgery_Schwartz | through a median sternotomy and the use of CPB. The pulmonary arteries are occluded once the distal aorta is cannulated, and a transaor-tic repair using a prosthetic patch for pulmonary artery closure is then carried out. The coronary ostia must be carefully visual-ized and included on the aortic side of the patch. Alternatively, a two-patch technique can be used, which may eliminate recurrent fistulas from suture line leaks that occasionally occur with the single-patch method.90Results. Results are generally excellent, with an operative mortality in most large series of less than 5%.Vascular Rings and Pulmonary Artery SlingsVascular rings constitute a group of disorders derived from anomalies that result from abnormal development of the aortic arches resulting in compression of the trachea or esophagus. The surgical management of vascular rings dates back to 1945 when Dr. Gross described the surgical management of a kid with double aortic arch.91 Most children present with symptoms | Surgery_Schwartz. through a median sternotomy and the use of CPB. The pulmonary arteries are occluded once the distal aorta is cannulated, and a transaor-tic repair using a prosthetic patch for pulmonary artery closure is then carried out. The coronary ostia must be carefully visual-ized and included on the aortic side of the patch. Alternatively, a two-patch technique can be used, which may eliminate recurrent fistulas from suture line leaks that occasionally occur with the single-patch method.90Results. Results are generally excellent, with an operative mortality in most large series of less than 5%.Vascular Rings and Pulmonary Artery SlingsVascular rings constitute a group of disorders derived from anomalies that result from abnormal development of the aortic arches resulting in compression of the trachea or esophagus. The surgical management of vascular rings dates back to 1945 when Dr. Gross described the surgical management of a kid with double aortic arch.91 Most children present with symptoms |
Surgery_Schwartz_5147 | Surgery_Schwartz | or esophagus. The surgical management of vascular rings dates back to 1945 when Dr. Gross described the surgical management of a kid with double aortic arch.91 Most children present with symptoms during the first few months of life. Vascular rings can be com-plete (e.g., double aortic arch, right aortic arch with left liga-ment) or partial (e.g., innominate artery compression syndrome, pulmonary artery sling).Anatomy. The embryologic basis of vascular rings involves the development of six pairs of aortic arches and the dorsal and ventral aortae. The development of a specific type of vascular ring depends of the deletion or preservation of a specific seg-ment of these structures. The persistence of the right and left fourth arches leads to the development of double aortic arch. Persistence of the fourth right aortic arch and the involution of the left fourth arch leads to the development of a right aor-tic arch system with various combinations of mirror imaging Figure 20-23. Cartoon | Surgery_Schwartz. or esophagus. The surgical management of vascular rings dates back to 1945 when Dr. Gross described the surgical management of a kid with double aortic arch.91 Most children present with symptoms during the first few months of life. Vascular rings can be com-plete (e.g., double aortic arch, right aortic arch with left liga-ment) or partial (e.g., innominate artery compression syndrome, pulmonary artery sling).Anatomy. The embryologic basis of vascular rings involves the development of six pairs of aortic arches and the dorsal and ventral aortae. The development of a specific type of vascular ring depends of the deletion or preservation of a specific seg-ment of these structures. The persistence of the right and left fourth arches leads to the development of double aortic arch. Persistence of the fourth right aortic arch and the involution of the left fourth arch leads to the development of a right aor-tic arch system with various combinations of mirror imaging Figure 20-23. Cartoon |
Surgery_Schwartz_5148 | Surgery_Schwartz | of the fourth right aortic arch and the involution of the left fourth arch leads to the development of a right aor-tic arch system with various combinations of mirror imaging Figure 20-23. Cartoon depicting the various types of aortopulmonary window. (Used with permission from Nicholas Clarke MD.)Figure 20-24. Echo demonstrating an aortopulmonary window (‘*’).Type IType IIType IIIBrunicardi_Ch20_p0751-p0800.indd 76922/02/19 2:55 PM 770SPECIFIC CONSIDERATIONSPART IIbranching, aberrant subclavian arteries or with a left-sided liga-mentum arterisum. When the developing left lung captures its blood supply from the right sixth arch caudad to the tracheo-bronchial tree, it leads to the development of a pulmonary artery sling. The left pulmonary artery arises from the right pulmonary artery and then wraps around the trachea and esophagus forming a “sling.”92 The pathophysiology of innominate artery compres-sion syndrome is not very well understood.Pathophysiology and Diagnosis. The | Surgery_Schwartz. of the fourth right aortic arch and the involution of the left fourth arch leads to the development of a right aor-tic arch system with various combinations of mirror imaging Figure 20-23. Cartoon depicting the various types of aortopulmonary window. (Used with permission from Nicholas Clarke MD.)Figure 20-24. Echo demonstrating an aortopulmonary window (‘*’).Type IType IIType IIIBrunicardi_Ch20_p0751-p0800.indd 76922/02/19 2:55 PM 770SPECIFIC CONSIDERATIONSPART IIbranching, aberrant subclavian arteries or with a left-sided liga-mentum arterisum. When the developing left lung captures its blood supply from the right sixth arch caudad to the tracheo-bronchial tree, it leads to the development of a pulmonary artery sling. The left pulmonary artery arises from the right pulmonary artery and then wraps around the trachea and esophagus forming a “sling.”92 The pathophysiology of innominate artery compres-sion syndrome is not very well understood.Pathophysiology and Diagnosis. The |
Surgery_Schwartz_5149 | Surgery_Schwartz | and then wraps around the trachea and esophagus forming a “sling.”92 The pathophysiology of innominate artery compres-sion syndrome is not very well understood.Pathophysiology and Diagnosis. The symptoms associated with vascular rings include respiratory distress, barking cough, stridor, apnea, dysphagia, and recurrent respiratory tract infec-tions. The diagnosis often requires a high index of suspicion. Minor respiratory tract infections may precipitate serious respi-ratory distress. The work up includes chest X-rays, echocardiog-raphy, bronchoscopy, CT scan (Fig. 20-25), MRI (Fig. 20-26), and, rarely, cardiac catheterization. Chest X-rays show the rela-tionship of the aortic arch to the trachea. Tracheal compression can be better evaluated using lateral films. Unilateral hyperinfla-tion of the lung is sometimes seen and is often associated with a pulmonary artery sling (Fig. 20-27). PA slings (Fig. 20-28) are often associated with complete tracheal rings necessitating a bronchoscopy | Surgery_Schwartz. and then wraps around the trachea and esophagus forming a “sling.”92 The pathophysiology of innominate artery compres-sion syndrome is not very well understood.Pathophysiology and Diagnosis. The symptoms associated with vascular rings include respiratory distress, barking cough, stridor, apnea, dysphagia, and recurrent respiratory tract infec-tions. The diagnosis often requires a high index of suspicion. Minor respiratory tract infections may precipitate serious respi-ratory distress. The work up includes chest X-rays, echocardiog-raphy, bronchoscopy, CT scan (Fig. 20-25), MRI (Fig. 20-26), and, rarely, cardiac catheterization. Chest X-rays show the rela-tionship of the aortic arch to the trachea. Tracheal compression can be better evaluated using lateral films. Unilateral hyperinfla-tion of the lung is sometimes seen and is often associated with a pulmonary artery sling (Fig. 20-27). PA slings (Fig. 20-28) are often associated with complete tracheal rings necessitating a bronchoscopy |
Surgery_Schwartz_5150 | Surgery_Schwartz | of the lung is sometimes seen and is often associated with a pulmonary artery sling (Fig. 20-27). PA slings (Fig. 20-28) are often associated with complete tracheal rings necessitating a bronchoscopy when this diagnosis is made (Fig. 20-29). Patients with dysphagia require a barium esophagogram as a part of their work-up (Fig. 20-30).Treatment. All symptomatic patients should undergo surgery. On close questioning nearly all patients are symptomatic.93 The treatment varies depending on the type of vascular ring. A left posterolateral thoracotomy provides good exposure to most types. A right thoracotomy is often used for innominate artery compression syndrome, and a median sternotomy often with cardiopulmonary bypass is used to treat pulmonary artery slings with or without associated complete tracheal rings. The out-comes and results for vascular rings are excellent (Fig. 20-31). Video-assisted thoracoscopic approaches have been developed for the management of these conditions.94-96 The | Surgery_Schwartz. of the lung is sometimes seen and is often associated with a pulmonary artery sling (Fig. 20-27). PA slings (Fig. 20-28) are often associated with complete tracheal rings necessitating a bronchoscopy when this diagnosis is made (Fig. 20-29). Patients with dysphagia require a barium esophagogram as a part of their work-up (Fig. 20-30).Treatment. All symptomatic patients should undergo surgery. On close questioning nearly all patients are symptomatic.93 The treatment varies depending on the type of vascular ring. A left posterolateral thoracotomy provides good exposure to most types. A right thoracotomy is often used for innominate artery compression syndrome, and a median sternotomy often with cardiopulmonary bypass is used to treat pulmonary artery slings with or without associated complete tracheal rings. The out-comes and results for vascular rings are excellent (Fig. 20-31). Video-assisted thoracoscopic approaches have been developed for the management of these conditions.94-96 The |
Surgery_Schwartz_5151 | Surgery_Schwartz | tracheal rings. The out-comes and results for vascular rings are excellent (Fig. 20-31). Video-assisted thoracoscopic approaches have been developed for the management of these conditions.94-96 The criticism often stated involves retraction of vascular structures into the medias-tinum and losing control of the stumps prior to definitve control leading to exsanguination.96DEFECTS REQUIRING PALLIATIONTricuspid AtresiaTricuspid atresia occurs in 2% to 3% of patients with CHD and is characterized by atresia of the tricuspid valve. This results in discontinuity between the right atrium and RV. The RV is generally hypoplastic, and left-heart filling is dependent on an ASD. Tricuspid atresia is the most common form of the single-ventricle complex, indicating that there is functionally only one ventricular chamber.Anatomy. As mentioned, tricuspid atresia results in a lack of communication between the right atrium and the RV, and in the 5Figure 20-25. CT angiogram showing the four artery sign | Surgery_Schwartz. tracheal rings. The out-comes and results for vascular rings are excellent (Fig. 20-31). Video-assisted thoracoscopic approaches have been developed for the management of these conditions.94-96 The criticism often stated involves retraction of vascular structures into the medias-tinum and losing control of the stumps prior to definitve control leading to exsanguination.96DEFECTS REQUIRING PALLIATIONTricuspid AtresiaTricuspid atresia occurs in 2% to 3% of patients with CHD and is characterized by atresia of the tricuspid valve. This results in discontinuity between the right atrium and RV. The RV is generally hypoplastic, and left-heart filling is dependent on an ASD. Tricuspid atresia is the most common form of the single-ventricle complex, indicating that there is functionally only one ventricular chamber.Anatomy. As mentioned, tricuspid atresia results in a lack of communication between the right atrium and the RV, and in the 5Figure 20-25. CT angiogram showing the four artery sign |
Surgery_Schwartz_5152 | Surgery_Schwartz | chamber.Anatomy. As mentioned, tricuspid atresia results in a lack of communication between the right atrium and the RV, and in the 5Figure 20-25. CT angiogram showing the four artery sign classic of double aortic arch.Figure 20-26. MRI showing a double aortic arch.Figure 20-27. Unilateral hyperinflation of the left lung associ-ated with a rare vascular ring: left ascending aorta and right sided descending aorta.Figure 20-28. CT angiogram showing a PA sling. Note the LPA wrapping around behind the trachea.Brunicardi_Ch20_p0751-p0800.indd 77022/02/19 2:55 PM 771CONGENITAL HEART DISEASECHAPTER 20majority of patients there is no identifiable valve tissue or rem-nant.98 The right atrium is generally enlarged and muscular, with a fibrofatty floor. An unrestrictive ASD is usually present. The LV is often enlarged as it receives both systemic and pulmonary blood flow, but the left AV valve is usually normal.The RV, however, is usually severely hypoplastic, and there is sometimes a VSD in | Surgery_Schwartz. chamber.Anatomy. As mentioned, tricuspid atresia results in a lack of communication between the right atrium and the RV, and in the 5Figure 20-25. CT angiogram showing the four artery sign classic of double aortic arch.Figure 20-26. MRI showing a double aortic arch.Figure 20-27. Unilateral hyperinflation of the left lung associ-ated with a rare vascular ring: left ascending aorta and right sided descending aorta.Figure 20-28. CT angiogram showing a PA sling. Note the LPA wrapping around behind the trachea.Brunicardi_Ch20_p0751-p0800.indd 77022/02/19 2:55 PM 771CONGENITAL HEART DISEASECHAPTER 20majority of patients there is no identifiable valve tissue or rem-nant.98 The right atrium is generally enlarged and muscular, with a fibrofatty floor. An unrestrictive ASD is usually present. The LV is often enlarged as it receives both systemic and pulmonary blood flow, but the left AV valve is usually normal.The RV, however, is usually severely hypoplastic, and there is sometimes a VSD in |
Surgery_Schwartz_5153 | Surgery_Schwartz | LV is often enlarged as it receives both systemic and pulmonary blood flow, but the left AV valve is usually normal.The RV, however, is usually severely hypoplastic, and there is sometimes a VSD in its trabeculated or infundibular portion. In many cases, the interventricular communication is a site of obstruction to pulmonary blood flow, but obstruction may also occur at the level of the outlet valve or in the subval-vular infundibulum.99 In most cases, pulmonary blood flow is dependent on the presence of a PDA, and there may be no flow into the pulmonary circulation except for this PDA.Tricuspid atresia is classified according to the relationship of the great vessels and by the degree of obstruction to pulmo-nary blood flow. Because of the rarity of tricuspid atresia with transposed great arteries, we will restrict our discussion to tri-cuspid atresia with normally related great vessels.Pathophysiology. The main pathophysiology in tricuspid atresia is that of a univentricular heart | Surgery_Schwartz. LV is often enlarged as it receives both systemic and pulmonary blood flow, but the left AV valve is usually normal.The RV, however, is usually severely hypoplastic, and there is sometimes a VSD in its trabeculated or infundibular portion. In many cases, the interventricular communication is a site of obstruction to pulmonary blood flow, but obstruction may also occur at the level of the outlet valve or in the subval-vular infundibulum.99 In most cases, pulmonary blood flow is dependent on the presence of a PDA, and there may be no flow into the pulmonary circulation except for this PDA.Tricuspid atresia is classified according to the relationship of the great vessels and by the degree of obstruction to pulmo-nary blood flow. Because of the rarity of tricuspid atresia with transposed great arteries, we will restrict our discussion to tri-cuspid atresia with normally related great vessels.Pathophysiology. The main pathophysiology in tricuspid atresia is that of a univentricular heart |
Surgery_Schwartz_5154 | Surgery_Schwartz | arteries, we will restrict our discussion to tri-cuspid atresia with normally related great vessels.Pathophysiology. The main pathophysiology in tricuspid atresia is that of a univentricular heart of left ventricular morphology. That is, the LV must receive systemic blood via the interatrial communication and then distribute it to both the pulmonary circulation and the systemic circulation. Unless there is a VSD (as is found in some cases), pulmonary flow is dependent on the presence of a PDA. As the ductus begins to close shortly after birth, infants become intensely cyanotic. Reestablishing ductal patency (with PGE1) restores pulmonary blood flow and stabilizes patients for surgical intervention. Pulmonary hypertension is unusual in tricuspid atresia. However, occasional patients have a large VSD between the LV and the infundibular portion of the RV (just below the pulmonary valve). If there is no obstruction at the level of this VSD or at the valve, these infants may actually | Surgery_Schwartz. arteries, we will restrict our discussion to tri-cuspid atresia with normally related great vessels.Pathophysiology. The main pathophysiology in tricuspid atresia is that of a univentricular heart of left ventricular morphology. That is, the LV must receive systemic blood via the interatrial communication and then distribute it to both the pulmonary circulation and the systemic circulation. Unless there is a VSD (as is found in some cases), pulmonary flow is dependent on the presence of a PDA. As the ductus begins to close shortly after birth, infants become intensely cyanotic. Reestablishing ductal patency (with PGE1) restores pulmonary blood flow and stabilizes patients for surgical intervention. Pulmonary hypertension is unusual in tricuspid atresia. However, occasional patients have a large VSD between the LV and the infundibular portion of the RV (just below the pulmonary valve). If there is no obstruction at the level of this VSD or at the valve, these infants may actually |
Surgery_Schwartz_5155 | Surgery_Schwartz | a large VSD between the LV and the infundibular portion of the RV (just below the pulmonary valve). If there is no obstruction at the level of this VSD or at the valve, these infants may actually present with heart failure from excessive pulmonary blood flow. Regardless of whether these infants are “ductal-dependent” for pulmonary blood flow or have pulmonary blood flow provided across a VSD, they will be cyanotic since the obligatory right-to-left shunt at the atrial level will provide complete mixing of systemic and pulmonary venous return so that the LV ejects a hypoxemic mixture into the aorta.Diagnosis. The signs and symptoms of tricuspid atresia are dependent on the underlying anatomic variant, but most infants are cyanotic and hypoxic as a result of decreased pulmonary blood flow and the complete mixing at the atrial level. When pulmonary blood flow is provided through a VSD, there may be a prominent systolic murmur. Tricuspid atresia with pulmonary blood flow from a PDA may | Surgery_Schwartz. a large VSD between the LV and the infundibular portion of the RV (just below the pulmonary valve). If there is no obstruction at the level of this VSD or at the valve, these infants may actually present with heart failure from excessive pulmonary blood flow. Regardless of whether these infants are “ductal-dependent” for pulmonary blood flow or have pulmonary blood flow provided across a VSD, they will be cyanotic since the obligatory right-to-left shunt at the atrial level will provide complete mixing of systemic and pulmonary venous return so that the LV ejects a hypoxemic mixture into the aorta.Diagnosis. The signs and symptoms of tricuspid atresia are dependent on the underlying anatomic variant, but most infants are cyanotic and hypoxic as a result of decreased pulmonary blood flow and the complete mixing at the atrial level. When pulmonary blood flow is provided through a VSD, there may be a prominent systolic murmur. Tricuspid atresia with pulmonary blood flow from a PDA may |
Surgery_Schwartz_5156 | Surgery_Schwartz | and the complete mixing at the atrial level. When pulmonary blood flow is provided through a VSD, there may be a prominent systolic murmur. Tricuspid atresia with pulmonary blood flow from a PDA may present with the soft, continuous murmur of a PDA in conjunction with cyanosis.In the minority of patients with tricuspid atresia, symp-toms of congestive heart failure will predominate. This is often related to excessive flow across a VSD. The natural history of the muscular VSDs in these infants is that they will close and the congestive heart failure will dissipate and transform into cyano-sis with reduced pulmonary blood flow. Chest radiography will show decreased pulmonary vascularity. The ECG is strongly suggestive because uncharacteristic left axis deviation will be present, due to underdevelopment of the RV. Two-dimensional echocardiography readily confirms the diagnosis and the ana-tomic subtype. (Fig 20-32)Treatment. The treatment for tricuspid atresia in the earlier era of | Surgery_Schwartz. and the complete mixing at the atrial level. When pulmonary blood flow is provided through a VSD, there may be a prominent systolic murmur. Tricuspid atresia with pulmonary blood flow from a PDA may present with the soft, continuous murmur of a PDA in conjunction with cyanosis.In the minority of patients with tricuspid atresia, symp-toms of congestive heart failure will predominate. This is often related to excessive flow across a VSD. The natural history of the muscular VSDs in these infants is that they will close and the congestive heart failure will dissipate and transform into cyano-sis with reduced pulmonary blood flow. Chest radiography will show decreased pulmonary vascularity. The ECG is strongly suggestive because uncharacteristic left axis deviation will be present, due to underdevelopment of the RV. Two-dimensional echocardiography readily confirms the diagnosis and the ana-tomic subtype. (Fig 20-32)Treatment. The treatment for tricuspid atresia in the earlier era of |
Surgery_Schwartz_5157 | Surgery_Schwartz | underdevelopment of the RV. Two-dimensional echocardiography readily confirms the diagnosis and the ana-tomic subtype. (Fig 20-32)Treatment. The treatment for tricuspid atresia in the earlier era of palliation was aimed at correcting the defect in the pul-monary circulation. That is, patients with too much pulmonary flow received a pulmonary band, and those with insufficient flow received a systemic-to-pulmonary artery shunt. Systemic-to-pulmonary artery shunts, or Blalock–Taussig (BT) shunts, were first applied to patients with tricuspid atresia in the 1940s and 1950s.98 Likewise pulmonary artery banding was applied Figure 20-29. Rigid bronchoscopy showing complete tracheal rings in a the patient with pulmonary artery sling.Figure 20-30. Barium esophagogram showing posterior indenta-tion of the esophagus caused by a vascular ring (right aortic arch, aberrant left subclavian artery and left ligamentum).Brunicardi_Ch20_p0751-p0800.indd 77122/02/19 2:55 PM 772SPECIFIC | Surgery_Schwartz. underdevelopment of the RV. Two-dimensional echocardiography readily confirms the diagnosis and the ana-tomic subtype. (Fig 20-32)Treatment. The treatment for tricuspid atresia in the earlier era of palliation was aimed at correcting the defect in the pul-monary circulation. That is, patients with too much pulmonary flow received a pulmonary band, and those with insufficient flow received a systemic-to-pulmonary artery shunt. Systemic-to-pulmonary artery shunts, or Blalock–Taussig (BT) shunts, were first applied to patients with tricuspid atresia in the 1940s and 1950s.98 Likewise pulmonary artery banding was applied Figure 20-29. Rigid bronchoscopy showing complete tracheal rings in a the patient with pulmonary artery sling.Figure 20-30. Barium esophagogram showing posterior indenta-tion of the esophagus caused by a vascular ring (right aortic arch, aberrant left subclavian artery and left ligamentum).Brunicardi_Ch20_p0751-p0800.indd 77122/02/19 2:55 PM 772SPECIFIC |
Surgery_Schwartz_5158 | Surgery_Schwartz | indenta-tion of the esophagus caused by a vascular ring (right aortic arch, aberrant left subclavian artery and left ligamentum).Brunicardi_Ch20_p0751-p0800.indd 77122/02/19 2:55 PM 772SPECIFIC CONSIDERATIONSPART IIto patients with tricuspid atresia and congestive failure in 1957. However, despite the initial relief of either cyanosis or conges-tive heart failure, long-term mortality was high, as the single ventricle was left unprotected from either volume or pressure overload.99Recognizing the inadequacies of the initial repairs, Glenn described the first successful cavopulmonary anastomosis, an end-to-side right pulmonary artery-to-superior vena cava shunt in 1958, and later modified this to allow flow to both pulmonary arteries.100 This end-to-side right pulmonary artery-to-superior vena cava anastomosis was known as the bidirectional Glenn, and it is the first stage to final Fontan repair in widespread use today (Fig. 20-33). The Fontan repair was a major advancement in the | Surgery_Schwartz. indenta-tion of the esophagus caused by a vascular ring (right aortic arch, aberrant left subclavian artery and left ligamentum).Brunicardi_Ch20_p0751-p0800.indd 77122/02/19 2:55 PM 772SPECIFIC CONSIDERATIONSPART IIto patients with tricuspid atresia and congestive failure in 1957. However, despite the initial relief of either cyanosis or conges-tive heart failure, long-term mortality was high, as the single ventricle was left unprotected from either volume or pressure overload.99Recognizing the inadequacies of the initial repairs, Glenn described the first successful cavopulmonary anastomosis, an end-to-side right pulmonary artery-to-superior vena cava shunt in 1958, and later modified this to allow flow to both pulmonary arteries.100 This end-to-side right pulmonary artery-to-superior vena cava anastomosis was known as the bidirectional Glenn, and it is the first stage to final Fontan repair in widespread use today (Fig. 20-33). The Fontan repair was a major advancement in the |
Surgery_Schwartz_5159 | Surgery_Schwartz | vena cava anastomosis was known as the bidirectional Glenn, and it is the first stage to final Fontan repair in widespread use today (Fig. 20-33). The Fontan repair was a major advancement in the treatment of CHD, as it essentially bypassed the right heart and allowed separation of the pulmonary and systemic circulations. It was first performed by Fontan in 1971 and con-sisted of a classic Glenn anastomosis, ASD closure, and direct connection of the right atrium to the proximal end of the left pulmonary artery using an aortic homograft.101 The main pul-monary artery was ligated, and a homograft valve was inserted into the orifice of the inferior vena cava.Figure 20-32. Echo showing tricuspid atresia. The ‘*’ demonstrates the membranous tissue instead of the presence of a tricuspid valve.Figure 20-33. Angiogram showing a widely patent Glenn. The SVC (‘*’) is seen draining into the central pulmonary artery.Figure 20-31. Bronchoscopy before and after repair of a vascular ring: right | Surgery_Schwartz. vena cava anastomosis was known as the bidirectional Glenn, and it is the first stage to final Fontan repair in widespread use today (Fig. 20-33). The Fontan repair was a major advancement in the treatment of CHD, as it essentially bypassed the right heart and allowed separation of the pulmonary and systemic circulations. It was first performed by Fontan in 1971 and con-sisted of a classic Glenn anastomosis, ASD closure, and direct connection of the right atrium to the proximal end of the left pulmonary artery using an aortic homograft.101 The main pul-monary artery was ligated, and a homograft valve was inserted into the orifice of the inferior vena cava.Figure 20-32. Echo showing tricuspid atresia. The ‘*’ demonstrates the membranous tissue instead of the presence of a tricuspid valve.Figure 20-33. Angiogram showing a widely patent Glenn. The SVC (‘*’) is seen draining into the central pulmonary artery.Figure 20-31. Bronchoscopy before and after repair of a vascular ring: right |
Surgery_Schwartz_5160 | Surgery_Schwartz | 20-33. Angiogram showing a widely patent Glenn. The SVC (‘*’) is seen draining into the central pulmonary artery.Figure 20-31. Bronchoscopy before and after repair of a vascular ring: right arch, left descending aorta, and left ligament.Multiple modifications of this initial repair were per-formed over the next 20 years. One of the most important was the description by deLeval and colleagues of the creation of an interatrial lateral tunnel that allowed the inferior vena caval blood to be channeled exclusively to the superior vena cava.102 A total cavopulmonary connection could then be accomplished by dividing the superior vena cava and suturing the superior portion to the upper side of the right pulmonary artery and the inferior end to the augmented undersurface of the right pulmonary artery. Pulmonary flow then occurs passively, in a laminar fashion, driven by the central venous pressure. This repair became known as the modified Fontan operation.Another important modification, the | Surgery_Schwartz. 20-33. Angiogram showing a widely patent Glenn. The SVC (‘*’) is seen draining into the central pulmonary artery.Figure 20-31. Bronchoscopy before and after repair of a vascular ring: right arch, left descending aorta, and left ligament.Multiple modifications of this initial repair were per-formed over the next 20 years. One of the most important was the description by deLeval and colleagues of the creation of an interatrial lateral tunnel that allowed the inferior vena caval blood to be channeled exclusively to the superior vena cava.102 A total cavopulmonary connection could then be accomplished by dividing the superior vena cava and suturing the superior portion to the upper side of the right pulmonary artery and the inferior end to the augmented undersurface of the right pulmonary artery. Pulmonary flow then occurs passively, in a laminar fashion, driven by the central venous pressure. This repair became known as the modified Fontan operation.Another important modification, the |
Surgery_Schwartz_5161 | Surgery_Schwartz | Pulmonary flow then occurs passively, in a laminar fashion, driven by the central venous pressure. This repair became known as the modified Fontan operation.Another important modification, the fenestrated Fontan repair, was introduced in 1988.103 In this procedure, a residual 20% to 30% right-to-left shunt is either created or left unre-paired at the time of cavopulmonary connection to help sustain systemic output in the face of transient elevations in the pulmo-nary vascular resistance postoperatively.103Brunicardi_Ch20_p0751-p0800.indd 77222/02/19 2:55 PM 773CONGENITAL HEART DISEASECHAPTER 20The last notable variation on the original Fontan repair uses an extracardiac prosthetic tube graft (Fig. 20-34), usually 18 to 20 mm in diameter, as the conduit directing inferior vena cava blood to the pulmonary arteries.105 This technique has the advantages of decreasing atrial geometric alterations by avoid-ing intra-atrial suture lines and improving flow dynamics in the systemic venous | Surgery_Schwartz. Pulmonary flow then occurs passively, in a laminar fashion, driven by the central venous pressure. This repair became known as the modified Fontan operation.Another important modification, the fenestrated Fontan repair, was introduced in 1988.103 In this procedure, a residual 20% to 30% right-to-left shunt is either created or left unre-paired at the time of cavopulmonary connection to help sustain systemic output in the face of transient elevations in the pulmo-nary vascular resistance postoperatively.103Brunicardi_Ch20_p0751-p0800.indd 77222/02/19 2:55 PM 773CONGENITAL HEART DISEASECHAPTER 20The last notable variation on the original Fontan repair uses an extracardiac prosthetic tube graft (Fig. 20-34), usually 18 to 20 mm in diameter, as the conduit directing inferior vena cava blood to the pulmonary arteries.105 This technique has the advantages of decreasing atrial geometric alterations by avoid-ing intra-atrial suture lines and improving flow dynamics in the systemic venous |
Surgery_Schwartz_5162 | Surgery_Schwartz | to the pulmonary arteries.105 This technique has the advantages of decreasing atrial geometric alterations by avoid-ing intra-atrial suture lines and improving flow dynamics in the systemic venous pathway by maximizing laminar flow. Several investigators have shown a decrease in supraventricular arrhyth-mias, as well as an improvement in ventricular function, which may be secondary to decreased atrial tension and alleviation of chronic elevations in coronary sinus pressure.102,103One potential disadvantage of the extracardiac Fontan is that it delays performance of the Fontan in order to allow placement of a conduit of sufficient size. Despite these innova-tive approaches, the current strategy for operative management still relies on the idea of palliation. Patients are approached in a staged manner, to maximize their physiologic state so that they will survive to undergo a Fontan operation. The therapeu-tic strategy must begin in the neonatal period and should be directed toward | Surgery_Schwartz. to the pulmonary arteries.105 This technique has the advantages of decreasing atrial geometric alterations by avoid-ing intra-atrial suture lines and improving flow dynamics in the systemic venous pathway by maximizing laminar flow. Several investigators have shown a decrease in supraventricular arrhyth-mias, as well as an improvement in ventricular function, which may be secondary to decreased atrial tension and alleviation of chronic elevations in coronary sinus pressure.102,103One potential disadvantage of the extracardiac Fontan is that it delays performance of the Fontan in order to allow placement of a conduit of sufficient size. Despite these innova-tive approaches, the current strategy for operative management still relies on the idea of palliation. Patients are approached in a staged manner, to maximize their physiologic state so that they will survive to undergo a Fontan operation. The therapeu-tic strategy must begin in the neonatal period and should be directed toward |
Surgery_Schwartz_5163 | Surgery_Schwartz | a staged manner, to maximize their physiologic state so that they will survive to undergo a Fontan operation. The therapeu-tic strategy must begin in the neonatal period and should be directed toward reducing the patient’s subsequent risk factors for a Fontan procedure. Accordingly, small systemic pulmonary shunts, which are usually performed through a median sternot-omy, should be constructed for palliation of ductus-dependent univentricular physiology. This can easily be replaced with a bidirectional Glenn shunt or hemi-Fontan operation at 6 months of life. In non–ductus-dependent univentricular physiology, the infant can be managed medically until primary construction of a bidirectional cavopulmonary anastomosis becomes feasible. This is possible in the majority of cases because the physiologi-cally elevated pulmonary vascular resistance prevents pulmo-nary overcirculation during the neonatal period.The Fontan is usually performed when the child is between 2 and 4 years of age, and | Surgery_Schwartz. a staged manner, to maximize their physiologic state so that they will survive to undergo a Fontan operation. The therapeu-tic strategy must begin in the neonatal period and should be directed toward reducing the patient’s subsequent risk factors for a Fontan procedure. Accordingly, small systemic pulmonary shunts, which are usually performed through a median sternot-omy, should be constructed for palliation of ductus-dependent univentricular physiology. This can easily be replaced with a bidirectional Glenn shunt or hemi-Fontan operation at 6 months of life. In non–ductus-dependent univentricular physiology, the infant can be managed medically until primary construction of a bidirectional cavopulmonary anastomosis becomes feasible. This is possible in the majority of cases because the physiologi-cally elevated pulmonary vascular resistance prevents pulmo-nary overcirculation during the neonatal period.The Fontan is usually performed when the child is between 2 and 4 years of age, and |
Surgery_Schwartz_5164 | Surgery_Schwartz | elevated pulmonary vascular resistance prevents pulmo-nary overcirculation during the neonatal period.The Fontan is usually performed when the child is between 2 and 4 years of age, and it is generally successful if the infant was staged properly, with a protected single ventricle, and there is adequate pulmonary artery growth. The pulmonary vascular resistance should be below 4 Wood units, and the ejection frac-tion should be more than 45% to ensure success.106 In patients with high pulmonary artery pressure, fenestration of the atrial baffle may be helpful because their pulmonary vascular resis-tance may preclude adequate cardiac output postoperatively.99,103Results. Recent reports of the Fontan procedure for tricuspid atresia have been encouraging, with an overall survival of 86% and an operative mortality of 2%.107 The main complications following repair are atrial arrhythmias, particularly atrial flutter; conduit obstruction requiring reoperation; protein-losing enter-opathy; and | Surgery_Schwartz. elevated pulmonary vascular resistance prevents pulmo-nary overcirculation during the neonatal period.The Fontan is usually performed when the child is between 2 and 4 years of age, and it is generally successful if the infant was staged properly, with a protected single ventricle, and there is adequate pulmonary artery growth. The pulmonary vascular resistance should be below 4 Wood units, and the ejection frac-tion should be more than 45% to ensure success.106 In patients with high pulmonary artery pressure, fenestration of the atrial baffle may be helpful because their pulmonary vascular resis-tance may preclude adequate cardiac output postoperatively.99,103Results. Recent reports of the Fontan procedure for tricuspid atresia have been encouraging, with an overall survival of 86% and an operative mortality of 2%.107 The main complications following repair are atrial arrhythmias, particularly atrial flutter; conduit obstruction requiring reoperation; protein-losing enter-opathy; and |
Surgery_Schwartz_5165 | Surgery_Schwartz | operative mortality of 2%.107 The main complications following repair are atrial arrhythmias, particularly atrial flutter; conduit obstruction requiring reoperation; protein-losing enter-opathy; and decreased exercise tolerance.A prospective multi-institutional study from the Congeni-tal Heart Surgeons Society reported the outcomes of 150 neo-nates with tricuspid atresia and normally related great vessels.107 Five-year survival was 86%, and by the age of 2 years, 89% had undergone cavopulmonary anastomosis, and 75% of those surviving cavopulmonary anastomosis underwent Fontan opera-tion within 3 years. Competing risks methodology was used in this study to determine the rates of transition to end-states and their associated determinants (Fig. 20-35). Risk factors for death without cavopulmonary anastomosis in this study included the presence of mitral regurgitation and palliation with systemic-to-pulmonary artery shunts not originating from the innominate artery. Factors associated | Surgery_Schwartz. operative mortality of 2%.107 The main complications following repair are atrial arrhythmias, particularly atrial flutter; conduit obstruction requiring reoperation; protein-losing enter-opathy; and decreased exercise tolerance.A prospective multi-institutional study from the Congeni-tal Heart Surgeons Society reported the outcomes of 150 neo-nates with tricuspid atresia and normally related great vessels.107 Five-year survival was 86%, and by the age of 2 years, 89% had undergone cavopulmonary anastomosis, and 75% of those surviving cavopulmonary anastomosis underwent Fontan opera-tion within 3 years. Competing risks methodology was used in this study to determine the rates of transition to end-states and their associated determinants (Fig. 20-35). Risk factors for death without cavopulmonary anastomosis in this study included the presence of mitral regurgitation and palliation with systemic-to-pulmonary artery shunts not originating from the innominate artery. Factors associated |
Surgery_Schwartz_5166 | Surgery_Schwartz | anastomosis in this study included the presence of mitral regurgitation and palliation with systemic-to-pulmonary artery shunts not originating from the innominate artery. Factors associated with decreased transition rate to cavo-pulmonary anastomosis included patient variables (younger age at admission to a participating institution and noncardiac anom-alies) and procedural variables (larger systemic-to-pulmonary arterial shunt diameter and previous palliation).9Hypoplastic Left Heart SyndromeHLHS comprises a wide spectrum of cardiac malformations, including hypoplasia or atresia of the aortic and mitral valves and hypoplasia of the LV and ascending aorta.108 HLHS has a reported prevalence of 0.2 per 1000 live births and occurs twice as often in boys as in girls. Left untreated, HLHS is invari-ably fatal and is responsible for 25% of early cardiac deaths in neonates.109 However, the recent evolution of palliative surgical procedures has dramatically improved the outlook for patients | Surgery_Schwartz. anastomosis in this study included the presence of mitral regurgitation and palliation with systemic-to-pulmonary artery shunts not originating from the innominate artery. Factors associated with decreased transition rate to cavo-pulmonary anastomosis included patient variables (younger age at admission to a participating institution and noncardiac anom-alies) and procedural variables (larger systemic-to-pulmonary arterial shunt diameter and previous palliation).9Hypoplastic Left Heart SyndromeHLHS comprises a wide spectrum of cardiac malformations, including hypoplasia or atresia of the aortic and mitral valves and hypoplasia of the LV and ascending aorta.108 HLHS has a reported prevalence of 0.2 per 1000 live births and occurs twice as often in boys as in girls. Left untreated, HLHS is invari-ably fatal and is responsible for 25% of early cardiac deaths in neonates.109 However, the recent evolution of palliative surgical procedures has dramatically improved the outlook for patients |
Surgery_Schwartz_5167 | Surgery_Schwartz | invari-ably fatal and is responsible for 25% of early cardiac deaths in neonates.109 However, the recent evolution of palliative surgical procedures has dramatically improved the outlook for patients with HLHS, and an improved understanding of anatomic and physiologic alterations has spurred advances in parallel arenas such as intrauterine diagnosis and fetal intervention, echocardio-graphic imaging, and neonatal critical care.Anatomy. As implied by its name, HLHS involves varying degrees of underdevelopment of left-sided structures (Fig. 20-36), including the LV and the aortic and mitral valves. Thus, HLHS can be classified into four anatomic subtypes based on the val-vular morphology: (a) aortic and mitral stenosis; (b) aortic and mitral atresia; (c) aortic atresia and mitral stenosis; and (d) AS and mitral atresia. Aortic atresia tends to be associated with more severe degrees of hypoplasia of the ascending aorta than does AS.Even in cases without frank aortic atresia, however, the | Surgery_Schwartz. invari-ably fatal and is responsible for 25% of early cardiac deaths in neonates.109 However, the recent evolution of palliative surgical procedures has dramatically improved the outlook for patients with HLHS, and an improved understanding of anatomic and physiologic alterations has spurred advances in parallel arenas such as intrauterine diagnosis and fetal intervention, echocardio-graphic imaging, and neonatal critical care.Anatomy. As implied by its name, HLHS involves varying degrees of underdevelopment of left-sided structures (Fig. 20-36), including the LV and the aortic and mitral valves. Thus, HLHS can be classified into four anatomic subtypes based on the val-vular morphology: (a) aortic and mitral stenosis; (b) aortic and mitral atresia; (c) aortic atresia and mitral stenosis; and (d) AS and mitral atresia. Aortic atresia tends to be associated with more severe degrees of hypoplasia of the ascending aorta than does AS.Even in cases without frank aortic atresia, however, the |
Surgery_Schwartz_5168 | Surgery_Schwartz | (d) AS and mitral atresia. Aortic atresia tends to be associated with more severe degrees of hypoplasia of the ascending aorta than does AS.Even in cases without frank aortic atresia, however, the aortic arch is generally hypoplastic and, in severe cases, may even be interrupted. There is an associated coarctation shelf in 80% of patients with HLHS, and the ductus itself is usually quite large, as is the main pulmonary artery.7The segmental pulmonary arteries, however, are small, secondary to reduced intrauterine pulmonary blood flow, which is itself a consequence of the left-sided outflow obstruction (Fig. 20-36). The left atrial cavity is generally smaller than nor-mal and is accentuated because of the leftward displacement of the septum primum. There is almost always an interatrial com-munication via the foramen ovale, which can be large, but more Figure 20-34. Angiogram in a patient with a fenestrated extra-cardiac fontan constructed with a 20 mm Gore-tex tube graft | Surgery_Schwartz. (d) AS and mitral atresia. Aortic atresia tends to be associated with more severe degrees of hypoplasia of the ascending aorta than does AS.Even in cases without frank aortic atresia, however, the aortic arch is generally hypoplastic and, in severe cases, may even be interrupted. There is an associated coarctation shelf in 80% of patients with HLHS, and the ductus itself is usually quite large, as is the main pulmonary artery.7The segmental pulmonary arteries, however, are small, secondary to reduced intrauterine pulmonary blood flow, which is itself a consequence of the left-sided outflow obstruction (Fig. 20-36). The left atrial cavity is generally smaller than nor-mal and is accentuated because of the leftward displacement of the septum primum. There is almost always an interatrial com-munication via the foramen ovale, which can be large, but more Figure 20-34. Angiogram in a patient with a fenestrated extra-cardiac fontan constructed with a 20 mm Gore-tex tube graft |
Surgery_Schwartz_5169 | Surgery_Schwartz | com-munication via the foramen ovale, which can be large, but more Figure 20-34. Angiogram in a patient with a fenestrated extra-cardiac fontan constructed with a 20 mm Gore-tex tube graft (‘*’).Brunicardi_Ch20_p0751-p0800.indd 77322/02/19 2:55 PM 774SPECIFIC CONSIDERATIONSPART IIcommonly restricts right-to-left flow. In rare cases, there is no atrial-level communication, which can be lethal for these infants because there is no way for pulmonary venous return to cross over to the RV.Associated defects can occur with HLHS, and many of them have importance with respect to operative repair. For example, if a VSD is present, the LV can retain its normal size during development even in the presence of mitral atresia. This is because a right-to-left shunt through the defect impels growth of the LV.110 This introduces the feasibility of biventricular repair for this subset of patients.Although HLHS undoubtedly results from a complex interplay of developmental errors in the early stages | Surgery_Schwartz. com-munication via the foramen ovale, which can be large, but more Figure 20-34. Angiogram in a patient with a fenestrated extra-cardiac fontan constructed with a 20 mm Gore-tex tube graft (‘*’).Brunicardi_Ch20_p0751-p0800.indd 77322/02/19 2:55 PM 774SPECIFIC CONSIDERATIONSPART IIcommonly restricts right-to-left flow. In rare cases, there is no atrial-level communication, which can be lethal for these infants because there is no way for pulmonary venous return to cross over to the RV.Associated defects can occur with HLHS, and many of them have importance with respect to operative repair. For example, if a VSD is present, the LV can retain its normal size during development even in the presence of mitral atresia. This is because a right-to-left shunt through the defect impels growth of the LV.110 This introduces the feasibility of biventricular repair for this subset of patients.Although HLHS undoubtedly results from a complex interplay of developmental errors in the early stages |
Surgery_Schwartz_5170 | Surgery_Schwartz | the LV.110 This introduces the feasibility of biventricular repair for this subset of patients.Although HLHS undoubtedly results from a complex interplay of developmental errors in the early stages of cardio-genesis, many investigators have hypothesized that the altered blood flow is responsible for the structural underdevelopment that characterizes HLHS. In other words, if the stimulus for nor-mal development of the ascending aorta from the primordial aortic sac is high-pressure systemic blood flow from the LV through the aortic valve, then an atretic or stenotic aortic valve, which impedes flow and leads to only low-pressure diastolic retrograde flow via the ductus, will change the developmental signals and result in hypoplasia of the downstream structures (Fig. 20-37). Normal growth and development of the LV and mitral valve can be secondarily affected, resulting in hypoplasia or atresia of these structures.108Pathophysiology and Diagnosis. In HLHS, pulmonary venous blood enters | Surgery_Schwartz. the LV.110 This introduces the feasibility of biventricular repair for this subset of patients.Although HLHS undoubtedly results from a complex interplay of developmental errors in the early stages of cardio-genesis, many investigators have hypothesized that the altered blood flow is responsible for the structural underdevelopment that characterizes HLHS. In other words, if the stimulus for nor-mal development of the ascending aorta from the primordial aortic sac is high-pressure systemic blood flow from the LV through the aortic valve, then an atretic or stenotic aortic valve, which impedes flow and leads to only low-pressure diastolic retrograde flow via the ductus, will change the developmental signals and result in hypoplasia of the downstream structures (Fig. 20-37). Normal growth and development of the LV and mitral valve can be secondarily affected, resulting in hypoplasia or atresia of these structures.108Pathophysiology and Diagnosis. In HLHS, pulmonary venous blood enters |
Surgery_Schwartz_5171 | Surgery_Schwartz | and development of the LV and mitral valve can be secondarily affected, resulting in hypoplasia or atresia of these structures.108Pathophysiology and Diagnosis. In HLHS, pulmonary venous blood enters the left atrium, but atrial systole cannot propel blood across the stenotic or atretic mitral valve into the LV. Thus, the blood is shunted across the foramen ovale into the right atrium, where it contributes to volume loading of the RV. The end result is pulmonary venous hypertension from outflow obstruction at the level of the left atrium, as well as pulmonary overcirculation and right ventricular failure. As the pulmonary vascular resistance falls postnatally, the condition is exacerbated because right ventricular output is preferentially directed away from the systemic circulation, resulting in profound underperfu-sion of the coronary arteries and the vital organs. Closure of the ductus is incompatible with life in these neonates.Neonates with severe HLHS receive all pulmonary, | Surgery_Schwartz. and development of the LV and mitral valve can be secondarily affected, resulting in hypoplasia or atresia of these structures.108Pathophysiology and Diagnosis. In HLHS, pulmonary venous blood enters the left atrium, but atrial systole cannot propel blood across the stenotic or atretic mitral valve into the LV. Thus, the blood is shunted across the foramen ovale into the right atrium, where it contributes to volume loading of the RV. The end result is pulmonary venous hypertension from outflow obstruction at the level of the left atrium, as well as pulmonary overcirculation and right ventricular failure. As the pulmonary vascular resistance falls postnatally, the condition is exacerbated because right ventricular output is preferentially directed away from the systemic circulation, resulting in profound underperfu-sion of the coronary arteries and the vital organs. Closure of the ductus is incompatible with life in these neonates.Neonates with severe HLHS receive all pulmonary, |
Surgery_Schwartz_5172 | Surgery_Schwartz | resulting in profound underperfu-sion of the coronary arteries and the vital organs. Closure of the ductus is incompatible with life in these neonates.Neonates with severe HLHS receive all pulmonary, sys-temic, and coronary blood flow from the RV. Generally, a child with HLHS will present with respiratory distress within the first day of life, and mild cyanosis may be noted. These infants must be rapidly triaged to a tertiary center, and echocardiography should be performed to confirm the diagnosis. Prostaglandin E1 must be administered to maintain ductal patency, and the Figure 20-36. Echo In a patient with HLHS. Note the extremely hypoplastic left ventricle (‘*’).0200.00.40.81.2Years from diagnosis1.62.0406080100Proportion (%) of patients in each stateBDCPA (2 year prevalence = 90%)Dead without BDCPA(2 year prevalence = 5%)Single-stage Fontan(2 year prevalence = 1%)Alive without BDCPA(2 year prevalence = 4%)Figure 20-35. Competing risks depiction of events after diagnosis in 150 | Surgery_Schwartz. resulting in profound underperfu-sion of the coronary arteries and the vital organs. Closure of the ductus is incompatible with life in these neonates.Neonates with severe HLHS receive all pulmonary, sys-temic, and coronary blood flow from the RV. Generally, a child with HLHS will present with respiratory distress within the first day of life, and mild cyanosis may be noted. These infants must be rapidly triaged to a tertiary center, and echocardiography should be performed to confirm the diagnosis. Prostaglandin E1 must be administered to maintain ductal patency, and the Figure 20-36. Echo In a patient with HLHS. Note the extremely hypoplastic left ventricle (‘*’).0200.00.40.81.2Years from diagnosis1.62.0406080100Proportion (%) of patients in each stateBDCPA (2 year prevalence = 90%)Dead without BDCPA(2 year prevalence = 5%)Single-stage Fontan(2 year prevalence = 1%)Alive without BDCPA(2 year prevalence = 4%)Figure 20-35. Competing risks depiction of events after diagnosis in 150 |
Surgery_Schwartz_5173 | Surgery_Schwartz | without BDCPA(2 year prevalence = 5%)Single-stage Fontan(2 year prevalence = 1%)Alive without BDCPA(2 year prevalence = 4%)Figure 20-35. Competing risks depiction of events after diagnosis in 150 patients with tricuspid atresia. All patients began alive and thereafter migrated to one of four mutually exclusive end states (death, bidirectional cavopulmonary anastomosis [BDCPA], single-stage Fontan completion, or remaining alive without BDCPA) at time-dependent rates defined by the underlying hazard functions. At any point in time, the sum of propor-tions of children in each state is 100%. For example, estimated prevalences after 2 years from diagnosis are as follows: 89% BDCPA, 6% dead without BDCPA, 4% alive without BDCPA, and 1% single-stage Fontan completion. Solid lines represent parametric point estimates; dashed lines enclose 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses indicate the estimated propor-tion of patients | Surgery_Schwartz. without BDCPA(2 year prevalence = 5%)Single-stage Fontan(2 year prevalence = 1%)Alive without BDCPA(2 year prevalence = 4%)Figure 20-35. Competing risks depiction of events after diagnosis in 150 patients with tricuspid atresia. All patients began alive and thereafter migrated to one of four mutually exclusive end states (death, bidirectional cavopulmonary anastomosis [BDCPA], single-stage Fontan completion, or remaining alive without BDCPA) at time-dependent rates defined by the underlying hazard functions. At any point in time, the sum of propor-tions of children in each state is 100%. For example, estimated prevalences after 2 years from diagnosis are as follows: 89% BDCPA, 6% dead without BDCPA, 4% alive without BDCPA, and 1% single-stage Fontan completion. Solid lines represent parametric point estimates; dashed lines enclose 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses indicate the estimated propor-tion of patients |
Surgery_Schwartz_5174 | Surgery_Schwartz | point estimates; dashed lines enclose 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses indicate the estimated propor-tion of patients in each state at 2 years from diagnosis. (Reproduced with permission from Karamlou T, Ashburn DA, Caldarone CA, et al: Matching procedure to morphology improves outcomes in neonates with tricuspid atresia, J Thorac Cardiovasc Surg. 2005 Dec;130(6):1503-1510.) Brunicardi_Ch20_p0751-p0800.indd 77422/02/19 2:55 PM 775CONGENITAL HEART DISEASECHAPTER 20ventilatory settings must be adjusted to avoid excessive oxygen-ation and increase carbon dioxide tension. These maneuvers will maintain pulmonary vascular resistance and promote improved systemic perfusion.5,7,108 Cardiac catheterization should gener-ally be avoided because it is not usually helpful and might result in injury to the ductus and compromised renal function second-ary to the osmotic dye load.Treatment. In 1983, Norwood and colleagues | Surgery_Schwartz. point estimates; dashed lines enclose 70% confidence intervals; circles with error bars represent nonparametric estimates; numbers in parentheses indicate the estimated propor-tion of patients in each state at 2 years from diagnosis. (Reproduced with permission from Karamlou T, Ashburn DA, Caldarone CA, et al: Matching procedure to morphology improves outcomes in neonates with tricuspid atresia, J Thorac Cardiovasc Surg. 2005 Dec;130(6):1503-1510.) Brunicardi_Ch20_p0751-p0800.indd 77422/02/19 2:55 PM 775CONGENITAL HEART DISEASECHAPTER 20ventilatory settings must be adjusted to avoid excessive oxygen-ation and increase carbon dioxide tension. These maneuvers will maintain pulmonary vascular resistance and promote improved systemic perfusion.5,7,108 Cardiac catheterization should gener-ally be avoided because it is not usually helpful and might result in injury to the ductus and compromised renal function second-ary to the osmotic dye load.Treatment. In 1983, Norwood and colleagues |
Surgery_Schwartz_5175 | Surgery_Schwartz | be avoided because it is not usually helpful and might result in injury to the ductus and compromised renal function second-ary to the osmotic dye load.Treatment. In 1983, Norwood and colleagues described a two-stage palliative surgical procedure for relief of HLHS111 that was later modified to the currently used three-stage method of palliation.109 Stage 1 palliation, also known as the modified Norwood procedure (Fig. 20-38), bypasses the LV by creating a single outflow vessel, the neoaorta, which arises from the RV.The current technique of arch reconstruction involves completion of a connection between the pulmonary root, the native ascending aorta, and a piece of pulmonary homograft used to augment the diminutive native aorta. There are several modifications of this anastomosis, most notably the Damus-Kaye-Stansel (DKS) anastomosis, which involves dividing both the aorta and the pulmonary artery at the sinotubular junction. The proximal aorta is anastomosed to the proximal | Surgery_Schwartz. be avoided because it is not usually helpful and might result in injury to the ductus and compromised renal function second-ary to the osmotic dye load.Treatment. In 1983, Norwood and colleagues described a two-stage palliative surgical procedure for relief of HLHS111 that was later modified to the currently used three-stage method of palliation.109 Stage 1 palliation, also known as the modified Norwood procedure (Fig. 20-38), bypasses the LV by creating a single outflow vessel, the neoaorta, which arises from the RV.The current technique of arch reconstruction involves completion of a connection between the pulmonary root, the native ascending aorta, and a piece of pulmonary homograft used to augment the diminutive native aorta. There are several modifications of this anastomosis, most notably the Damus-Kaye-Stansel (DKS) anastomosis, which involves dividing both the aorta and the pulmonary artery at the sinotubular junction. The proximal aorta is anastomosed to the proximal |
Surgery_Schwartz_5176 | Surgery_Schwartz | most notably the Damus-Kaye-Stansel (DKS) anastomosis, which involves dividing both the aorta and the pulmonary artery at the sinotubular junction. The proximal aorta is anastomosed to the proximal pulmonary artery, creating a “double-barreled” outlet from the heart. This outlet is anastomosed to the distal aorta, which can be augmented with homograft material if there is an associated coarctation. At the completion of arch reconstruction, a 3.5or 4-mm shunt is placed from the innominate artery to the right pulmonary artery. The interatrial septum is then widely excised, thereby creating a large interatrial communication and prevent-ing pulmonary venous hypertension.The DKS connection, as described earlier, might avoid postoperative distortion of the tripartite connection in the neo-aorta, and thus decrease the risk of coronary insufficiency.112 It can be used when the aorta is 4 mm or larger. Unfortunately, in many infants with HLHS, especially if there is aortic atresia, the aorta | Surgery_Schwartz. most notably the Damus-Kaye-Stansel (DKS) anastomosis, which involves dividing both the aorta and the pulmonary artery at the sinotubular junction. The proximal aorta is anastomosed to the proximal pulmonary artery, creating a “double-barreled” outlet from the heart. This outlet is anastomosed to the distal aorta, which can be augmented with homograft material if there is an associated coarctation. At the completion of arch reconstruction, a 3.5or 4-mm shunt is placed from the innominate artery to the right pulmonary artery. The interatrial septum is then widely excised, thereby creating a large interatrial communication and prevent-ing pulmonary venous hypertension.The DKS connection, as described earlier, might avoid postoperative distortion of the tripartite connection in the neo-aorta, and thus decrease the risk of coronary insufficiency.112 It can be used when the aorta is 4 mm or larger. Unfortunately, in many infants with HLHS, especially if there is aortic atresia, the aorta |
Surgery_Schwartz_5177 | Surgery_Schwartz | and thus decrease the risk of coronary insufficiency.112 It can be used when the aorta is 4 mm or larger. Unfortunately, in many infants with HLHS, especially if there is aortic atresia, the aorta is diminutive and often less than 2 mm in diameter. The alternate technique available to provide pulmonary blood flow instead of a shunt is a RV-PA conduit often referred to as a “Sano.” It is usually a 5 or 6 mm ribbed Gore-tex graft.113The postoperative management of infants following stage 1 palliation is complex because favorable outcomes depend on establishing a delicate balance between pulmonary and systemic perfusion. Recent literature suggests that these infants require adequate postoperative cardiac output in order to supply both the pulmonary and the systemic circulations and that the use of oxi-metric catheters to monitor mixed venous oxygen saturation (Svo2) aids clinicians in both the selection of inotropic agents and in ventilatory management.114 Introduction of a shunt between | Surgery_Schwartz. and thus decrease the risk of coronary insufficiency.112 It can be used when the aorta is 4 mm or larger. Unfortunately, in many infants with HLHS, especially if there is aortic atresia, the aorta is diminutive and often less than 2 mm in diameter. The alternate technique available to provide pulmonary blood flow instead of a shunt is a RV-PA conduit often referred to as a “Sano.” It is usually a 5 or 6 mm ribbed Gore-tex graft.113The postoperative management of infants following stage 1 palliation is complex because favorable outcomes depend on establishing a delicate balance between pulmonary and systemic perfusion. Recent literature suggests that these infants require adequate postoperative cardiac output in order to supply both the pulmonary and the systemic circulations and that the use of oxi-metric catheters to monitor mixed venous oxygen saturation (Svo2) aids clinicians in both the selection of inotropic agents and in ventilatory management.114 Introduction of a shunt between |
Surgery_Schwartz_5178 | Surgery_Schwartz | of oxi-metric catheters to monitor mixed venous oxygen saturation (Svo2) aids clinicians in both the selection of inotropic agents and in ventilatory management.114 Introduction of a shunt between the RV and the pulmonary artery (Sano shunt) dimin-ishes the diastolic flow created by the modified BT shunt and may augment coronary perfusion, resulting in improved postop-erative cardiac function.113 A recent prospective, randomized, multi-institutional trial sponsored by the National Institutes of Health, the Systemic Ventricle Reconstruction (SVR) trial, com-pared the outcomes of neonates having either a modified Blalock–Taussig shunt (MBTS) or a Sano shunt.115 The SVR trial demonstrated that transplantation-free survival 12 months after randomization was higher with the Sano shunt than with the MBTS (74% vs. 64%, P = .01). However, the Sano shunt group had more unintended interventions (P = .003) and complications (P = .002). Right ventricular size and function at the age of 14 months | Surgery_Schwartz. of oxi-metric catheters to monitor mixed venous oxygen saturation (Svo2) aids clinicians in both the selection of inotropic agents and in ventilatory management.114 Introduction of a shunt between the RV and the pulmonary artery (Sano shunt) dimin-ishes the diastolic flow created by the modified BT shunt and may augment coronary perfusion, resulting in improved postop-erative cardiac function.113 A recent prospective, randomized, multi-institutional trial sponsored by the National Institutes of Health, the Systemic Ventricle Reconstruction (SVR) trial, com-pared the outcomes of neonates having either a modified Blalock–Taussig shunt (MBTS) or a Sano shunt.115 The SVR trial demonstrated that transplantation-free survival 12 months after randomization was higher with the Sano shunt than with the MBTS (74% vs. 64%, P = .01). However, the Sano shunt group had more unintended interventions (P = .003) and complications (P = .002). Right ventricular size and function at the age of 14 months |
Surgery_Schwartz_5179 | Surgery_Schwartz | the MBTS (74% vs. 64%, P = .01). However, the Sano shunt group had more unintended interventions (P = .003) and complications (P = .002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (± standard deviation) follow-up period of 32 ± 11 months showed a nonsignificant difference in transplanta-tion-free survival between the two groups (P = .06).115Since the initial SVR publications in 2010, the 3-year and 6-year results have been analyzed. At 3 years, the com-bined death and cardiac transplantation rates for the RVPAS vs. MBTS groups were 33% vs. 39% (P = 0.14). When all available data were examined by Kaplan-Meier analysis (mean follow-up 4.4 ± 1.0 years), there was also no difference between groups (log rank P = 0.11). Overall, there were 100 deaths and 10 trans-plantations in the MBTS cohort and 86 deaths and 11 transplan-tations in the RVPAS | Surgery_Schwartz. the MBTS (74% vs. 64%, P = .01). However, the Sano shunt group had more unintended interventions (P = .003) and complications (P = .002). Right ventricular size and function at the age of 14 months and the rate of nonfatal serious adverse events at the age of 12 months were similar in the two groups. Data collected over a mean (± standard deviation) follow-up period of 32 ± 11 months showed a nonsignificant difference in transplanta-tion-free survival between the two groups (P = .06).115Since the initial SVR publications in 2010, the 3-year and 6-year results have been analyzed. At 3 years, the com-bined death and cardiac transplantation rates for the RVPAS vs. MBTS groups were 33% vs. 39% (P = 0.14). When all available data were examined by Kaplan-Meier analysis (mean follow-up 4.4 ± 1.0 years), there was also no difference between groups (log rank P = 0.11). Overall, there were 100 deaths and 10 trans-plantations in the MBTS cohort and 86 deaths and 11 transplan-tations in the RVPAS |
Surgery_Schwartz_5180 | Surgery_Schwartz | years), there was also no difference between groups (log rank P = 0.11). Overall, there were 100 deaths and 10 trans-plantations in the MBTS cohort and 86 deaths and 11 transplan-tations in the RVPAS group.116 At 6 years, although the point averages continued to reflect a difference favoring the RVPAS (combined death/transplantation rate, 36%) in comparison with the MBTS (41%), the number of subjects was not sufficient to 6Figure 20-37. Angiogram obtained in a patient with HLSH (AS/MS). Note the extremely diminutive ascending aorta (‘*’).PatchmBTSRPALPAFigure 20-38. Cartoon depicting the Norwood procedure. The anas-tomosis of the aortic and pulmonary valve annulus is not shown. The ascending aorta and hyplastic arch are reconstructed by patch augmentation. The pulmonary blood flow has been provided in this case by a mBTS. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 77522/02/19 2:55 PM 776SPECIFIC CONSIDERATIONSPART IIdemonstrate a statistically | Surgery_Schwartz. years), there was also no difference between groups (log rank P = 0.11). Overall, there were 100 deaths and 10 trans-plantations in the MBTS cohort and 86 deaths and 11 transplan-tations in the RVPAS group.116 At 6 years, although the point averages continued to reflect a difference favoring the RVPAS (combined death/transplantation rate, 36%) in comparison with the MBTS (41%), the number of subjects was not sufficient to 6Figure 20-37. Angiogram obtained in a patient with HLSH (AS/MS). Note the extremely diminutive ascending aorta (‘*’).PatchmBTSRPALPAFigure 20-38. Cartoon depicting the Norwood procedure. The anas-tomosis of the aortic and pulmonary valve annulus is not shown. The ascending aorta and hyplastic arch are reconstructed by patch augmentation. The pulmonary blood flow has been provided in this case by a mBTS. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 77522/02/19 2:55 PM 776SPECIFIC CONSIDERATIONSPART IIdemonstrate a statistically |
Surgery_Schwartz_5181 | Surgery_Schwartz | been provided in this case by a mBTS. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 77522/02/19 2:55 PM 776SPECIFIC CONSIDERATIONSPART IIdemonstrate a statistically significant difference between the two groups (log rank P = 0.13). Similar to the 3-year results, RVPAS subjects had a higher incidence of any catheter inter-vention (0.38 vs. 0.23 interventions/patient-year, P <0.001), including balloon angioplasty (P = 0.014), stent (P = 0.009), and coiling (P <0.001).113,114 Currently, there remains an ongoing controversy regarding MBTS vs. RV-PA conduit as the source of pulmonary blood flow after the Norwood operation.119,120Although surgical palliation with the Norwood procedure is still the mainstay of therapy for infants with HLHS, a combined surgical and percutaneous option (hybrid procedure), which con-sists of bilateral pulmonary artery banding and placement of a ductal stent, has emerged as a promising alternative that obviates the need for CPB | Surgery_Schwartz. been provided in this case by a mBTS. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 77522/02/19 2:55 PM 776SPECIFIC CONSIDERATIONSPART IIdemonstrate a statistically significant difference between the two groups (log rank P = 0.13). Similar to the 3-year results, RVPAS subjects had a higher incidence of any catheter inter-vention (0.38 vs. 0.23 interventions/patient-year, P <0.001), including balloon angioplasty (P = 0.014), stent (P = 0.009), and coiling (P <0.001).113,114 Currently, there remains an ongoing controversy regarding MBTS vs. RV-PA conduit as the source of pulmonary blood flow after the Norwood operation.119,120Although surgical palliation with the Norwood procedure is still the mainstay of therapy for infants with HLHS, a combined surgical and percutaneous option (hybrid procedure), which con-sists of bilateral pulmonary artery banding and placement of a ductal stent, has emerged as a promising alternative that obviates the need for CPB |
Surgery_Schwartz_5182 | Surgery_Schwartz | percutaneous option (hybrid procedure), which con-sists of bilateral pulmonary artery banding and placement of a ductal stent, has emerged as a promising alternative that obviates the need for CPB in the fragile neonatal period.121,122 The hybrid procedure is performed in a “hybrid suite,” incorporating both advanced fluoroscopic imaging facilities combined with com-plete operating room capabilities. A 3or 3.5-mm PTFE tube graft is cut to a width of 3 to 4 mm and used as the bands on the branch pulmonary arteries, placed just distal to the main pulmo-nary artery. The ductal stent is then positioned in order to cover all ductal tissue and is deployed through a purse-string suture in the main pulmonary artery. A reverse systemic-to-pulmonary shunt is considered in patients with aortic atresia and preductal coarctation to improve coronary perfusion; however, a recent study demonstrated no difference in survival between those with and without the shunt.123 The hybrid procedure can also be | Surgery_Schwartz. percutaneous option (hybrid procedure), which con-sists of bilateral pulmonary artery banding and placement of a ductal stent, has emerged as a promising alternative that obviates the need for CPB in the fragile neonatal period.121,122 The hybrid procedure is performed in a “hybrid suite,” incorporating both advanced fluoroscopic imaging facilities combined with com-plete operating room capabilities. A 3or 3.5-mm PTFE tube graft is cut to a width of 3 to 4 mm and used as the bands on the branch pulmonary arteries, placed just distal to the main pulmo-nary artery. The ductal stent is then positioned in order to cover all ductal tissue and is deployed through a purse-string suture in the main pulmonary artery. A reverse systemic-to-pulmonary shunt is considered in patients with aortic atresia and preductal coarctation to improve coronary perfusion; however, a recent study demonstrated no difference in survival between those with and without the shunt.123 The hybrid procedure can also be |
Surgery_Schwartz_5183 | Surgery_Schwartz | and preductal coarctation to improve coronary perfusion; however, a recent study demonstrated no difference in survival between those with and without the shunt.123 The hybrid procedure can also be used as a bridge to heart transplantation in those infants with severe AV valve regurgitation or otherwise unsuitable single-ventricle anatomy.124Following stage 1 palliation, the second surgical proce-dure is the creation of a bidirectional cavopulmonary shunt (Fig. 20-39) or hemi-Fontan, generally at 3 to 6 months of life when the pulmonary vascular resistance has decreased to nor-mal levels. This is the first step in separating the pulmonary and systemic circulations, and it decreases the volume load on the single ventricle. The existing innominate artery-to-pulmonary shunt (or RV-to-pulmonary shunt) or MBTS is eliminated dur-ing the same operation.The third stage of surgical palliation, known as the modi-fied Fontan procedure, completes the separation of the sys-temic and pulmonary | Surgery_Schwartz. and preductal coarctation to improve coronary perfusion; however, a recent study demonstrated no difference in survival between those with and without the shunt.123 The hybrid procedure can also be used as a bridge to heart transplantation in those infants with severe AV valve regurgitation or otherwise unsuitable single-ventricle anatomy.124Following stage 1 palliation, the second surgical proce-dure is the creation of a bidirectional cavopulmonary shunt (Fig. 20-39) or hemi-Fontan, generally at 3 to 6 months of life when the pulmonary vascular resistance has decreased to nor-mal levels. This is the first step in separating the pulmonary and systemic circulations, and it decreases the volume load on the single ventricle. The existing innominate artery-to-pulmonary shunt (or RV-to-pulmonary shunt) or MBTS is eliminated dur-ing the same operation.The third stage of surgical palliation, known as the modi-fied Fontan procedure, completes the separation of the sys-temic and pulmonary |
Surgery_Schwartz_5184 | Surgery_Schwartz | shunt) or MBTS is eliminated dur-ing the same operation.The third stage of surgical palliation, known as the modi-fied Fontan procedure, completes the separation of the sys-temic and pulmonary circulations and is performed between 18 months and 3 years of age, or when the patient experiences increased cyanosis (i.e., has outgrown the capacity to perfuse the systemic circulation with adequately oxygenated blood). This has traditionally required a lateral tunnel within the right atrium to direct blood from the inferior vena cava to the pulmo-nary artery, allowing further relief of the volume load on the RV and providing increased pulmonary blood flow to alleviate cyanosis. More recently, many favor using an extracardiac con-duit (e.g., 18to 20-mm tube graft) to connect the inferior vena cava to the pulmonary artery (Fig. 20-40).Not all patients with HLHS require this three-stage pallia-tive repair. Some infants afflicted with a milder form of HLHS, recently described as hypoplastic left | Surgery_Schwartz. shunt) or MBTS is eliminated dur-ing the same operation.The third stage of surgical palliation, known as the modi-fied Fontan procedure, completes the separation of the sys-temic and pulmonary circulations and is performed between 18 months and 3 years of age, or when the patient experiences increased cyanosis (i.e., has outgrown the capacity to perfuse the systemic circulation with adequately oxygenated blood). This has traditionally required a lateral tunnel within the right atrium to direct blood from the inferior vena cava to the pulmo-nary artery, allowing further relief of the volume load on the RV and providing increased pulmonary blood flow to alleviate cyanosis. More recently, many favor using an extracardiac con-duit (e.g., 18to 20-mm tube graft) to connect the inferior vena cava to the pulmonary artery (Fig. 20-40).Not all patients with HLHS require this three-stage pallia-tive repair. Some infants afflicted with a milder form of HLHS, recently described as hypoplastic left |
Surgery_Schwartz_5185 | Surgery_Schwartz | to the pulmonary artery (Fig. 20-40).Not all patients with HLHS require this three-stage pallia-tive repair. Some infants afflicted with a milder form of HLHS, recently described as hypoplastic left heart complex (HLHC), have aortic or mitral hypoplasia without intrinsic valve stenosis and antegrade flow in the ascending aorta. In this group, a two-ventricle repair can be achieved with reasonable outcome. Tch-ervenkov has published the results with 12 patients with HLHC who underwent biventricular repair at a mean age of 7 days.114 The operative technique consisted of a pulmonary homograft patch aortoplasty of the aortic arch and ascending aorta and closure of the interatrial and interventricular communications. The left heart was capable of sustaining systemic perfusion in 92% of patients, and early mortality was 15.4%. Four patients required reoperations to relieve LVOT obstruction, most com-monly between 12 and 39 months following repair. The group from Boston Children’s Hospital | Surgery_Schwartz. to the pulmonary artery (Fig. 20-40).Not all patients with HLHS require this three-stage pallia-tive repair. Some infants afflicted with a milder form of HLHS, recently described as hypoplastic left heart complex (HLHC), have aortic or mitral hypoplasia without intrinsic valve stenosis and antegrade flow in the ascending aorta. In this group, a two-ventricle repair can be achieved with reasonable outcome. Tch-ervenkov has published the results with 12 patients with HLHC who underwent biventricular repair at a mean age of 7 days.114 The operative technique consisted of a pulmonary homograft patch aortoplasty of the aortic arch and ascending aorta and closure of the interatrial and interventricular communications. The left heart was capable of sustaining systemic perfusion in 92% of patients, and early mortality was 15.4%. Four patients required reoperations to relieve LVOT obstruction, most com-monly between 12 and 39 months following repair. The group from Boston Children’s Hospital |
Surgery_Schwartz_5186 | Surgery_Schwartz | and early mortality was 15.4%. Four patients required reoperations to relieve LVOT obstruction, most com-monly between 12 and 39 months following repair. The group from Boston Children’s Hospital has been very aggressive in left ventricular recruitment. These operations still carry a high burden of late death and several reoperations.Although the Norwood procedure is the most widely per-formed initial operation for HLHS, transplantation can be used as a first-line therapy and may be preferred when anatomic or physiologic considerations exist that preclude a favorable out-come with palliative repair. Significant tricuspid regurgitation, intractable pulmonary artery hypertension, or progressive right ventricular failure are cases where cardiac replacement may be advantageous. Widespread adaptation of transplantation as SVCLPAAtriumFigure 20-39. Cartoon depicting a bidirectional Glenn. (Used with permission from Kelly Rosso MD.)SVCGore-textube graftAtriumIVCFigure 20-40. Extra cardiac | Surgery_Schwartz. and early mortality was 15.4%. Four patients required reoperations to relieve LVOT obstruction, most com-monly between 12 and 39 months following repair. The group from Boston Children’s Hospital has been very aggressive in left ventricular recruitment. These operations still carry a high burden of late death and several reoperations.Although the Norwood procedure is the most widely per-formed initial operation for HLHS, transplantation can be used as a first-line therapy and may be preferred when anatomic or physiologic considerations exist that preclude a favorable out-come with palliative repair. Significant tricuspid regurgitation, intractable pulmonary artery hypertension, or progressive right ventricular failure are cases where cardiac replacement may be advantageous. Widespread adaptation of transplantation as SVCLPAAtriumFigure 20-39. Cartoon depicting a bidirectional Glenn. (Used with permission from Kelly Rosso MD.)SVCGore-textube graftAtriumIVCFigure 20-40. Extra cardiac |
Surgery_Schwartz_5187 | Surgery_Schwartz | of transplantation as SVCLPAAtriumFigure 20-39. Cartoon depicting a bidirectional Glenn. (Used with permission from Kelly Rosso MD.)SVCGore-textube graftAtriumIVCFigure 20-40. Extra cardiac fenestrated Fontan. ‘*’ shows the fen-estration. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 77622/02/19 2:55 PM 777CONGENITAL HEART DISEASECHAPTER 20first-line treatment for HLHS has been limited by improved Norwood survival rates as the operation and preand postop-erative management of the patient have evolved and by lim-ited organ availability. Organ availability should be considered prior to electing transplantation, as 24% of infants died awaiting transplantation in the largest series to date.126,127Results. Outcomes for HLHS are still significantly worse than those for other complex cardiac defects. However, with improvements in perioperative care and modifications in surgical technique, the survival following the Norwood proce-dure now exceeds 90% in | Surgery_Schwartz. of transplantation as SVCLPAAtriumFigure 20-39. Cartoon depicting a bidirectional Glenn. (Used with permission from Kelly Rosso MD.)SVCGore-textube graftAtriumIVCFigure 20-40. Extra cardiac fenestrated Fontan. ‘*’ shows the fen-estration. (Used with permission from Kelly Rosso MD.)Brunicardi_Ch20_p0751-p0800.indd 77622/02/19 2:55 PM 777CONGENITAL HEART DISEASECHAPTER 20first-line treatment for HLHS has been limited by improved Norwood survival rates as the operation and preand postop-erative management of the patient have evolved and by lim-ited organ availability. Organ availability should be considered prior to electing transplantation, as 24% of infants died awaiting transplantation in the largest series to date.126,127Results. Outcomes for HLHS are still significantly worse than those for other complex cardiac defects. However, with improvements in perioperative care and modifications in surgical technique, the survival following the Norwood proce-dure now exceeds 90% in |
Surgery_Schwartz_5188 | Surgery_Schwartz | those for other complex cardiac defects. However, with improvements in perioperative care and modifications in surgical technique, the survival following the Norwood proce-dure now exceeds 90% in experienced centers.115-120 The out-come for low-birth-weight infants has improved, but low weight still remains a major predictor of adverse survival, especially when accompanied by significant tricuspid valve insufficiency, a restructive interatrial communication, poor RV function, or extracardiac or chromosomal anomalies.DEFECTS THAT MAY BE PALLIATED OR REPAIREDEbstein’s AnomalyAnatomy. This is a rare defect, occurring in less than 1% of CHD patients. The predominant maldevelopment in this lesion is the inferior displacement of the tricuspid valve into the RV, although Bove128 and others have emphasized the fact that Ebstein’s anomaly is primarily a defect in right ventricular morphology rather than an isolated defect in the tricuspid valve. The anterior leaflet is usually attached in its | Surgery_Schwartz. those for other complex cardiac defects. However, with improvements in perioperative care and modifications in surgical technique, the survival following the Norwood proce-dure now exceeds 90% in experienced centers.115-120 The out-come for low-birth-weight infants has improved, but low weight still remains a major predictor of adverse survival, especially when accompanied by significant tricuspid valve insufficiency, a restructive interatrial communication, poor RV function, or extracardiac or chromosomal anomalies.DEFECTS THAT MAY BE PALLIATED OR REPAIREDEbstein’s AnomalyAnatomy. This is a rare defect, occurring in less than 1% of CHD patients. The predominant maldevelopment in this lesion is the inferior displacement of the tricuspid valve into the RV, although Bove128 and others have emphasized the fact that Ebstein’s anomaly is primarily a defect in right ventricular morphology rather than an isolated defect in the tricuspid valve. The anterior leaflet is usually attached in its |
Surgery_Schwartz_5189 | Surgery_Schwartz | emphasized the fact that Ebstein’s anomaly is primarily a defect in right ventricular morphology rather than an isolated defect in the tricuspid valve. The anterior leaflet is usually attached in its normal position to the annulus, but the septal and posterior leaflets are displaced toward the ventricle. This effectively divides the RV into two parts: the inlet portion (atrialized RV) and the outlet portion (true or trabeculated RV) (Fig. 20-41). The atrialized RV is usu-ally thin and dilated. Similarly, the tricuspid annulus and the right atrium are extremely dilated, and the tricuspid valve is usually regurgitant with a “sail-like” leaflet (Fig. 20-42). There is commonly an ASD present, which results in a right-to-left shunt at the atrial level. Occasionally, there is true anatomic pulmonary atresia or milder forms of RVOT obstruction.A Wolff-Parkinson-White (WPW) syndrome (Fig. 20-43) type of accessory pathway with associated preexcitation is pres-ent in 15% of | Surgery_Schwartz. emphasized the fact that Ebstein’s anomaly is primarily a defect in right ventricular morphology rather than an isolated defect in the tricuspid valve. The anterior leaflet is usually attached in its normal position to the annulus, but the septal and posterior leaflets are displaced toward the ventricle. This effectively divides the RV into two parts: the inlet portion (atrialized RV) and the outlet portion (true or trabeculated RV) (Fig. 20-41). The atrialized RV is usu-ally thin and dilated. Similarly, the tricuspid annulus and the right atrium are extremely dilated, and the tricuspid valve is usually regurgitant with a “sail-like” leaflet (Fig. 20-42). There is commonly an ASD present, which results in a right-to-left shunt at the atrial level. Occasionally, there is true anatomic pulmonary atresia or milder forms of RVOT obstruction.A Wolff-Parkinson-White (WPW) syndrome (Fig. 20-43) type of accessory pathway with associated preexcitation is pres-ent in 15% of |
Surgery_Schwartz_5190 | Surgery_Schwartz | true anatomic pulmonary atresia or milder forms of RVOT obstruction.A Wolff-Parkinson-White (WPW) syndrome (Fig. 20-43) type of accessory pathway with associated preexcitation is pres-ent in 15% of patients.128Pathophysiology. Right ventricular dysfunction occurs in patients with Ebstein’s anomaly because of two basic mecha-nisms: the inflow obstruction at the level of the atrialized ven-tricle, which produces ineffective RV filling and contractile dysfunction. Inflow obstruction and tricuspid regurgitation, which is exacerbated by progressive annular dilatation, both produce ineffective RV filling. Contractile dysfunction of the RV is a result of a decrease in the number of myocardial fibers, as well as the discordant contraction of the large atrialized portion.The lack of forward flow at the right ventricular level may lead to physiologic or functional pulmonary atresia, and the infant is dependent on ductal patency for survival. All sys-temic venous return must be directed through | Surgery_Schwartz. true anatomic pulmonary atresia or milder forms of RVOT obstruction.A Wolff-Parkinson-White (WPW) syndrome (Fig. 20-43) type of accessory pathway with associated preexcitation is pres-ent in 15% of patients.128Pathophysiology. Right ventricular dysfunction occurs in patients with Ebstein’s anomaly because of two basic mecha-nisms: the inflow obstruction at the level of the atrialized ven-tricle, which produces ineffective RV filling and contractile dysfunction. Inflow obstruction and tricuspid regurgitation, which is exacerbated by progressive annular dilatation, both produce ineffective RV filling. Contractile dysfunction of the RV is a result of a decrease in the number of myocardial fibers, as well as the discordant contraction of the large atrialized portion.The lack of forward flow at the right ventricular level may lead to physiologic or functional pulmonary atresia, and the infant is dependent on ductal patency for survival. All sys-temic venous return must be directed through |
Surgery_Schwartz_5191 | Surgery_Schwartz | the right ventricular level may lead to physiologic or functional pulmonary atresia, and the infant is dependent on ductal patency for survival. All sys-temic venous return must be directed through an ASD to the left atrium, where it can be shunted through the ductus for gas exchange. However, the left ventricular function is usually compromised in infants with severe Ebstein’s anomaly as well because the enormous RV and the to-and-fro flow within the atrialized RV prevent adequate intracardiac mixing. Left ven-tricular function may also be severely compromised in Ebstein’s anomaly because the large RV causes left ventricular compres-sion (Fig. 20-44A,B).Diagnosis. There is a spectrum of clinical presentation in infants with Ebstein’s anomaly that mirrors the anatomic spec-trum of this anomaly. Some infants with less severe forms may present with a mild degree of cyanosis, whereas the onset of clinical symptoms in patients surviving childhood is gradual, with the average age of | Surgery_Schwartz. the right ventricular level may lead to physiologic or functional pulmonary atresia, and the infant is dependent on ductal patency for survival. All sys-temic venous return must be directed through an ASD to the left atrium, where it can be shunted through the ductus for gas exchange. However, the left ventricular function is usually compromised in infants with severe Ebstein’s anomaly as well because the enormous RV and the to-and-fro flow within the atrialized RV prevent adequate intracardiac mixing. Left ven-tricular function may also be severely compromised in Ebstein’s anomaly because the large RV causes left ventricular compres-sion (Fig. 20-44A,B).Diagnosis. There is a spectrum of clinical presentation in infants with Ebstein’s anomaly that mirrors the anatomic spec-trum of this anomaly. Some infants with less severe forms may present with a mild degree of cyanosis, whereas the onset of clinical symptoms in patients surviving childhood is gradual, with the average age of |
Surgery_Schwartz_5192 | Surgery_Schwartz | anomaly. Some infants with less severe forms may present with a mild degree of cyanosis, whereas the onset of clinical symptoms in patients surviving childhood is gradual, with the average age of diagnosis in the mid-teens.However, the infant with severe atrialization and pulmo-nary stenosis will be both cyanotic and acidotic at birth. The chest radiograph may demonstrate the classic appearance, which 7Figure 20-41. Echo showing a patient with Ebsteins anomaly. Note the inferiorly displaced tricuspid valve (‘*’) and the atrialized por-tion of the RV (arrow).Figure 20-42. Echo in a patient with severe Ebsteins anomaly showing the large ‘sail like’ anterior leaflet (‘*’).Brunicardi_Ch20_p0751-p0800.indd 77722/02/19 2:56 PM 778SPECIFIC CONSIDERATIONSPART IIconsists of a globular “wall-to-wall” heart (Fig. 20-45), similar to that seen with pericardial effusion. The ECG may show right bundle-branch block and right axis deviation. WPW syndrome, as mentioned earlier, is a common finding | Surgery_Schwartz. anomaly. Some infants with less severe forms may present with a mild degree of cyanosis, whereas the onset of clinical symptoms in patients surviving childhood is gradual, with the average age of diagnosis in the mid-teens.However, the infant with severe atrialization and pulmo-nary stenosis will be both cyanotic and acidotic at birth. The chest radiograph may demonstrate the classic appearance, which 7Figure 20-41. Echo showing a patient with Ebsteins anomaly. Note the inferiorly displaced tricuspid valve (‘*’) and the atrialized por-tion of the RV (arrow).Figure 20-42. Echo in a patient with severe Ebsteins anomaly showing the large ‘sail like’ anterior leaflet (‘*’).Brunicardi_Ch20_p0751-p0800.indd 77722/02/19 2:56 PM 778SPECIFIC CONSIDERATIONSPART IIconsists of a globular “wall-to-wall” heart (Fig. 20-45), similar to that seen with pericardial effusion. The ECG may show right bundle-branch block and right axis deviation. WPW syndrome, as mentioned earlier, is a common finding |
Surgery_Schwartz_5193 | Surgery_Schwartz | heart (Fig. 20-45), similar to that seen with pericardial effusion. The ECG may show right bundle-branch block and right axis deviation. WPW syndrome, as mentioned earlier, is a common finding in these patients. Echocardiography will confirm the diagnosis and provide criti-cal information including tricuspid valvular function, size of the atrialized portion of the RV, degree of pulmonary stenosis, and the atrial size.128The Great Ormond Street Score (GOSE) (Table 20-1),129 which consists of the area of the right atrium plus the area of the atrialized portion of the RV divided by the diastolic area of the remaining cardiac chambers, has been proposed as a useful prognostic tool to stratify neonates with Ebstein’s anomaly. A score of greater than 2 translates into uniformly fatal outcome. Electrophysiology study with radiofrequency ablation is indi-cated in patients with evidence of WPW syndrome or in children Figure 20-43. EKG of a newborn with Ebsteins anomaly and WPW syndrome. Note | Surgery_Schwartz. heart (Fig. 20-45), similar to that seen with pericardial effusion. The ECG may show right bundle-branch block and right axis deviation. WPW syndrome, as mentioned earlier, is a common finding in these patients. Echocardiography will confirm the diagnosis and provide criti-cal information including tricuspid valvular function, size of the atrialized portion of the RV, degree of pulmonary stenosis, and the atrial size.128The Great Ormond Street Score (GOSE) (Table 20-1),129 which consists of the area of the right atrium plus the area of the atrialized portion of the RV divided by the diastolic area of the remaining cardiac chambers, has been proposed as a useful prognostic tool to stratify neonates with Ebstein’s anomaly. A score of greater than 2 translates into uniformly fatal outcome. Electrophysiology study with radiofrequency ablation is indi-cated in patients with evidence of WPW syndrome or in children Figure 20-43. EKG of a newborn with Ebsteins anomaly and WPW syndrome. Note |
Surgery_Schwartz_5194 | Surgery_Schwartz | Electrophysiology study with radiofrequency ablation is indi-cated in patients with evidence of WPW syndrome or in children Figure 20-43. EKG of a newborn with Ebsteins anomaly and WPW syndrome. Note the pre-excitation (arrow).ABFigure 20-44. A. Echo (short axis view) of a patient with severe Ebsteins anomaly showing the large RV (‘*’) and small LV (arrow) in diastole. B. Echo (short axis view) of a patient with severe Ebsteins anomaly showing the large RV (‘*’) and small ‘pancaked’ LV (arrow) in systole.Brunicardi_Ch20_p0751-p0800.indd 77822/02/19 2:56 PM 779CONGENITAL HEART DISEASECHAPTER 20with a history of supraventricular tachycardia, undefined wide-complex tachycardia, or syncope.Treatment. Surgery is indicated for symptomatic infants and for older children and adults with arrhythmias, progressive cya-nosis, or New York Heart Association class III or IV. How-ever, the operative repair may be different, depending on the patient’s age, because older children usually are | Surgery_Schwartz. Electrophysiology study with radiofrequency ablation is indi-cated in patients with evidence of WPW syndrome or in children Figure 20-43. EKG of a newborn with Ebsteins anomaly and WPW syndrome. Note the pre-excitation (arrow).ABFigure 20-44. A. Echo (short axis view) of a patient with severe Ebsteins anomaly showing the large RV (‘*’) and small LV (arrow) in diastole. B. Echo (short axis view) of a patient with severe Ebsteins anomaly showing the large RV (‘*’) and small ‘pancaked’ LV (arrow) in systole.Brunicardi_Ch20_p0751-p0800.indd 77822/02/19 2:56 PM 779CONGENITAL HEART DISEASECHAPTER 20with a history of supraventricular tachycardia, undefined wide-complex tachycardia, or syncope.Treatment. Surgery is indicated for symptomatic infants and for older children and adults with arrhythmias, progressive cya-nosis, or New York Heart Association class III or IV. How-ever, the operative repair may be different, depending on the patient’s age, because older children usually are |
Surgery_Schwartz_5195 | Surgery_Schwartz | arrhythmias, progressive cya-nosis, or New York Heart Association class III or IV. How-ever, the operative repair may be different, depending on the patient’s age, because older children usually are candidates for a biventricular or one-and-a-half ventricle repair, whereas moder-ate survival has been reported for neonates, using a procedure that converts the anatomy to a single-ventricle physiology, as described by Starnes and coworkers.130The surgical approach in widespread use today for patients surviving infancy was described by Danielson and colleagues in 1992.128,131 This procedure entails excision of redundant right atrial tissue and patch closure of any associated ASD, plication of the atrialized portion of the ventricle with obliteration of the aneurysmal cavity, posterior tricuspid annuloplasty to narrow the tricuspid annulus, reconstruction of the tricuspid valve if the anterior leaflet is satisfactory, or replacement of the tricuspid valve if necessary.131 If the tricuspid | Surgery_Schwartz. arrhythmias, progressive cya-nosis, or New York Heart Association class III or IV. How-ever, the operative repair may be different, depending on the patient’s age, because older children usually are candidates for a biventricular or one-and-a-half ventricle repair, whereas moder-ate survival has been reported for neonates, using a procedure that converts the anatomy to a single-ventricle physiology, as described by Starnes and coworkers.130The surgical approach in widespread use today for patients surviving infancy was described by Danielson and colleagues in 1992.128,131 This procedure entails excision of redundant right atrial tissue and patch closure of any associated ASD, plication of the atrialized portion of the ventricle with obliteration of the aneurysmal cavity, posterior tricuspid annuloplasty to narrow the tricuspid annulus, reconstruction of the tricuspid valve if the anterior leaflet is satisfactory, or replacement of the tricuspid valve if necessary.131 If the tricuspid |
Surgery_Schwartz_5196 | Surgery_Schwartz | annuloplasty to narrow the tricuspid annulus, reconstruction of the tricuspid valve if the anterior leaflet is satisfactory, or replacement of the tricuspid valve if necessary.131 If the tricuspid valve is not amenable to reconstruction, valve replacement should be considered. Care must be taken when performing the posterior annuloplasty, or during the conduct of tricuspid valve replacement, to avoid the conduction system, because complete heart block can compli-cate this procedure. In addition, patients who demonstrated preoperative evidence of preexcitation should undergo electro-physiologic mapping and ablation.Neonatal Ebstein’s anomaly is a separate entity. Results with surgical correction have been poor, and many neonates are not candidates for operative repair as previously described. Surgical options for the symptomatic neonate include palliative procedures, the one-and-a-half ventricle repair, or conversion to single-ventricle physiology.132 Arguably, the most favorable | Surgery_Schwartz. annuloplasty to narrow the tricuspid annulus, reconstruction of the tricuspid valve if the anterior leaflet is satisfactory, or replacement of the tricuspid valve if necessary.131 If the tricuspid valve is not amenable to reconstruction, valve replacement should be considered. Care must be taken when performing the posterior annuloplasty, or during the conduct of tricuspid valve replacement, to avoid the conduction system, because complete heart block can compli-cate this procedure. In addition, patients who demonstrated preoperative evidence of preexcitation should undergo electro-physiologic mapping and ablation.Neonatal Ebstein’s anomaly is a separate entity. Results with surgical correction have been poor, and many neonates are not candidates for operative repair as previously described. Surgical options for the symptomatic neonate include palliative procedures, the one-and-a-half ventricle repair, or conversion to single-ventricle physiology.132 Arguably, the most favorable |
Surgery_Schwartz_5197 | Surgery_Schwartz | Surgical options for the symptomatic neonate include palliative procedures, the one-and-a-half ventricle repair, or conversion to single-ventricle physiology.132 Arguably, the most favorable out-comes in symptomatic neonatal Ebstein’s anomaly or repair in slightly older infants have been achieved using the right ventric-ular exclusion premise. This technique, known as the “Starnes” procedure (Fig. 20-46),130 uses a fenestrated patch to close the tricuspid valve orifice coupled with systemic-to-pulmonary artery shunt. The patch must be fenestrated to allow decom-pression of the RV in instances of anatomic pulmonary atresia. Although Knott-Craig and colleagues132 have described tricus-pid valve repair for the full spectrum of neonates and infants with excellent shortand mid-term results, these results have not been reproduced in other institutions.133 The one-and-a-half ventricle repair was first described by Billingsly and cowork-ers as an attempt to achieve a more physiologic | Surgery_Schwartz. Surgical options for the symptomatic neonate include palliative procedures, the one-and-a-half ventricle repair, or conversion to single-ventricle physiology.132 Arguably, the most favorable out-comes in symptomatic neonatal Ebstein’s anomaly or repair in slightly older infants have been achieved using the right ventric-ular exclusion premise. This technique, known as the “Starnes” procedure (Fig. 20-46),130 uses a fenestrated patch to close the tricuspid valve orifice coupled with systemic-to-pulmonary artery shunt. The patch must be fenestrated to allow decom-pression of the RV in instances of anatomic pulmonary atresia. Although Knott-Craig and colleagues132 have described tricus-pid valve repair for the full spectrum of neonates and infants with excellent shortand mid-term results, these results have not been reproduced in other institutions.133 The one-and-a-half ventricle repair was first described by Billingsly and cowork-ers as an attempt to achieve a more physiologic |
Surgery_Schwartz_5198 | Surgery_Schwartz | these results have not been reproduced in other institutions.133 The one-and-a-half ventricle repair was first described by Billingsly and cowork-ers as an attempt to achieve a more physiologic “pulsatile” pul-monary circulation in patients with a hypoplastic or dysplastic RV.134 This is accomplished by diverting the superior vena caval blood directly into the pulmonary arterial system by a bidirec-tional cavopulmonary shunt while recruiting the RV to propel the inferior vena caval blood directly to the pulmonary arteries via the RVOT. Thus, the hemodynamics of the one-and-a-half ventricle repair are characterized by separate systemic and pul-monary circulations in series. The systemic circulation is fully supported by a systemic ventricle, and the pulmonary circula-tion is supported by both the bidirectional Glenn shunt and the hypoplastic (pulmonary) ventricle. Proponents of this approach report a decreased right atrial pressure and a decrease in inferior vena cava hypertension, | Surgery_Schwartz. these results have not been reproduced in other institutions.133 The one-and-a-half ventricle repair was first described by Billingsly and cowork-ers as an attempt to achieve a more physiologic “pulsatile” pul-monary circulation in patients with a hypoplastic or dysplastic RV.134 This is accomplished by diverting the superior vena caval blood directly into the pulmonary arterial system by a bidirec-tional cavopulmonary shunt while recruiting the RV to propel the inferior vena caval blood directly to the pulmonary arteries via the RVOT. Thus, the hemodynamics of the one-and-a-half ventricle repair are characterized by separate systemic and pul-monary circulations in series. The systemic circulation is fully supported by a systemic ventricle, and the pulmonary circula-tion is supported by both the bidirectional Glenn shunt and the hypoplastic (pulmonary) ventricle. Proponents of this approach report a decreased right atrial pressure and a decrease in inferior vena cava hypertension, |
Surgery_Schwartz_5199 | Surgery_Schwartz | both the bidirectional Glenn shunt and the hypoplastic (pulmonary) ventricle. Proponents of this approach report a decreased right atrial pressure and a decrease in inferior vena cava hypertension, which is theorized to be responsible for many of the dreaded complications of the Fontan circulation, including protein-losing encephalopathy, hepatic congestion, atrial arrhythmias, and systemic ventricular failure. In addition, the maintenance of pulsatile pulmonary blood flow, as opposed to continuous laminar flow as in the Fontan circulation, may be advantageous to the pulmonary microcirculation, although it has not been proven in any studies thus far.134,135 Certain criteria, most notably an adequate tricuspid valve Z score, as well as Figure 20-45. CXR in a newborn with severe Ebsteins anomaly showing a ‘wall-to-wall’ heart.Table 20-1The Great Ormond Street Score (GOSE)GOSE Score: Area of RA + aRA/Area of RV + LA + LVGOSE ScoreRatioMortality (%)1<0.5820.5–1.0831.1–1.41004>1.5100Figure | Surgery_Schwartz. both the bidirectional Glenn shunt and the hypoplastic (pulmonary) ventricle. Proponents of this approach report a decreased right atrial pressure and a decrease in inferior vena cava hypertension, which is theorized to be responsible for many of the dreaded complications of the Fontan circulation, including protein-losing encephalopathy, hepatic congestion, atrial arrhythmias, and systemic ventricular failure. In addition, the maintenance of pulsatile pulmonary blood flow, as opposed to continuous laminar flow as in the Fontan circulation, may be advantageous to the pulmonary microcirculation, although it has not been proven in any studies thus far.134,135 Certain criteria, most notably an adequate tricuspid valve Z score, as well as Figure 20-45. CXR in a newborn with severe Ebsteins anomaly showing a ‘wall-to-wall’ heart.Table 20-1The Great Ormond Street Score (GOSE)GOSE Score: Area of RA + aRA/Area of RV + LA + LVGOSE ScoreRatioMortality (%)1<0.5820.5–1.0831.1–1.41004>1.5100Figure |
Surgery_Schwartz_5200 | Surgery_Schwartz | showing a ‘wall-to-wall’ heart.Table 20-1The Great Ormond Street Score (GOSE)GOSE Score: Area of RA + aRA/Area of RV + LA + LVGOSE ScoreRatioMortality (%)1<0.5820.5–1.0831.1–1.41004>1.5100Figure 20-46. Echo appearance after a Starnes operation. Note the jet of flow across the fenestration In the patch.Brunicardi_Ch20_p0751-p0800.indd 77922/02/19 2:56 PM 780SPECIFIC CONSIDERATIONSPART IIthe absence of severe pulmonary hypertension or concomitant defects requiring intricate intracardiac repair, should be satis-fied prior to electing the one-and-a-half ventricle approach.136 Patients who do not fulfill these criteria may be approached with a two-ventricle repair and atrial fenestration or a Fontan repair.In the infant with severe Ebstein’s anomaly, initial stabili-zation with prostaglandin to maintain ductal patency, mechanical ventilation, and correction of cyanosis is mandatory. Metabolic acidosis, if present from compromised systemic perfusion, must be aggressively treated with | Surgery_Schwartz. showing a ‘wall-to-wall’ heart.Table 20-1The Great Ormond Street Score (GOSE)GOSE Score: Area of RA + aRA/Area of RV + LA + LVGOSE ScoreRatioMortality (%)1<0.5820.5–1.0831.1–1.41004>1.5100Figure 20-46. Echo appearance after a Starnes operation. Note the jet of flow across the fenestration In the patch.Brunicardi_Ch20_p0751-p0800.indd 77922/02/19 2:56 PM 780SPECIFIC CONSIDERATIONSPART IIthe absence of severe pulmonary hypertension or concomitant defects requiring intricate intracardiac repair, should be satis-fied prior to electing the one-and-a-half ventricle approach.136 Patients who do not fulfill these criteria may be approached with a two-ventricle repair and atrial fenestration or a Fontan repair.In the infant with severe Ebstein’s anomaly, initial stabili-zation with prostaglandin to maintain ductal patency, mechanical ventilation, and correction of cyanosis is mandatory. Metabolic acidosis, if present from compromised systemic perfusion, must be aggressively treated with |
Surgery_Schwartz_5201 | Surgery_Schwartz | to maintain ductal patency, mechanical ventilation, and correction of cyanosis is mandatory. Metabolic acidosis, if present from compromised systemic perfusion, must be aggressively treated with afterload reduction. Many of these infants will improve over 1 to 2 weeks as pulmonary vascu-lar resistance falls and they are able to improve antegrade flow into the pulmonary circulation through their abnormal RV and tricuspid valve. When stabilization and medical palliation fail, surgical management remains an option, although its success depends on numerous anatomic factors (e.g., adequacy of the tricuspid valve, RV, and pulmonary outflow tract), and surgery for symptomatic neonates with Ebstein’s anomaly carries a high risk. Knott-Craig and associates reported three cases where two-ventricle 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 | Surgery_Schwartz. to maintain ductal patency, mechanical ventilation, and correction of cyanosis is mandatory. Metabolic acidosis, if present from compromised systemic perfusion, must be aggressively treated with afterload reduction. Many of these infants will improve over 1 to 2 weeks as pulmonary vascu-lar resistance falls and they are able to improve antegrade flow into the pulmonary circulation through their abnormal RV and tricuspid valve. When stabilization and medical palliation fail, surgical management remains an option, although its success depends on numerous anatomic factors (e.g., adequacy of the tricuspid valve, RV, and pulmonary outflow tract), and surgery for symptomatic neonates with Ebstein’s anomaly carries a high risk. Knott-Craig and associates reported three cases where two-ventricle 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 |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.