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Surgery_Schwartz. primary repair during the first few months of life without prior palliative surgery.However, systemic-to-pulmonary shunts, generally an MBTS, may still be preferred with an unstable neonate younger than 3 months of age, when an extracardiac conduit is required because of an anomalous left anterior descending coronary artery, or when pulmonary atresia, significant branch pulmo-nary artery hypoplasia, or severe noncardiac anomalies coexist with TOF.Traditionally, TOF was repaired through a right ventricu-lotomy, providing excellent exposure for closure of the VSD and relief of the RVOT obstruction, but concerns that the resul-tant scar would significantly impair right ventricular function or lead to lethal arrhythmias led to the development of a transatrial approach. Transatrial repair, except in cases when the presence of diffuse RVOT hypoplasia requires insertion of a transannular patch, is now being increasingly advocated by many, although its superiority has not been conclusively


Surgery_Schwartz

Surgery_Schwartz. in cases when the presence of diffuse RVOT hypoplasia requires insertion of a transannular patch, is now being increasingly advocated by many, although its superiority has not been conclusively demonstrated.161The operative technique involves the use of CPB. All existing systemic-to-pulmonary arterial shunts, as well as the ductus arteriosus, are ligated. A right atriotomy is then made, and the anatomy of the VSD and the RVOT are assessed by retracting the tricuspid valve. The outflow tract obstruction is relieved by resecting the offending portion of the infundibular septum as well as any muscle trabeculations. If necessary, a pul-monary valvotomy or, alternatively, a longitudinal incision in the main pulmonary artery can be performed to improve expo-sure. The diameter of the pulmonary valve annulus is assessed by inserting Hegar dilators across the outflow tract; if the pul-monary artery/aorta diameter is less than 0.5, or the estimated RV/LV pressure is greater than 0.7, or the


Surgery_Schwartz

Surgery_Schwartz. valve annulus is assessed by inserting Hegar dilators across the outflow tract; if the pul-monary artery/aorta diameter is less than 0.5, or the estimated RV/LV pressure is greater than 0.7, or the size of the pulmo-nary valve is less than a Z score of −2.5, a transannular patch is inserted. Patch closure of the VSD is then accomplished, taking Figure 20-56. CT aortogram showing the large aorta often associated with conotruncal anomalies, rotated coronaries, and extremely hypoplastic main and branch pulmonary arteries in a patient with TOF.Figure 20-55. Chest x-ray showing a boot shaped heart in an infant with tetralogy of Fallot.Brunicardi_Ch20_p0751-p0800.indd 78522/02/19 2:56 PM 786SPECIFIC CONSIDERATIONSPART IIcare when placing sutures along the posteroinferior portion to avoid the conduction system.Results. Operative mortality for primary repair of TOF in infancy is less than 5% in most series.161 Previously reported risk factors such as transannular patch insertion or


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Surgery_Schwartz. the conduction system.Results. Operative mortality for primary repair of TOF in infancy is less than 5% in most series.161 Previously reported risk factors such as transannular patch insertion or younger age at time of repair have been eliminated secondary to improved intraoperative and postoperative care. According to the Society of Thoracic Surgeons Congenital Heart Surgery Database, dis-charge mortality from 3059 operations from 2002 to 2007 was 7.5% for initial palliation, 1.3% for primary repair, and 0.9% for staged repair, indicating similar outcomes for patients get-ting primary repair compared to staged repair.162 Nevertheless, for neonatal repair, discharge mortality increased to 6.2% with palliation and 7.8% with primary repair. This may be partly explained by a higher chance of postoperative complications in neonates.A major complication of repaired TOF is the develop-ment of pulmonary insufficiency, which subjects the RV to the adverse effects of acute and chronic volume


Surgery_Schwartz

Surgery_Schwartz. postoperative complications in neonates.A major complication of repaired TOF is the develop-ment of pulmonary insufficiency, which subjects the RV to the adverse effects of acute and chronic volume overload. This is especially problematic if residual lesions such as a VSD or peripheral pulmonary stenosis exist. Pulmonary valve regurgita-tion after repair of TOF is relatively well tolerated in the short term, partly because the hypertrophied RV usually adapts to the altered hemodynamic load.163 The detrimental effects of chronic pulmonary valve regurgitation are, however, numerous, and include progressive right ventricular dilatation and failure, tri-cuspid valve regurgitation, exercise intolerance, arrhythmia, and sudden death. Mechanoelectrical interaction, by which a dila-tated RV provides the substrate for electrical instability, might underlie the propensity toward ventricular arrhythmia.164 In sup-port of this contention, Gatzoulis and colleagues163,164 found that the risk of


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Surgery_Schwartz. the substrate for electrical instability, might underlie the propensity toward ventricular arrhythmia.164 In sup-port of this contention, Gatzoulis and colleagues163,164 found that the risk of symptomatic arrhythmia was high in patients with marked right ventricular enlargement and QRS prolongation on resting ECG of more than 180 ms. Karamlou et al have shown that similar structural and hemodynamic abnormalities, including a larger right atrial volume and right ventricular chamber size, are also related to atrial arrhythmias in patients following TOF repair.165 We found that prolongation of the QRS duration beyond a threshold of 160 ms increased the risk of atrial arrhythmias.165 Together, these data show that a similar mechanism could be responsible for both atrial and ventricular arrhythmias after repair in TOF patients.When significant deterioration of ventricular func-tion occurs, insertion of a pulmonary valve may be required, although this is rarely necessary in infants.


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Surgery_Schwartz. arrhythmias after repair in TOF patients.When significant deterioration of ventricular func-tion occurs, insertion of a pulmonary valve may be required, although this is rarely necessary in infants. Unfortunately, there are no universal criteria establishing the timing of pulmonary valve replacement. The current criteria for pulmonary valve replacement are the presence of two of the following criteria: RVEDD index >160 ml/m2, RVEDI >70 ml/m2, LVEDV index >65 ml/m2, RVEF <45%, RVOT aneurysm, and clinical symp-toms or signs, including syncope or VT.166 PVR can be achieved with minimal morbidity and mortality.167The alternative to surgical PVR is percutaneous pulmo-nary valve implantation. The Melody valve system (Fig. 20-57) is the most popular of such systems. Following risk adjustment, no significant differences were observed between surgical or transcatheter PVR. However, transcatheter PVR was associated with a shorter hospitalization. Hospitalization costs are similar for both


Surgery_Schwartz

Surgery_Schwartz. no significant differences were observed between surgical or transcatheter PVR. However, transcatheter PVR was associated with a shorter hospitalization. Hospitalization costs are similar for both procedures.168Arrhythmias are potentially the most serious late complication following TOF repair. In a multicenter cohort of 793 patients studied by Gatzoulis et al,164 a steady increase was documented in the prevalence of ventricular and atrial tachyarrhythmia and sudden cardiac death in the first 5 to 10 years after intracardiac repair. Clinical events were reported in 12% of patients at 35 years after repair. Prevalence of atrial arrhythmias from other studies, however, ranges from 1% to 11%,163,164 which is a reflection of the strong time dependence of arrhythmia onset.Underlying causes of arrhythmia following repair are complex and multifactorial, resulting in poorly defined opti-mum screening and treatment algorithms. Older repair age has been associated with an increased frequency of


Surgery_Schwartz

Surgery_Schwartz. following repair are complex and multifactorial, resulting in poorly defined opti-mum screening and treatment algorithms. Older repair age has been associated with an increased frequency of both atrial and ventricular arrhythmias. Impaired ventricular function second-ary to a protracted period of cyanosis before repair might con-tribute to the propensity for arrhythmia in older patients.Ventricular Septal DefectAnatomy. VSD refers to a hole between the LV and RV. These defects are common, comprising 20% to 30% of all cases of CHD, and may occur as an isolated lesion or as part of a more Figure 20-57. The Melody valve.Brunicardi_Ch20_p0751-p0800.indd 78622/02/19 2:56 PM 787CONGENITAL HEART DISEASECHAPTER 20complex malformation.169 VSDs vary in size from 3 to 4 mm to more than 3 cm and are classified into four types based on their location in the ventricular septum: perimembranous (or paramembranous, conoventricular), AV canal (inlet), outlet or supracristal, and muscular (Fig.


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Surgery_Schwartz. and are classified into four types based on their location in the ventricular septum: perimembranous (or paramembranous, conoventricular), AV canal (inlet), outlet or supracristal, and muscular (Fig. 20-58).Perimembranous VSDs are the most common type requir-ing surgical intervention, comprising approximately 80% of cases.169 These defects involve the membranous septum and include the malalignment defects seen in tetralogy of Fallot. In rare instances, the anterior and septal leaflets of the tricus-pid valve adhere to the edges of the perimembranous defect, forming a channel between the LV and the right atrium. These defects result in a large left-to-right shunt due to the large pres-sure differential between the two chambers.AV canal defects, also known as inlet defects, occur when part or all of the septum of the AV canal is absent. The VSD lies beneath the tricuspid valve and is limited upstream by the tricuspid annulus, without intervening muscle.The supracristal or outlet VSD


Surgery_Schwartz

Surgery_Schwartz. or all of the septum of the AV canal is absent. The VSD lies beneath the tricuspid valve and is limited upstream by the tricuspid annulus, without intervening muscle.The supracristal or outlet VSD results from a defect within the conal septum. Characteristically, these defects are limited upstream by the pulmonary valve and are otherwise surrounded by the muscle of the infundibular septum.Muscular VSDs are the most common type and may lie in four locations: anterior, midventricular, posterior, or apical. These are surrounded by muscle and can occur anywhere along the trabecular portion of the septum. The rare “Swiss-cheese” type of muscular VSD consists of multiple communications between the RV and LV, complicating operative repair.Pathophysiology and Clinical Presentation. The size of the VSD determines the initial pathophysiology of the disease. Large VSDs are classified as nonrestrictive and are at least equal in diameter to the aortic annulus. These defects allow free flow of


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Surgery_Schwartz. the VSD determines the initial pathophysiology of the disease. Large VSDs are classified as nonrestrictive and are at least equal in diameter to the aortic annulus. These defects allow free flow of blood from the LV to the RV, elevating right ventricular pres-sures to the same level as systemic pressure.Consequently, the pulmonary-to-systemic flow ratio (Qp to Qs) is inversely dependent on the ratio of pulmonary vas-cular resistance to systemic vascular resistance. Nonrestrictive VSDs produce a large increase in pulmonary blood flow, and the afflicted infant will present with symptoms of congestive heart failure. However, if untreated, these defects will cause pulmonary hypertension with a corresponding increase in pulmonary vascular resistance. This will lead to a reversal of flow (a right-to-left shunt), which is known as Eisenmenger’s syndrome.Small restrictive VSDs offer significant resistance to the passage of blood across the defect, and therefore right ventricu-lar pressure is


Surgery_Schwartz

Surgery_Schwartz. shunt), which is known as Eisenmenger’s syndrome.Small restrictive VSDs offer significant resistance to the passage of blood across the defect, and therefore right ventricu-lar pressure is either normal or only minimally elevated and the ratio of Qp to Qs rarely exceeds 1.5. These defects are generally asymptomatic because there are few physiologic consequences. However, there is a long-term risk of endocarditis because endo-cardial damage from the jet of blood through the defect may serve as a possible nidus for colonization (Fig. 20-59A,B).Diagnosis. The child with a large VSD will present with severe congestive heart failure and frequent respiratory tract infections. Children with Eisenmenger’s syndrome may be deceptively asymptomatic until frank cyanosis develops.The chest radiograph will show cardiomegaly and pulmo-nary overcirculation, and the ECG will show signs of left ven-tricular or biventricular hypertrophy. Echocardiography provides definitive diagnosis and can estimate


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Surgery_Schwartz. will show cardiomegaly and pulmo-nary overcirculation, and the ECG will show signs of left ven-tricular or biventricular hypertrophy. Echocardiography provides definitive diagnosis and can estimate the degree of shunting as well as pulmonary arterial pressures. Cardiac catheterization has MembranousMuscularInletSupracristalTVFigure 20-58. Types of VSD. (Used with permission from Kelly Rosso MD.)ABFigure 20-59. A. Severe TV and VSD endocarditis (‘*’) in a 4 yo untreated patient. B. Echocardiographic appearance of the same patient after patch repair(‘*’) of the VSD and complete exci-sion of the tricuspid valve.Brunicardi_Ch20_p0751-p0800.indd 78722/02/19 2:56 PM 788SPECIFIC CONSIDERATIONSPART IIlargely been supplanted by echocardiography, except in older children where measurement of pulmonary resistance is neces-sary prior to recommending closure of the defect.Treatment. VSDs may close or narrow spontaneously, and the probability of closure is inversely related to the age at which


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Surgery_Schwartz. resistance is neces-sary prior to recommending closure of the defect.Treatment. VSDs may close or narrow spontaneously, and the probability of closure is inversely related to the age at which the defect is observed. Thus, infants at 1 month of age have an 80% incidence of spontaneous closure, whereas a child at 12 months of age has only a 25% chance of closure.170 This has an important impact on operative decision-making because a small or moder-ate-size VSD may be observed for a period of time in the absence of symptoms. Large defects and those in severely symptomatic neonates should be repaired during infancy to relieve symptoms and because irreversible changes in pulmonary vascular resis-tance may develop during the first year of life.Repair of isolated VSDs requires the use of CPB with moderate hypothermia and cardioplegic arrest. The right atrial approach (Fig. 20-60) is preferable for most defects, except apical muscular defects, which often require a right ventricu-lotomy for


Surgery_Schwartz

Surgery_Schwartz. moderate hypothermia and cardioplegic arrest. The right atrial approach (Fig. 20-60) is preferable for most defects, except apical muscular defects, which often require a right ventricu-lotomy for adequate exposure. Supracristal defects may alter-natively be exposed via a pulmonary arteriotomy or through an incision in the RV immediately beneath the pulmonary valve (Fig. 20-61). Regardless of the type of defect present, a right atrial approach can be used initially to inspect the anatomy, as this may be abandoned should it offer inadequate exposure for repair. After careful inspection of the heart for any associated malformations, a patch repair is employed, taking care to avoid the conduction system. Routine use of intraoperative trans-esophageal echocardiography should be used to assess for any residual defect.Successful percutaneous device closure of VSDs using the Amplatzer device has been described.152 The device has demon-strated a 100% closure rate in a small series of patients


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Surgery_Schwartz. for any residual defect.Successful percutaneous device closure of VSDs using the Amplatzer device has been described.152 The device has demon-strated a 100% closure rate in a small series of patients with iso-lated or residual VSDs, or as a collaborative treatment strategy for the VSD component in more complex congenital lesions. Proponents of device closure argue that its use can decrease the complexity of surgical repair, avoid reoperation for a small residual lesion, or avoid the need for a ventriculotomy. The use of devices to close paramembranous defects can cause heart block because the defect is in close association to the conduction system (Fig 20-62).171 The procedure can be performed percuta-neously or through the per ventricular approach. Embolization of the device is an added risk.Multiple or “Swiss-cheese” VSDs represent a special case, and many cannot be repaired during infancy. In patients in whom definitive VSD closure cannot be accomplished, tem-porary placement of a


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Surgery_Schwartz. risk.Multiple or “Swiss-cheese” VSDs represent a special case, and many cannot be repaired during infancy. In patients in whom definitive VSD closure cannot be accomplished, tem-porary placement of a pulmonary artery band can be employed to control pulmonary flow. This allows time for spontaneous closure of many of the smaller defects, thus simplifying surgi-cal repair.172Some centers, however, have advocated early definitive repair of the Swiss-cheese septum, by using oversize patches, fibrin glue, and combined intraoperative device closure, as well as techniques to complete the repair transatrially.173Results. Even in very small infants, closure of VSDs can be safely performed with hospital mortality near 0%. The main risk factor remains the presence of other associated lesions, espe-cially when present in symptomatic neonates with large VSDs.Figure 20-60. Intra-op picture during a VSD closure performed by interrupted suture technique with patch closure.Figure


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Surgery_Schwartz. lesions, espe-cially when present in symptomatic neonates with large VSDs.Figure 20-60. Intra-op picture during a VSD closure performed by interrupted suture technique with patch closure.Figure 20-61. Echocardiographic appearance of a supracristal VSD (arrow). Note its location just beneath the pulmonary valve (‘*’).Brunicardi_Ch20_p0751-p0800.indd 78822/02/19 2:56 PM 789CONGENITAL HEART DISEASECHAPTER 20Atrioventricular Canal DefectsAnatomy. AV canal defects result from failure of fusion of the endocardial cushions in the central portion of the heart, caus-ing a lesion that involves the atrial and the ventricular septum, as well as the anterior mitral and septal tricuspid valve leaf-lets. Defects involving primarily the atrial septum are known as partial AV canal defects and frequently occur in conjunction with a cleft anterior mitral leaflet. Complete AV canal defects have a combined deficiency of the atrial and ventricular sep-tum associated with a common AV orifice rather than


Surgery_Schwartz

Surgery_Schwartz. occur in conjunction with a cleft anterior mitral leaflet. Complete AV canal defects have a combined deficiency of the atrial and ventricular sep-tum associated with a common AV orifice rather than separate tricuspid and mitral valves. The common AV valve generally has five leaflets, three lateral (free wall) and two bridging (septal) leaflets. The defect in the ventricular septum can lie either between the two bridging leaflets or beneath them. The relationship between the septal defect and the anterior bridging leaflet forms the basis of the Rastelli classification for complete AV canal defects (Fig. 20-63).174,175Pathophysiology and Diagnosis. Partial AV canal defects, in the absence of AV valvular regurgitation, frequently resemble isolated ASDs. Left-to-right shunting predominates as long as pulmonary vascular resistance remains low. However, 40% of patients with partial AV canal defects have moderate-to-severe valve incompetence, and progressive heart failure occurs early in


Surgery_Schwartz

Surgery_Schwartz. as long as pulmonary vascular resistance remains low. However, 40% of patients with partial AV canal defects have moderate-to-severe valve incompetence, and progressive heart failure occurs early in this patient population.175 Complete AV canal defects produce more severe pathophysiologic changes because the large intra-cardiac communication and significant AV valve regurgitation contribute to ventricular volume loading and pulmonary hyper-tension. Children with complete AV canal defects develop signs of congestive heart failure within the first few months of life.Physical examination may reveal a right ventricular heave and a systolic murmur. Children may also present with endo-carditis or paradoxical emboli as a result of the intracardiac communication. Chest radiography will be consistent with con-gestive heart failure, and the EKG demonstrates right ventricu-lar hypertrophy with a prolonged PR interval and is classically associated with left axis deviation.Two-dimensional


Surgery_Schwartz

Surgery_Schwartz. consistent with con-gestive heart failure, and the EKG demonstrates right ventricu-lar hypertrophy with a prolonged PR interval and is classically associated with left axis deviation.Two-dimensional echocardiography (Fig. 20-64) with color-flow mapping is confirmatory, but cardiac catheterization can be employed to define the status of the pulmonary vascula-ture, with a pulmonary vascular resistance greater than 12 Wood units indicating inoperability.Treatment. The management of patients with AV canal defects can be especially challenging. Timing of operation is individualized. Patients with partial defects can be electively repaired between 2 and 5 years of age, whereas complete AV canal defects should be repaired within the first year of life to prevent irreversible changes in the pulmonary circulation. Complete repair in infancy should be accomplished, with palliative procedures such as pulmonary artery banding reserved for only those infants with other complex lesions or who are


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Surgery_Schwartz. circulation. Complete repair in infancy should be accomplished, with palliative procedures such as pulmonary artery banding reserved for only those infants with other complex lesions or who are too ill to tolerate CPB.The operative technique requires the use of either continu-ous hypothermic CPB or, for small infants, deep hypothermic circulatory arrest. The heart is initially approached through an oblique right atriotomy, and the anatomy is carefully observed. In the case of a partial AV canal, the cleft in the mitral valve is repaired with interrupted sutures and the ASD is closed with a pericardial patch. Complete AV canal defects are repaired by patch closure of the VSD, separating the common AV valve into tricuspid and mitral components and suspending the neovalves from the top of the VSD patch and closing the ASD.Results. Partial AV canal defects have an excellent outcome, with a mortality rate of 0% to 2% in most series.175 Complete AV canal defects are associated with


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Surgery_Schwartz. of the VSD patch and closing the ASD.Results. Partial AV canal defects have an excellent outcome, with a mortality rate of 0% to 2% in most series.175 Complete AV canal defects are associated with anoperative mortality of 3% to 4%.176The most frequently encountered postoperative problems are complete heart block (1%–2%), right bundle-branch block (22%), arrhythmias (11%), RVOT obstruction (11%), and severe mitral regurgitation (13%–24%).175 The increasing use of intraoperative transesophageal echocardiography may positively Figure 20-62. Intraoperative picture at the time of removal of a percutaneously placed VSD device causing severe TR and complete heart block. Note the close association of the device to the tricuspid valve leaflet (arrow) and cordae.Type AType BType CFigure 20-63. Rastelli classification of complete AVSD. (Used with permission from Kelly Rosso MD.)Figure 20-64. Echo of an infant with complete AVSD. Note the prominent absence of the ‘crux’ (‘*’) of the heart in this


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Surgery_Schwartz. classification of complete AVSD. (Used with permission from Kelly Rosso MD.)Figure 20-64. Echo of an infant with complete AVSD. Note the prominent absence of the ‘crux’ (‘*’) of the heart in this defect.Brunicardi_Ch20_p0751-p0800.indd 78922/02/19 2:56 PM 790SPECIFIC CONSIDERATIONSPART IIinfluence outcomes, as the adequacy of repair can be assessed and treated without need for subsequent reoperation.174-175Interrupted Aortic ArchAnatomy. Interrupted aortic arch (IAA) is a rare defect, com-prising approximately 1% of all cases of CHD.177 It is defined as an absence of luminal continuity between the ascending and descending aorta and does not occur as an isolated defect in most cases because a VSD or PDA is usually present. IAA is classified based on the location of the interruption (Fig. 20-65 to Fig. 20-67).Clinical Manifestations and Diagnosis. Infants with IAA have ductal-dependent systemic blood flow and will develop profound metabolic acidosis and hemodynamic collapse upon


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Surgery_Schwartz. (Fig. 20-65 to Fig. 20-67).Clinical Manifestations and Diagnosis. Infants with IAA have ductal-dependent systemic blood flow and will develop profound metabolic acidosis and hemodynamic collapse upon ductal closure. In the rare instance of failed ductal closure, the diagnosis may be missed during infancy, and the child will pres-ent with symptoms of congestive heart failure from a persistent left-to-right shunt.Once definitive diagnosis is made in infants, usually with echocardiography, preparations are made for operative interven-tion, and prostaglandin E1 is infused to maintain ductal patency and correct acidosis. The infant’s hemodynamic status should Figure 20-66. CT angiogram of a Type A IAA.AoAoPAType AType BType CPAPAAoFigure 20-65. Types of IAA. (Used with permission from Nicholas Clarke MD.)Figure 20-67. MRI reconstruction of a Type B IAA.be optimized with mechanical ventilation and inotropic support. An effort should be made to increase pulmonary vascular resis-tance by


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Surgery_Schwartz. Clarke MD.)Figure 20-67. MRI reconstruction of a Type B IAA.be optimized with mechanical ventilation and inotropic support. An effort should be made to increase pulmonary vascular resis-tance by decreasing the fractional inspired oxygen and avoiding hyperventilation because this will preferentially direct blood into the systemic circulation.Treatment. Initial strategies for the management of IAA involved palliation though a left thoracotomy by using one of the arch vessels as a conduit to restore aortic continuity. Pulmo-nary artery banding can be simultaneously performed to limit left-to-right shunting because it is not feasible to repair the VSD or other intracardiac communications with this approach.However, complete one stage surgical repair in infants with IAA is now preferable. The operative technique involves use of a median sternotomy and CPB with short periods of cir-culatory arrest. Aortic arch reconstruction can be accomplished with either direct anastomosis or patch


Surgery_Schwartz

Surgery_Schwartz. The operative technique involves use of a median sternotomy and CPB with short periods of cir-culatory arrest. Aortic arch reconstruction can be accomplished with either direct anastomosis or patch aortoplasty followed by closure of the VSD.178In certain cases, the defect will involve hypoplasia of the left heart, precluding attempts at definitive repair. These infants should be managed with a Norwood procedure followed by a Fontan repair.Results. Outcomes in infants with IAA have improved sub-stantially over the last decades as a result of improved periop-erative care. Operative mortality is now less than 10% in most series.177,179 Some authors advocate the use of patch augmenta-tion of the aorta to ensure adequate relief of LVOT obstruction and to diminish anastomotic tension, thus reducing the subse-quent risk of restenosis and tracheobronchial compression.178Pediatric Mechanical Circulatory SupportMechanical circulatory support has become standard therapy for adults with end stage


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Surgery_Schwartz. the subse-quent risk of restenosis and tracheobronchial compression.178Pediatric Mechanical Circulatory SupportMechanical circulatory support has become standard therapy for adults with end stage heart failure. There has been a sig-nificant lag with development of similar devices for the pediatric population. This is probably related to the smaller mar-ket for these devices and the technical challenges associated with the anatomical constraints secondary to anatomy and size of the patients. Extracorporeal membrane oxygenation (ECMO) 8Brunicardi_Ch20_p0751-p0800.indd 79022/02/19 2:56 PM 791CONGENITAL HEART DISEASECHAPTER 20has been the mainstay of mechanical support in many centers for the pediatric population. The adaptation of other adult devices to the pediatric population las led to the slow but steady devel-opment of pediatric durable mechanical devices. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) device was approved by the FDA in 2011 in the United States as a


Surgery_Schwartz

Surgery_Schwartz. to the slow but steady devel-opment of pediatric durable mechanical devices. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) device was approved by the FDA in 2011 in the United States as a paracor-poreal device that can be used as a bridge to transplantation. This device has a 73% overall survival post implant at 12 months.97 Infection, stroke and bleeding remain significant morbidities associated with it. Young age and small body surface area still remain poor prognostic factors. In 2010, the National Heart, Lung, and Blood Institute launched the Pumps for Kids, Infants, and Neonates (PumpKIN) program to promote development of new devices with the goal of clinical use.ECMO remains the most commonly used form of mechan-ical support in the pediatric population in the United States. Per the ECLS Registry report released by the Extracorporeal Life Support Organization, as of January 2017, there were a total of 16,531 ECMO runs performed for cardiac causes, internation-ally.180


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Surgery_Schwartz. Per the ECLS Registry report released by the Extracorporeal Life Support Organization, as of January 2017, there were a total of 16,531 ECMO runs performed for cardiac causes, internation-ally.180 The survival to discharge is about 40% in the neona-tal population as opposed to 50% in the pediatric population. ECMO remains the only means of salvage for newborns and infants in many institutions. The biggest limitation remains the short duration it can be used. It is often used as a bridge to recovery and sometimes as a bridge to transplantation. The abil-ity to place small infants on ECMO with peripheral cannulation continues to make it a very attractive first line option.Ventricular assist devices can be either of the pulsatile or continuous types. The Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) remains a classic example of a pulsa-tile device. The Impella 2.5 (Abiomed) (Fig. 20-68) has been used in the pediatric population as a temporary support device for recovering


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Surgery_Schwartz. AG, Berlin, Germany) remains a classic example of a pulsa-tile device. The Impella 2.5 (Abiomed) (Fig. 20-68) has been used in the pediatric population as a temporary support device for recovering myocarditis, during treatment of acute rejection after heart transplantation and high-risk interventions in frag-ile patients with marginal function.181,182 Other continuous flow devices available for the pediatric patient include the Heartmate II Figure 20-68. Impella 2.5 (Abiomed). (Reproduced with permis-sion from Abiomed. Danvers, MA.)Figure 20-69. Heartware HVAD. (HeartWare® HVAD (Heart-Ware Inc., Miami Lakes, FL.)and Heartmate III devices (Thoratec, Pleasanton, CA), DeBakey VAD Child (MicroMed Technology, Houston, TX), PediMag (Thoratec, Pleasanton, CA), Jarvik2015 and HeartWare HVAD (Fig 20-69) (HeartWare international Inc, Framingham, MA).183 The total artificial heart (SynCardia Systems Inc, Tuscon, Az, USA) is an implantable biventricular device that replaces both ventricles. With


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Surgery_Schwartz. 20-69) (HeartWare international Inc, Framingham, MA).183 The total artificial heart (SynCardia Systems Inc, Tuscon, Az, USA) is an implantable biventricular device that replaces both ventricles. With the new introduction of the 50 ml pump, its popularity in the pediatric population has risen.Posttransplant survival of patients bridged with and with-out mechanical circulatory support (ventricular assist device or total artificial heart) at 5 years post transplant remains the same. However, patients bridged to transplant with ECMO have a sig-nificantly worse survival.184 All in all, the field of pediatric heart surgery is very exciting and rapidly expanding.Pediatric Heart TransplantationHeart transplantation is currently an accepted mode of therapy in infants and children. Annually, about 600 pediatric heart transplants are performed worldwide,184 about 400 of which are performed in the United States.185 The common indications for heart transplant in the pediatric population are


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Surgery_Schwartz. about 600 pediatric heart transplants are performed worldwide,184 about 400 of which are performed in the United States.185 The common indications for heart transplant in the pediatric population are congenital heart disease, dilated cardiomyopathy, retransplantation, and other rare indications (e.g., arrhythmogenic right ventricular dysplasia, cancer, muscular dystrophy, and restrictive cardio-myopathy). The most common congenital heart defect requir-ing transplantation remains hypoplastic left heart syndrome. Although in the past some centers have advocated primary heart transplantation for this lesion, the improved outcomes with surgical palliation have eliminated this as an option. The first year post transplant remains the greatest risk for mortality. The overall median survival is 20.7 years for infants, 18.2 years for children age 1 to 5 years, 14 years for age 6 to 10 years, and 12.7 years for those age 11 to 17 years.184 Males seem to have a modestly superior overall survival


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Surgery_Schwartz. years for infants, 18.2 years for children age 1 to 5 years, 14 years for age 6 to 10 years, and 12.7 years for those age 11 to 17 years.184 Males seem to have a modestly superior overall survival compared with females. The causes of mortality include cardiac allograft vasculopathy, acute Brunicardi_Ch20_p0751-p0800.indd 79122/02/19 2:57 PM 792SPECIFIC CONSIDERATIONSPART IIrejection, infections, and graft failures. In the current era, the expected 1-year survival rate is 80% to 90%, the 2-year survival rate is 80% to 85%, and the 5-year survival rate is approximately 70% to 80% in experienced centers.186 Interestingly, infants who undergo transplantation in the first month of life appear to have a survival advantage over infants who undergo transplantation during the remainder of the first year of life.The two main techniques for performing the implant of the heart are the right atrial technique developed by Lower and Shumway and the bicaval-left atrial technique described by


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Surgery_Schwartz. the first year of life.The two main techniques for performing the implant of the heart are the right atrial technique developed by Lower and Shumway and the bicaval-left atrial technique described by Sievers and associates.187 In the latter technique, implantation consists of five anastamoses performed using a running prolene suture. These include the left atrial cuff, aorta, pulmonary artery, and the superior and inferior vena cave. One of the cornerstones of postoperative management remains immunosuppression. The triple drug regimen remains popular, corticosteroids, calcineurin inhibitor (cyclosporine or tacrolimus), and an antiproliferative agent (azathioprine or mycophenolate mofetil). Endomyocardial biopsy and coronary angiography are performed at regular inter-vals to monitor rejection. The field of pediatric heart transplan-tation has made huge strides since the days of “Baby Fae.”188,189Public Reporting and the STS Database in Congenital Heart SurgeryThere has been a recent


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Surgery_Schwartz. The field of pediatric heart transplan-tation has made huge strides since the days of “Baby Fae.”188,189Public Reporting and the STS Database in Congenital Heart SurgeryThere has been a recent impetus in the filed of congenital and pediatric cardiac surgery toward public reporting of out-comes. The advantages of this include promoting patient autonomy, shows a commitment to quality improvement, and also serves as a free marketing tool. The Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD), is the largest clinical database in the world for congenital and pedi-atric cardiac surgery. It was founded in 1994. It contains data of about 394,980 operations as of September 9, 2016.192 These data are the foundation for assessment of performance by benchmark and comparison of individual programmatic outcomes to national aggregate data, development and subsequent applica-tion of sophisticated risk adjustment models, quality improve-ment initiatives, research, voluntary


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Surgery_Schwartz. of individual programmatic outcomes to national aggregate data, development and subsequent applica-tion of sophisticated risk adjustment models, quality improve-ment initiatives, research, voluntary public reporting, development of reimbursement strategies, and governmental and regulatory collaborations.190 The database is currently in its 25th overall data harvest and records and represents data from 120 participants and 392 surgeons. Thus, this database has greater than 95% penetrance. STS CHSD public reporting started in January 2015, and participation is voluntary. Report-ing is restricted to the hospital level and involves a rolling 4-year analytic window of data. Public reporting is based on the STS CHSD Operative Mortality Risk Model. Developed in 2014, this risk model calculates the operative mortality rate of hospitals performing such surgery, adjusting for procedural and patient level factors. The overall mortality rate over a 4-year period and the operative mortality rate


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Surgery_Schwartz. the operative mortality rate of hospitals performing such surgery, adjusting for procedural and patient level factors. The overall mortality rate over a 4-year period and the operative mortality rate for each of the five STAT (Society of Thoracic Surgeons—European Association for Cardio-Thoracic Surgery) categories is reported. The STAT categories are a multi-institutional, validated complexity stratification tool. They range from a score of 1 to 5, and the risk of mortality increases with each category.190 In addition, the STS star rating system was introduced, and every institution is rated as one, two, or three stars. This system is based on the confidence limits of the O/E (observed to expected) overall mortality for the institu-tion (Fig. 20-70). One star equals higher than expected operative 9Rady Children’s Hospital San DiegoRady Children’s Hospital San Diego SurgeonsEnc Devaney, MDDaniel DiBardino, MDJohn Lambert, MDPeter Pastuszko, MDOverall Star RatingPopulation:


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Surgery_Schwartz. expected operative 9Rady Children’s Hospital San DiegoRady Children’s Hospital San Diego SurgeonsEnc Devaney, MDDaniel DiBardino, MDJohn Lambert, MDPeter Pastuszko, MDOverall Star RatingPopulation: Neonates,Infants, Children & AdultsOvarallSTAT Mortality Category 1STAT Mortality Category 2STAT Mortality Category 3STAT Mortality Category 4STAT Mortality Category 5#/Eligible28/11650/3067/3991/12817/2953/37Observed2.4%0.0%1.8%0.8%5.8%8.1%Expected3.0%0.6%1.5%2.1%6.7%14.7%OE (95% CI)0.79 (0.53, 1.14)0.00 (0.00, 2.16)1.20 (0.48, 2.45)0.37 (0.01, 2.05)0.87 (0.51, 1.36)0.55 (0.12, 1.49)Adj. Rate (95% CI)2.5 (1.6, 3.5)0.0 (0.0, 1.1)2.0 (0.8, 4.1)1.0 (0.0, 5.3)6.0 (3.5, 9.4)8.7 (1.8, 23.6)San DiegoCAWebsite: http://www.rchsd.org/programs-services/cardiologyOperative and Adjusted Operative Mortality, Last 4 Years (January 2012–December 2015)Figure 20-70. Program performance as currently reported by the STS-CHSD.Brunicardi_Ch20_p0751-p0800.indd 79222/02/19 2:57 PM 793CONGENITAL HEART


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Surgery_Schwartz. Last 4 Years (January 2012–December 2015)Figure 20-70. Program performance as currently reported by the STS-CHSD.Brunicardi_Ch20_p0751-p0800.indd 79222/02/19 2:57 PM 793CONGENITAL HEART DISEASECHAPTER 20mortality (the 95% confidence interval for their risk-adjusted O/E mortality ratio was entirely above the number 1), two stars equals the same as expected operative mortality (the 95% con-fidence interval for their risk-adjusted O/E mortality ratio over-lapped with the number 1), and three stars equals lower than expected operative mortality (the 95% confidence interval for their risk-adjusted O/E mortality ratio was entirely below the number 1). The Spring 2016 STS CHSD Feedback Report includes data from 117 participants in the STS-CHSD, including 14 one-star programs, 83 two-star programs, and 8 three-star programs. Twelve participants did not receive a star rating due to incomplete data.191 Public reporting increased from 23% to 57.6% (all three-star programs, 50 two-star and


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Surgery_Schwartz. and 8 three-star programs. Twelve participants did not receive a star rating due to incomplete data.191 Public reporting increased from 23% to 57.6% (all three-star programs, 50 two-star and three one-star programs). The online public reporting portal can be accessed at www.sts.org/congenital-public-reporting-module-search.There are several criticisms to the current methodology used for reporting. Important limitations of current publicly reported data (including the STS star rating system) will need to be addressed in future initiatives in order to completely engage parents of children with CHD and reassure providers that risk-adjustment models are optimized. There are four spe-cific areas that should be considered when making decisions how to improve this methodology: (a) While the mortality risk-adjustment model on which the star rating system is based is mature now, there are not comparable models that provide risk-adjusted morbidity (complication) rates. The assessment of the


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Surgery_Schwartz. risk-adjustment model on which the star rating system is based is mature now, there are not comparable models that provide risk-adjusted morbidity (complication) rates. The assessment of the quality of congenital heart disease care at different centers should include complication metrics and incorporate failure-to-rescue as an important discriminator; (b) the star rating system does not provide risk-adjusted outcomes for specific procedures or, more importantly, for specific diagnoses. This is mainly because of the exceptionally wide spectrum of diagnoses and procedures in pediatric cardiac surgery that preclude sufficiently large numbers in most procedure-specific categories; (c) the star rating system, although the “best” we have at present, may not be understood equally by all families. It will be critical to provide equivalent information to the large numbers of under-resourced and non–English-speaking families; (d) finally, the current adjusted mortality rate reported by the STS


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Surgery_Schwartz. It will be critical to provide equivalent information to the large numbers of under-resourced and non–English-speaking families; (d) finally, the current adjusted mortality rate reported by the STS is calculated from a statistical formula and refers to what the hospital’s mor-tality rate would be if the measured performance (in this case the mortality rate) were extrapolated to the overall case-mix or make-up of patients within the entire STS database. This is a critical point because a hospital’s case-mix is highly variable, and discrimination based on mortality is mostly related to out-comes of more complex procedures. In other words, if hospital A has excellent survival for less complex procedures and therefore performs very few highly complex procedures (i.e., choosing a case-mix consistent with its expertise), the application of an extrapolated mortality rate may not reflect the actual quality of care for that particular hospital. This issue is evident because the majority of


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Surgery_Schwartz. with its expertise), the application of an extrapolated mortality rate may not reflect the actual quality of care for that particular hospital. This issue is evident because the majority of experienced centers with arguably the highest complexity received a “middle star” rating of 2. This rating may reflect calibration issues with the current rating system, whereby centers are potentially penalized for high-complexity predominance.Fortunately, there are efforts to correct these deficien-cies. In 2016, the STS CHSD Task Force and STS Quality Measurement Task Force began to collaborate on an initiative to refine risk adjustment for chromosomal abnormalities, syn-dromes, and noncardiac congenital anatomic abnormalities and to then enhance the STS CHSD Mortality Risk Model with this additional information. Upon completion of this project, STS CHSD Task Force plans to collaborate with the STS Quality Measurement Task Force to study the relationship between vol-ume (programmatic volume and


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Surgery_Schwartz. information. Upon completion of this project, STS CHSD Task Force plans to collaborate with the STS Quality Measurement Task Force to study the relationship between vol-ume (programmatic volume and surgeon volume) and outcome using this enhanced STS CHSD Mortality Risk Model.192 Also, currently under development is a multidomain quality metric that incorporates mortality, morbidity, postoperative length of stay, and the occurrence of complications. As the largest con-genital and pediatric cardiac surgical clinical data registry in the world, containing data about nearly all pediatric cardiac operations performed in the United States, STS CHSD contains a truly representative sample of national aggregate data that is useful for multiple purposes.192Future DirectionsThe future of congenital heart surgery remains very bright and exciting. The development of novel technologies such as four-dimensional MRI flow studies (Fig. 20-71) and three-dimen-sional printing have offered this field


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Surgery_Schwartz. heart surgery remains very bright and exciting. The development of novel technologies such as four-dimensional MRI flow studies (Fig. 20-71) and three-dimen-sional printing have offered this field several new tools to help understand complex anatomy and pathophysiology. Three-dimensional printing of complex congenital heart defects has helped surgeons in preoperative planning by allowing transla-tion of two-dimensional cross-sectional imaging studies into a tangible and easily visualized model.193 The hollow nature of the human heart and the direct correlation of structure to disease in the congenital population allows this technology to be used in abundance in this field. Its utilization to train young surgeons is very appealing (Figs. 20-72 and 20-73).194,196 Current research in the field of genetics, device bioengineering and miniaturization, stem cell therapy, and fusion imaging technology is expected to further improve patient outcome.195,198 The improved outcomes and survival of


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Surgery_Schwartz. of genetics, device bioengineering and miniaturization, stem cell therapy, and fusion imaging technology is expected to further improve patient outcome.195,198 The improved outcomes and survival of these young and fragile patients with congeni-tal heart disease has led to the development of a complex new field termed adult congenital heart disease. The field of con-genital heart surgery is young and offers brilliant, motivated, and upcoming surgeons a very daunting challenge to better the future of these babies.Figure 20-71. 4D MRI flow study obtained in a complex single ventricle patient for the evaluation of persistent hypoxia.Brunicardi_Ch20_p0751-p0800.indd 79322/02/19 2:57 PM 794SPECIFIC CONSIDERATIONSPART IIFigure 20-72. 3D printed models of complex heart defects which were very helpful for preoperative surgical planning and patient education.ADEFBCMAPCAPulmonaryArteryAortaFigure 20-73. Example of Pre-Interventional Planning Using 3D Printed Models. Transthoracic


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Surgery_Schwartz. were very helpful for preoperative surgical planning and patient education.ADEFBCMAPCAPulmonaryArteryAortaFigure 20-73. Example of Pre-Interventional Planning Using 3D Printed Models. Transthoracic echocardiogram (A) confirms tetralogy of Fallot/pulmonary atresia/multiple aortopulmonary collateral arteries (MAPCAs) diagnosis. Three-dimensional (3D) reconstruction (B and C) illustrates spatial relationship of patient-specific geometry such as true pulmonary arteries (blue), aorta (red), and MAPCAs (green and yellow) for central aortopulmonary shunt placement and coil planning. Three-dimensional printing (D) provides absolute scaling for planning purposes, as well as patient/family education. Angiography (E and F) captured after central shunt and prior to placement of MAPCA embolization coils. (Reproduced with permission from Ryan JR, Moe TG, Richardson R, et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary


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Surgery_Schwartz. coils. (Reproduced with permission from Ryan JR, Moe TG, Richardson R, et al: A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals, JACC Cardiovasc Imaging. 2015 Jan;8(1):103-104.)Brunicardi_Ch20_p0751-p0800.indd 79422/02/19 2:57 PM 795CONGENITAL HEART DISEASECHAPTER 20REFERENCESEntries highlighted in bright blue are key references. 1. American Heart Association. About congenital heart defects. Available at: http://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/Congenital-Heart-Defects_UCM_001090_SubHomePage.jsp. Accessed May 18, 2018. 2. Congenital Heart Public Health Consortium. FAQ fact sheet. Available at: https://www.aap.org/en-us/Documents/chphc/chd_fact_sheet_long.pdf. Accessed May 18, 2018. 3. Society for Thoracic Surgeons. Congenital heart surgery pub-lic reporting. Available at: https://www.sts.org/congenital-public-reporting-module-search. Accessed May 18, 2018. 4. Kouchoukos NT,


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Surgery_Schwartz. for Thoracic Surgeons. Congenital heart surgery pub-lic reporting. Available at: https://www.sts.org/congenital-public-reporting-module-search. Accessed May 18, 2018. 4. Kouchoukos NT, Blackstone EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:716. 5. Kirklin JW, Pacifico AD, Kirklin JK. The surgical treat-ment of atrioventricular canal defects. In: Arciniegas E, ed. Pediatric Cardiac Surgery. Chicago: Yearbook Medical; 1985:2398. 6. Peterson GE, Brickner ME, Reimold SC. Transesophageal echocardiography: clinical indications and applications. Circulation. 2003;107:2398-2402. 7. Kouchoukos NT, Blackstone EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed.


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Surgery_Schwartz. EH, Doty DB, et al. Atrial septal defect and partial anomalous pulmonary venous connection. In: Kouchoukos NT, Blackstone EH, Doty DB, et al, eds. Kirklin/Barrat-Boyes Cardiac Surgery. 3rd ed. Philadelphia: Churchill Livingstone; 2003:740. 8. Reddy VM. Cardiac surgery for premature and low birth weight neonates. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2001;4:271-276. Congenital heart defects in low-birth-weight infants are typically managed with sup-portive therapy or palliative surgery, and definitive repair is delayed. This paper describes the outcomes in 116 neonates and infants under 2500 g who underwent complete repair of simple and complex cardiac defects using cardiopulmo-nary bypass. 9. Thompson JD, Abuwari EH, Watterson KG, et al. Surgi-cal and transcatheter (Amplatzer) closure of atrial septal defect: a prospective comparison of results and cost. Heart. 2002;87:466-469. 10. Du ZD, Hijazi ZM, Kleinman CS, et al. Comparison between transcatheter and surgical


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Surgery_Schwartz. closure of atrial septal defect: a prospective comparison of results and cost. Heart. 2002;87:466-469. 10. Du ZD, Hijazi ZM, Kleinman CS, et al. Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: results of a multicenter nonran-domized trial. J Am Coll Cardiol. 2002;39:1836-1844. 11. Kutty S, Hazeem AA, Brown K, et al. Long-term (5-to 20-year) outcomes after transcatheter or surgical treatment of hemodynamically significant isolated secundum atrial septal defect. Am J Cardiol. 2012;109:1348-1352. 12. Highes ML, Maskell G, Goh TH, Wilkinson JL. Prospective comparison of costs and short term health outcomes of surgi-cal versus device closure of atrial septal defect in children. Heart. 2002;88:67-70. 13. Murphy JG, Gersh BJ, McGoon MD, et al. Long-term out-come after surgical repair of isolated atrial septal defect. N Engl J Med. 1990;323:1645-1650. 14. Hanninen M, Kmet A, Taylor DA, et al. Atrial septal defect closure in the


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Surgery_Schwartz. MD, et al. Long-term out-come after surgical repair of isolated atrial septal defect. N Engl J Med. 1990;323:1645-1650. 14. Hanninen M, Kmet A, Taylor DA, et al. Atrial septal defect closure in the elderly is associated with excellent quality of life, functional improvement, and ventricular remodeling. Can J Cardiol. 2011;27:698-704. 15. Mascio CE, Pasquali SK, Jacobs JP, et al. Outcomes in adult congenital heart surgery: analysis of the Society of Tho-racic Surgeons (STS) Database. J Thorac Cardiovasc Surg. 2011;142:1090-1097. 16. Hopkins RA, Bert AA, Buchholz B, et al. Surgical patch closure of atrial septal defects. Ann Thorac Surg. 2004;77:2144-2150. 17. Liu G, Qiao Y, Zou C, et al. Totally thoracoscopic surgical treatment for atrial septal defect: mid-term follow-up results in 45 consecutive patients. Heart Lung Circ. 2012;S1443-S9506. 18. Argenziano M, Oz M, Kohmoto T, et al. Totally endoscopic atrial septal defect repair with robotic assistance. Circulation. 2003;108(suppl


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Surgery_Schwartz. consecutive patients. Heart Lung Circ. 2012;S1443-S9506. 18. Argenziano M, Oz M, Kohmoto T, et al. Totally endoscopic atrial septal defect repair with robotic assistance. Circulation. 2003;108(suppl II):II-191-II-194. 19. Luo W, Chang C, Chen S. Ministernotomy vs. full sternotomy in congenital heart defects: a prospective randomized study. Ann Thorac Surg. 2001;71(2):473-475. 20. Sebastian VA, Guleserian KJ, Leonard SR, Forbess JM. Min-isternotomy for repair of congenital cardiac disease. Interact Cardiovasc Thorac Surg. 2009;9(5):819-821. 21. Srivastava AR, Banerjee A, Tempe DK, et al. A comprehen-sive approach to fast tracking in cardiac surgery: ambula-tory low-risk open-heart surgery. Eur J Cardiothorac Surg. 2008;33:955-960. 22. King TD, Thompson SL, Steiner C, Mills NL. Secundum atrial septal defects: nonoperative closure during cardiac cath-eterization. JAMA. 1976;235(23):2506-2509. 23. Karamlou T, Diggs BS, McCrindle BW, Ungerleider RM, Welke KF. The rush to atrial septal


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Surgery_Schwartz. atrial septal defects: nonoperative closure during cardiac cath-eterization. JAMA. 1976;235(23):2506-2509. 23. Karamlou T, Diggs BS, McCrindle BW, Ungerleider RM, Welke KF. The rush to atrial septal defect closure: is the introduction of percutaneous closure driving utilization? Ann Thorac Surg. 2008;86:1584-1590. 24. Zeevi B, Keane JF, Castaneda AR, Perry SB, Lock JE. Neonatal critical valvular aortic stenosis. A comparison of surgical and balloon dilatation therapy. Circulation. 1989;80(4):831-839. 25. Alsoufi B, Karamlou T, McCrindle BW, Caldarone CA. Man-agement options in neonates and infants with critical left ven-tricular outflow tract obstruction. Eur J Cardiothorac Surg. 2007;31(6):1013-1021. 26. Hammon JW Jr, Lupinetti FM, Maples MD, et al. Predictors of operative mortality in critical aortic stenosis presenting in infancy. Ann Thorac Surg. 1988;45(5):537-540. 25. Hill GD, Ginde S, Rios R, Frommelt PC, Hill KD. Surgical valvotomy versus balloon valvuloplasty for congenital


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Surgery_Schwartz. aortic stenosis presenting in infancy. Ann Thorac Surg. 1988;45(5):537-540. 25. Hill GD, Ginde S, Rios R, Frommelt PC, Hill KD. Surgical valvotomy versus balloon valvuloplasty for congenital aor-tic valve stenosis: a systematic review and meta-analysis. J Am Heart Assoc. 2016;5(8):e003931. A review article and a meta-analysis comparing balloon valvuloplasty to surgi-cal aortic valvotomy. This article describes the outcomes and reintervention rates in both the groups. 26. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilatation of congenital aortic stenosis: predictors of success. J Am Coll Cardiol. 1996;27(5):1257-1263. 27. Ross DN. Replacement of aortic and mitral valves with a pul-monary autograft. Lancet. 1967;57(7523):956-958. 28. Jones TK, Lupinetti FM. Comparison of Ross procedures and aortic valve allografts in children. Ann Thorac Surg. 1998;66(6 suppl):S170-S173. 29. Karamlou T, Jang K, Williams WG, et al. Outcomes and associated risk


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Surgery_Schwartz. FM. Comparison of Ross procedures and aortic valve allografts in children. Ann Thorac Surg. 1998;66(6 suppl):S170-S173. 29. Karamlou T, Jang K, Williams WG, et al. Outcomes and associated risk factors for aortic valve replacement in 160 children: a competing risks analysis. Circulation. 2005;29(22):3462-3469. 30. Marasini M, Zannini L, Ussia GP, et al. Discrete subaor-tic stenosis: incidence, morphology, and surgical impact of associated subaortic anomalies. Ann Thorac Surg. 2003;75(6):1763-1768. 31. Karamlou T, Gurofsky R, Bojcevski A, et al. Prevalence and associated risk factors for intervention in 313 children with subaortic stenosis. Ann Thorac Surg. 2007;84:900-906. 32. Somerville J, Stone S, Ross D. Fate of patients with fixed subaortic stenosis after surgical removal. Br Heart J. 1980; 43(6):629-647. 33. Williams JCP, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation. 1961;24:1311-1318.Brunicardi_Ch20_p0751-p0800.indd 79522/02/19 2:57 PM 796SPECIFIC


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Surgery_Schwartz. J. 1980; 43(6):629-647. 33. Williams JCP, Barratt-Boyes BG, Lowe JB. Supravalvular aortic stenosis. Circulation. 1961;24:1311-1318.Brunicardi_Ch20_p0751-p0800.indd 79522/02/19 2:57 PM 796SPECIFIC CONSIDERATIONSPART II 34. Tworetzky W, Wilkins-Haug L, Jennings RW, et al. Balloon dilation of severe aortic stenosis in the fetus: potential for pre-vention of hypoplastic left heart syndrome: candidate selection, technique, and results of successful intervention. Circulation. 2004;110(15):2125-2131. 35. van Son JM, Danielson GK, Puga FJ, et al. Supravalvular aor-tic stenosis: long-term results of surgical treatment. J Thorac Cardiovasc Surg. 1994;107(1):103-114. 36. Sharma BK, Fujiwara H, Hallman GL, et al. Supravalvular aortic stenosis: a 29-year review of surgical experience. Ann Thorac Surg. 1991;51:1031-1039. 37. McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, Hanley FL. Issues and outcomes in the management of supravalvular aortic stenosis. Ann Thorac Surg. 2000;69(2):


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Surgery_Schwartz. Surg. 1991;51:1031-1039. 37. McElhinney DB, Petrossian E, Tworetzky W, Silverman NH, Hanley FL. Issues and outcomes in the management of supravalvular aortic stenosis. Ann Thorac Surg. 2000;69(2): 562-567. 38. Clyman RI, Mauray F, Roman C, Rudolph AM, Heymann MA. Circulating PGE2 concentration and patent ductus arteriosus in fetal and neonatal lambs. J Pediatr. 1982;97(3):455-463. 39. McMurphy DM, Heymann MA, Rudolph AM, Melmon KL. Developmental change in constriction of the ductus arteriosus: response to oxygen and vasoactive substances in the isolated duc-tus arteriosus of the fetal lamb. Pediatr Res. 1972;6(4):231-238. 40. Mitchell SC, Korones SB, Berendes HW. Congenital heart disease in 56,109 births. Incidence and natural history. Circu-lation. 1971;43(3):323-332. 40. Campbell M. Natural history of persistent ductus arteriosus. Br Heart J. 1968;30(1):4-13. 41. Itabashi K, Ohno T, Nishida H. Indomethacin responsive-ness of patent ductus arteriosus and renal abnormalities in


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Surgery_Schwartz. M. Natural history of persistent ductus arteriosus. Br Heart J. 1968;30(1):4-13. 41. Itabashi K, Ohno T, Nishida H. Indomethacin responsive-ness of patent ductus arteriosus and renal abnormalities in preterm infants treated with indomethacin. J Pediatr. 2003;143(2):203-207. 42. Rashkind WJ, Cuaso CC. Transcatheter closure of patent duc-tus arteriosus. Pediatr Cardiol. 1979;1(1):3-7. 43. Moore JW, Schneider DJ, Dimeglio D. The duct-occlud device: design, clinical results, and future directions. J Interv Cardiol. 2001;14(2):231-237. 44. Zahn EM, Peck D, Phillips A, et al. Transcatheter closure of patent ductus arteriosus in extremely premature newborns: early results and midterm follow-up. JACC Cardiovasc Interv. 2016;9(23):2429-2437. This article shows that percutaneous closure of PDAs even in extremely small babies is possible. 45. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilation of congenital aor-tic stenosis: predictors of success. J Am


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Surgery_Schwartz. in extremely small babies is possible. 45. Moore P, Egito E, Mowrey H, Perry SB, Lock JE, Keane JF. Midterm results of balloon dilation of congenital aor-tic stenosis: predictors of success. J Am Coll Cardiol. 1996;27(5):1257-1263. 46. Mavroudis C, Backer CL, Gevitz M. Forty-six years of pat-ent ductus arteriosus division at Children’s Memorial Hospital of Chicago. Standards for comparison. Ann Thorac Surg. 1994;220(3):402-409. 47. Elzenga NJ, Gittenberger-de Groot AC, Oppenheimer-Dekker A. Coarctation and other obstructive arch anoma-lies: their relationship to the ductus arteriosus. Int J Cardiol. 1986;13(3):289-308. 48. Locher JP, Kron IL. Coarctation of the aorta. In: Mavroudis C, Backer CL, eds. Pediatric Cardiac Surgery. St. Louis: Mosby; 1994:167. 49. Presbitero P, Demaie D, Villani M, et al. Long-term results (15–30 years) of surgical repair of coarctation. Br Heart J. 1987;57(5):462-467. 50. Cohen M, Fuster V, Steele PM, Driscoll D, McGoon DC. Coarctation of the aorta:


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