Individualized approach to the surgical treatment of tetralogy of Fallot with pulmonary atresia

Abstract

An individualized approach based on the morphology of the pulmonary arterial supply permits achievement of a high rate of complete intracardiac repairs, basing pulmonary arterial flow on the intrapericardial pulmonary arteries in the great majority of cases, and has a low rate of reoperation and mortality.

Results of Primary Repair Versus Shunt Palliation in Ductal Dependent Infants With Pulmonary Atresia and Ventricular Septal Defect

“…The 2 main surgical strategies include either a single stage approach with primary neonatal repair or a staged approach with initial palliation with a shunt followed by later repair [2][3][4]. Disadvantages of primary repair include the use of cardiopulmonary bypass in the neonatal period, diastolic dysfunction of the noncompliant right ventricle that is often worsened in the presence of small pulmonary arteries or pulmonary regurgitation, and finally the common need to use a small sized RV-PA conduit with the need for early reoperation for conduit change [2][3][4].…”

“…The 2 main surgical strategies include either a single stage approach with primary neonatal repair or a staged approach with initial palliation with a shunt followed by later repair [2][3][4]. Disadvantages of primary repair include the use of cardiopulmonary bypass in the neonatal period, diastolic dysfunction of the noncompliant right ventricle that is often worsened in the presence of small pulmonary arteries or pulmonary regurgitation, and finally the common need to use a small sized RV-PA conduit with the need for early reoperation for conduit change [2][3][4]. On the other hand, disadvantages of BTS include the hospital and interstage mortality due to shunt-related complications such as occlusion or systemic steal, in addition to the risk of distortion of the small pulmonary arteries and the obvious need for the second stage reoperation for complete repair [4][5][6][7][8].…”

“…While the intracardiac anatomy is generally constant, PAVSD is an extremely heterogeneous malformation due to great differences in the development of the pulmonary circulation [1][2][3]. The source of pulmonary blood flow in PAVSD varies and ranges from a simple form associated with well developed intra-pericardial pulmonary arteries, to the other extreme arrangement in which there is absence of the intra-pericardial pulmonary arteries with pulmonary arterial supply derived exclusively from multiple aortopulmonary collateral arteries (MAPCAs) [1][2][3].…”

“…Surgical management of neonates born with the simple form of PAVSD is naturally less complicated than that of those born with MAPCAs [2][3][4]. Nonetheless, patients born with the simple form of PAVSD have ductaldependent pulmonary blood flow and require early neonatal palliation or repair.…”

“…The 2 surgical treatment strategies that are applied in ductal-dependent PAVSD patients include either a single stage strategy of primary neonatal biventricular repair (BVR) or a staged strategy of initial palliation with a modified Blalock-Taussig shunt (BTS) followed by a second stage repair. Each of those two strategies is associated with specific advantages and drawbacks [2][3][4][5][6][7][8]. We aim in the current series to report current era results of surgical treatment of neonates born with ductal-dependent PAVSD at our institution; to examine risk factors affecting early and late outcomes and to compare early mortality, late survival, and right ventricular outflow tract (RVOT) reoperation risk between the 2 management strategies.…”