Independent Factors Associated With Mortality, Reintervention, and Achievement of Complete Repair in Children With Pulmonary Atresia With Ventricular Septal Defect

Abstract

Outcomes in children with PAVSD have improved over time, and are better in completely repaired cases. Bronchopulmonary arterial supply is an important determinant of mortality, achievement of definitive repair, and post-repair reoperation.

Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries

“…One aspect of this diversity is the variable anatomy of the central branch pulmonary arteries [2][3][4][5]. Approximately 80% of patients with PA/VSD/MAPCAs have central branch pulmonary arteries, whereas 20% of patients have a complete absence of intrapericardial pulmonary arteries [6][7][8][9][10][11]. The developmental factors resulting in the presence or absence of central pulmonary arteries in PA/VSD/MAPCAs are currently not well defined.…”

“…Several groups continue to advocate the use of either a central shunt or right ventricle to pulmonary artery conduit as the principal method to achieve "pulmonary artery rehabilitation" [12][13][14][15][16]. The central pulmonary arteries can also be utilized in conjunction with unifocalization of MAPCAs for repair of PA/VSD/MAPCAs [6][7][8][9][10][11][17][18][19]. This strategy provides a versatile approach to the management of PA/VSD/MAPCAs and has yielded excellent hemodynamic results [20].…”

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

“…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.…”

Radiofrequency perforation of the right ventricular outflow tract as a palliative strategy for pulmonary atresia with ventricular septal defect

“…The presence of aortopulmonary collaterals, delayed palliation, and earlier age at complete repair were associated with an increased risk of reintervention after repair. This would indicate that an early palliative intervention with a later complete repair may offer some therapeutic benefit [11].…”