Pulmonary atresia, ventricular septal defect and major aorto-pulmonary collateral arteries

Abstract

The term pulmonary atresia and ventricular septal defect (VSD) covers a spectrum of conditions with various degrees of severity. The terminology of pulmonary atresia, VSD and major aortopulmonary collateral arteries (MAPCAs) is usually restricted to patients with diminutive central pulmonary arteries whose pulmonary circulation is dependent from systemic-pulmonary collateral arteries. It therefore excludes patients with ductus-dependent pulmonary circulation. The natural history of the condition is severe. In the 90s, only a fifth of the patients born with pulmonary atresia, VSD and MAPCAs survived to 30 years of age. Significant progress has been made in the management of patients with pulmonary atresia, VSD and MAPCAs. Our understanding of the variations in pulmonary vasculature has evolved, although there remains debate about the best methods of classification. There is an ongoing debate on optimal surgical strategies between surgical rehabilitation of the native hypoplastic PA that our group favors and unifocalisation of MAPCAs early in infancy. There continues to be debate on the most effective strategy to provide long-term survival in this patient group.