Pulmonary Atresia, Ventricular Septal Defect and Major Aorto-Pulmonary Collateral Arteries

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.