ABSTRACT
Pulmonary atresia (PA) is rare and accounts for about 1% of all congenital heart diseases (CHD). This anomaly is a heterogeneous CHD and encompasses a broad range of anomalies, whose common feature is absence of continuity of blood flow from the right ventricle to the pulmonary vascular bed, a subaortic ventricular septal defect and a biventricular heart. In terms of prognosis, the least favorable condition is PA, whereby pulmonary artery branches are completely absent, with the pulmonary vascular bed supplied solely by major aortopulmonary collateral arteries (MAPCA). A critical factor regarding prognosis is the degree of development of pulmonary artery branches. The native pulmonary arteries can be normal, but they are frequently hypoplastic and abnormal in structure. In the presence of hypoplastic or absent pulmonary artery branches, the pulmonary vascular bed is additionally supplied by MAPCA.
