ABSTRACT
Pulmonary atresia with ventricular septal defect (PA-VSD) is an uncommon complex congenital cardiac lesion in which no luminal continuity exists between the right ventricle (RV) and the pulmonary arteries. PA-VSD represents the most severe end of the spectrum of Tetralogy of Fallot and is often referred to as “Tetralogy of Fallot with pulmonary atresia.” The intracardiac anatomy is consistent and similar to that of Tetralogy of Fallot with an anteriorly malaligned VSD, a well-developed left ventricle (LV), and a variable degree of RV infundibular hypoplasia. The extracardiac pulmonary blood supply, however, has great morphological variability as a hallmark characteristic.
