Tetralogy of Fallot with Pulmonary Atresia

A subset of patients with Tetralogy of Fallot (TOFTetralogy of Fallot (TOF)) have associated pulmonary atresiaPulmonary atresia also referred to as ‘Pulmonary AtresiaPulmonary atresia with VSDVentricular septal defect (VSD)’. The branch pulmonary arteries are usually confluent and normally developed, fed by the arterial duct, and the intracardiac anatomy is that of TOFTetralogy of Fallot (TOF) with a well-developed right ventricleVentricles, large VSDVentricular septal defect (VSD) and overriding aorta. By definition, these are duct dependent at birth and management is either primary repair or initial palliation with a ductal stent or a surgical systemic to pulmonary artery shunt (BTT most commonly). Definitive repair, whether as a primary or staged procedure, involves VSDVentricular septal defect (VSD) closure with either an RVBorderline right ventricle (RV)-PA conduit or a trans-annular patch if there is membranous atresia. Mortality is low and reoperation is usually for conduit replacement.