Tetralogy of Fallot with Pulmonary Atresia

Tetralogy of Fallot with pulmonary atresia, one of the sets of lesions making up pulmonary atresia with ventricular septal defect, is the extreme end of the spectrum of clinical manifestation of tetralogy of Fallot. The lesion is classified according to the complexity of pulmonary blood flow, which is supplied either by intrapericardial pulmonary arteries or major systemic-to-pulmonary collateral arteries. When there are confluent intrapericardial pulmonary arteries that are normal in size, surgical repair is relatively straightforward. However, when the intrapericardial pulmonary arteries are nonconfluent and diminutive or absent, the systemic-to-pulmonary collateral arteries become more important sources of pulmonary blood flow and present challenges to surgical repair. Each patient becomes unique in terms of the size, origin, and distribution of the intrapericardial pulmonary arteries versus the collateral arteries. The outcomes of surgical repair in the early surgical era were dismal, but several institutions have evolved algorithms based on their own experiences. An algorithm-based approach, with an individualized surgical repair for each patient, may lead to improvement in surgical outcomes and survival.