Tetralogy of fallot, pulmonary atresia, and diminutive pulmonary arteries

Abstract

The surgical management of classical tetralogy of Fallot (TOF), with right ventricular-to-pulmonary artery continuity and pulmonic stenosis, has been well established and standardized, and both early and late results are good, even in critically ill infants. In other patients with pulmonary atresia with ventricular septal defect, however, significant additional therapeutic challenges are seen, primarily because of the wide range of origin, size, and distribution of pulmonary blood supply found in this entity. The most fortunate subset of patients with tetralogy of Fallot with pulmonary atresia (TOFPA) are those with valvar pulmonary atresia, a ductus-dependent pulmonary arterial circulation and essentially normal pulmonary arteries. At the other end of anatomic complexity are those patients with TOF-PA without a patent ductus arteriosus (PDA) and with diminutive or absent central pulmonary arteries. In these patients pulmonary blood flow is supplied exclusively by aortopulmonary collateral arteries (APCAs). Because of the significant variations in pulmonary arterial structure and blood flow, no single effective treatment plan is applicable to this diverse group of patients. Generally,