Tetralogy of Fallot with Pulmonary Atresia

Abstract

Tetralogy of Fallot with Pulmonary Atresia (TOF-PA) accounts for 1.5–3.4% of all forms of congenital heart disease and for 20% of all forms of TOF [1]. The Baltimore Washington Infant study reported an incidence of 0.07 per 1,000 live births for TOF-PA. TOF-PA is slightly more prevalent in males than in females. The intra-cardiac anatomy of TOF-PA has all the features of classic Tetralogy of Fallot: ventricular septal defect, overriding of the aorta, right ventricular outflow obstruction, and right ventricular hypertrophy. The difference is in the membranous or complete atresia of the pulmonary valve, and extreme variability of the architecture of the main and distal pulmonary arteries [Fig. 21.1]. The central pulmonary arteries can be of good size, variably hypoplastic, discontinuous, or even absent. Blood flow to the pulmonary vasculature may be provided by a persistent ductus arteriosus (PDA), major aorto-pulmonary collaterals (MAPCAs), or both.