PRIMARY INSTRUMENTAL EXAMINATION IN THE ANOMALY OF PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM

Abstract

Pulmonary atresia with intact ventricular septum is a cyanotic congenital heart disease characterized by complete obstruction of the right ventricular outflow tract, right ventricular hypoplasia, myocardial hypertrophy, and deranged coronary arteries. Historically, treatment strategies have resulted in high mortality rates. Advances in fetal imaging have led to improvements in our ability to prognosticate which patients are likely to have functionally single versus biventricular therapeutic pathways.