Pulmonary Atresia with Intact Ventricular Septum

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. In addition, early data on fetal intervention reveals a potential to promote the growth of the right-sided structures. Postnatally, our understanding of the anatomical and physiological factors has resulted in a tailored approach to patient care, with improved survival. Angiographic assessment of the coronary arteries has long been a key diagnostic tool, and transcatheter interventions are becoming more refined, with resulting improvement in outcomes.