Pulmonary atresia with intact ventricular septum: A brief overview of management strategies and rationale

Abstract 

Pulmonary atresia with intact ventricular septum is a rare congenital cardiac anomaly characterized by complete obstruction of outflow from the right ventricle and pulmonary blood flow that is completely ductal dependent. Appropriate therapeutic decision making early in the neonatal period is crucial to immediate survival. Ultimately, definitive palliation or correction is very much dependent upon the adequacy of right-sided cardiac structures. This article will review management strategies designed to optimize both immediate and longer term survival.

Keywords: Pulmonary atresia, Intact ventricular septum, Ductal dependent pulmonary blood flow, Hypoplastic right ventricle, Coronary sinusoids