Management strategy in neonates presenting with pulmonary atresia with intact ventricular septum

The management of neonates with pulmonary atresia and intact ventricular septum presents a challenging problem mainly because of the profound heterogeneity of the morphology of the right ventricle. This article outlines a different approach to the management based on the presence or absence of a well-formed infundibulum (outflow) of the right ventricle. In most of the children with a well-formed infundibulum, successful biventricular repair can be achieved, after promoting the growth of the right ventricle by promoting ategrade flow through it. In patients with poorly formed or an absent infundibulum, on the other hand, a Fontan procedure is an excellent solution.