Consequences of a hypertensive right ventricle on left ventricular performance of patients with pulmonary atresia and intact ventricular septum after right heart bypass surgery

A hypertensive right ventricle adversely affects left ventricular function in patients who are Fontan candidates with pulmonary atresia with intact ventricular septum (PAIVS). Decompression of a hypertensive right ventricle should improve left ventricular performance. Recently, a right ventricular outflow tract reconstruction, the closed Brock procedure, and/or percutaneous balloon pulmonary valvuloplasty were performed to decompress the hypertensive right ventricle before and during the bidirectional Glenn procedure in Fontan-candidate patients with PAIVS in our hospital. This strategy significantly improved left ventricular performance (contractility and ventricular efficiency) after the bidirectional Glenn procedure and a staged total cavopulmonary connection.