best management in isolated right ventricular hypoplasia with septal defects in adults

Hypoplastic right ventricle is a rare congenital disease usually associated with pulmonary atresia or tricuspid atresia. isolated right ventricular hypoplasia is a rare anomaly without important valvular abnormalities. it is associated with interatrial septal defects leading to the right-to-left shunting of blood. patients with isolated right ventricular hypoplasia usually have different and variable courses. in some patients, it is recognized in the perinatal period and necessitates prompt intervention; nonetheless, there are some reports of this anomaly in old age with no significant symptoms. in this report, we describe the clinical data and management of 6 adult cases with isolated right ventricular hypoplasia treated medically or surgically based on the severity of the disease and symptoms and then offer an indepth discussion regarding this rare anomaly.