A rare case of biventricular outflow obstruction with intact ventricular septum.

We report a case of pulmonary atresia with intact ventricular septum and aortic valve stenosis in a neonate. This combination is extremely rare. Cardiac catheterization was performed at 2 days of age, which confirmed the prenatal echocardiographic diagnosis and identified associated moderate aortic valve stenosis. Soon after the examination, an urgent surgical intervention was required due to uncontrollable circulatory instability. The baby underwent a successful Fontan-type repair achieved by surgical management only.