Absent pulmonary valve syndrome with aortic regurgitation in a 50-year-old man

A 50-year-old man was diagnosed as having absent pulmonary valve syndrome with aneurysmal dilatation of the pulmonary arteries. He also had a large subaortic ventricular septal defect and severe aortic regurgitation. He underwent successful intracardiac repair and aortic valve replacement. This case is presented in view of its rarity and controversies in the surgical management.