Congenital aortic insufficiency with ascending aortic aneurysm: Dealing with a challenging case

Abstract Congenital aortic insufficiency is a challenging condition; without appropriate management from birth, it can lead to left ventricle dilation and heart failure. Aortic insufficiency due to leaflet agenesia is rarely described in literature. We report here a case of severe congenital aortic regurgitation that required surgical treatment until the boy reached five-year-old thanks to prenatal diagnosis and adequate medical care since birth. The patient underwent a mechanical Bentall procedure for associated dystrophic pulmonary valve with mild stenosis, ascending aortic aneurysm of 34 mm and anomalous position of the left coronary ostium. We safely performed a rare paediatric procedure with encouraging short-term results.