Congenital valvar aortic stenosis and abnormal origin of the right coronary artery: rare combination with important clinical implications.

Congenital valvar aortic stenosis in association with an abnormal coronary artery origin and distribution has not been previously reported. The right coronary artery arose in the left ventricular (LV) outflow tract and supplied much of the LV myocardium. Aortic valvotomy with standard antegrade cardioplegia resulted in infarction of the left ventricle. Aggressive attempts to ascertain the origin of the coronary arteries in right dominant coronary systems must be performed before any intervention in this group of patients.