Aortic atresia with interrupted aortic arch: a combination incompatible with life?: Figure 1:

Abstract

Aortic valve atresia is a common feature of hypoplastic left heart syndrome, which consists of various degrees of underdevelopment of the left ventricle aorta complex; nevertheless it can also be associated with a large ventricular septal defect (VSD) and a rather developed left ventricle. Atresia of the aortic valve prevents antegrade blood flow from the left ventricle to the ascending aorta, so perfusion of the coronary arteries, ascending aorta and aortic arch is provided by the ductus arteriosus in a retrograde fashion. Interrupted aortic arch (IAA) is quite often associated with aortic or subaortic stenosis; however, very few cases of IAA have been reported in association with aortic valve atresia. Aortic valve atresia with IAA is an extremely rare association which would be incompatible with life, unless blood flow is provided to the ascending aorta and coronary arteries from (i) the ductus/descending aorta through ‘collaterals’ [1], or (ii) from the pulmonary trunk and pulmonary arteries through an aorto-pulmonary window [2] or bilateral ductus [3]. In the absence of a direct connection, blood flow to the coronary arteries is exclusively provided by the Circle of Willis through both carotid arteries, in a reverse