Hypoplastic Left Heart Syndrome and Ventriculocoronary Connections

Abstract

Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect. There are variants of HLHS, consisting of permutations of stenosis or atresia of the mitral and aortic valves. A notable phenomenon described in all HLHS subtypes is ventriculocoronary connections (VCCs). The significance of VCCs remains undetermined. Some studies have demonstrated higher mortality associated with VCCs, while others have shown no difference in mortality compared to other HLHS variants. All variants of HLHS are associated with myocardial disarray and endomyocardial fibroelastosis, although recent evidence demonstrates a higher association of those findings with the mitral stenosis–aortic atresia (MS-AA) variant. And VCCs may have a higher incidence in MS-AA as they constitute the only egress of blood from the LV. These associations may play a role in the survival of patients with MS-AA. We present a case of HLHS with MS-AA and significant VCCs. Figure 1 depicts the diminutive LV, no outflow, and opacification of the entire coronary system. Coronary opacification can only be achieved via VCCs, given the aortic valve atresia. Figure 2 demonstrates the severely hypoplastic ascending aorta. The aorta is similar in size to the 3F catheter and the coronaries do not opacify.