EP04.20: Prenatal subtyping of ventriculocoronary connections in hypoplastic left heart syndrome: review of the literature and a case report

Abstract

Ventriculocoronary connections (VCC) are a frequent phenomenon in hypoplastic left-heart syndrome (HLHS). They are especially common in fetuses manifesting HLHS with mitral stenosis und aortic atresia (MSAA subtype). According to Blake et al, there are three subtypes of ventriculocoronary connections (aterioluminal, arteriosinusoidal and ateriocapillary). The clinical importance of these VCC is still undetermined. While VCC have previously been considered to be prognostic for a poor outcome after surgical stage I palliation, some evidence suggests that they are not associated with a higher mortality. Here we will present the case of a child with HLHS of the MSAA subtype with several venticulocoronary connections who was diagnosed prenatally and treated postnatally in our centre. The child was delivered at 37+3 weeks' gestation due to a restriction in the foramen ovale. The child adapted well postnatally presenting a retrograde flow through the VCC into the ascending aorta. This rare condition caused high O2 saturations and contributed to the satisfying clinical performance of the newborn. We will discuss the details of this case and review the literature due to the importance of VVC subtyping.