P03.09: Ventriculocoronary connections in fetuses with pulmonary or aortic atresia with intact ventricular septum

Abstract

Objective: Ventriculocoronary connections (VCC) can be prenatally visualized in fetuses with pulmonary (PA) and aortic atresia (AA) and intact ventricular septum (IVS). After prenatal diagnosis an improvement of outcome may be achieved by modification of perinatal and operative management. Method: We identified retrospectively 13 fetuses with prenatally diagnosed VCC, 10 with PA-IVS and 3 with AA-IVS in our database between 2003 and 2008. Prenatal findings, perinatal management and outcome were analysed. Results: In fetuses with PA-IVS prenatal diagnosis of VCC was correlated with small right ventricle and tricuspid annulus and absence of tricuspid regurgitation. One fetus had an additional tricuspid atresia resulting into a back-and-forth blood flow via VCC between the hypoplastic right ventricle (RV) and thickened coronary arteries. Three fetuses with AA-IVS showed a hypoplastic left ventricle with endocardial fibroelastosis and two of those had a restrictive foramen ovale. Of the ten fetuses with PA-IVS, termination of pregnancy was performed in 1 fetus; 1 severely growth restricted fetus died as consequence of uteroplacental dysfunction and acute CMV-infection, 1 fetus died postoperatively during surgical commissurotomy: 1 fetus died days after BT anastomosis; four fetuses had single ventricle palliation and two fetuses a biventricular repair. Norwood procedure was successfully performed in the three fetuses with severe obstruction of the left ventricular outflow tract. Conclusions: Prenatal diagnosis of VCC has important implications for prenatal counselling. A special perinatal approach is required in fetuses with PA-IVS because hypoplastic RV and RV-dependent coronary circulation do not allow opening of the obstructed pulmonary valve resulting in a single ventricle repair. In fetuses with hypoplastic left heart the presence of VCC seems not to worsen the outcome of Norwood procedure.