Outcome of patients with hypoplastic left heart (HLH) and aortic stenosis (AS) with prenatally diagnosed ventriculocoronary arterial fistula (VAF) and endocardial fibroelastosis (EFE)

Objective: The aim of this retrospective analysis is to compare the 30-day-survival for newborns with a prenatal diagnosis of HLH or AS with VAF to those of patients without VAF.

Methods: Database was reviewed for cases of HLH or AS between 2008 to 2013 at Justus-Liebig University of Gießen Germany.

Results: 72 fetuses were analysed. 8/72 fetuses (11, 1%) presented with VAF. Six had VAF and EFE, two had VAF without EFE. Twenty-four had EFE without VAF and 40 fetuses had neither VAF nor EFE. There is one ongoing pregnancy with VAF and EFE. The other five newborns survived with Pulmonary-Artery-Banding (PA-Banding) and stenting of Ductus arteriosus (PDA-Stent) as intervention (Gießen-Procedure). 1/5 newborns had HLH with AA/MA, three had MS/AA and one MA, AS and VSD. Two patients with VAF without EFE survived. One had AA/MA and survived after Gießen Procedure, the other with unknown type of HLHS had Norwood- Operation.

15/24 fetuses with EFE without VAF survived. Ten newborns had Norwood-Procedure, four had Gießen Procedure. One newborn with AS had valvuloplasty. 2/24 newborns died within 30 days pp. 3/20 patients are still pregnant, four termination of pregnancy (TOPs) occurred.

5/40 with HLH were lost for follow up. 30/35 evaluable fetuses with HLH with neither VAF nor EFE survived (85, 7%). Eight of them had Norwood-Procedure, 17 had Gießen-Procedure, 4 had other operation and one with borderline left ventricle needed no surgical intervention. 5/35 newborns died, two of these patients had compassionate care.

Conclusions: 30 day survival in case of HLH with VAF and EFE is comparable to HLH without EFE and/or VAF. Long-term data is needed to precise outcome data for parental counseling.