Prenatal assessment of ventriculocoronary connections and ventricular endocardial fibroelastosis in hypoplastic left heart

Ultraschall Med. 2014 Aug;35(4):357-63. doi: 10.1055/s-0034-1366361. Epub 2014 May 22.


Objective: The outlook for newborns with hypoplastic left heart (HLH) has substantially improved over the last decade. However, differences in outcome among various anatomical subgroups have been described. We aimed to describe the incidence of ventriculocoronary communications and endocardial fibroelastosis in HLH and the possible implication on hospital survival (30 d).

Methods: We retrospectively reviewed our medical records, still frames and video loops of 72 fetuses with HLH and critical aortic valve stenosis and evolving HLH from 2008 - 2013. The presence of VCAC and EFE were systematically assessed. Outcome parameters were incidence of VCAC and EFE among different anatomical subgroups of HLH and hospital survival (30 d).

Results: 72 fetuses were included in this series. The incidence of VCAC was 11.1 % (8 cases) and EFE occurred in 33.3 % (24 cases). 5 fetuses with VCAC occurred in the subgroup of mitral valve stenosis/aortic valve atresia (MS/AA, 62.5 %) and 2 fetuses with VCAC occurred in the group of mitral atresia/aortic valve atresia (MA/AA, 25 %). Further classification was not possible in one case with VCAC (12.5 %). EFE predominantly occurred in the subgroup of MS/AA, MA/AA and in those cases with aortic valve stenosis and evolving HLH. The overall hospital survival on an intention-to-treat basis was 91.2 % (52/57 newborns). Hospital survival was 91 % for the subgroup of cases with MS/AA and for all other anatomical subgroups.

Conclusion: The presence of VCAC in HLH can be diagnosed by fetal echocardiography predominantly occurring in cases with obstructed outflow and to some extent patent mitral valve. EFE is a frequent coexisting finding. Hospital survival was comparable among different anatomical subgroups and in cases with VCAC. The presence of VCAC in HLH did not limit the results of surgical palliation within the observation period of 30 days.

MeSH terms

  • Aortic Valve / abnormalities
  • Aortic Valve / diagnostic imaging
  • Coronary Vessel Anomalies / diagnostic imaging*
  • Echocardiography*
  • Echocardiography, Doppler, Color*
  • Endocardial Fibroelastosis / diagnostic imaging*
  • Endocardial Fibroelastosis / mortality
  • Female
  • Heart Ventricles / abnormalities*
  • Heart Ventricles / diagnostic imaging*
  • Hospital Mortality
  • Humans
  • Hypoplastic Left Heart Syndrome / diagnostic imaging*
  • Hypoplastic Left Heart Syndrome / mortality
  • Infant, Newborn
  • Pregnancy
  • Prognosis
  • Survival Rate
  • Ultrasonography, Prenatal*