Prenatal Assessment of Ventriculocoronary Connections and Ventricular Endocardial Fibroelastosis in Hypoplastic Left Heart

Abstract

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.

Zusammenfassung

Hintergrund: Die Prognose für Neugeborene mit hypoplastischem Linksherz (HLH) nach chirurgischer Palliation hat sich im letzten Jahrzehnt verbessert. Für anatomische Subgruppen des HLH werden unterschiedliche Mortalitätszahlen beschrieben. Ziel der Arbeit war die Analyse des outcomes (30 Tage) in Fällen mit HLH und ventrikulokoronarer Fistel (VCAC) und mit Endokardfibroelastose (EFE).

Methoden: Es handelt sich um eine retrospektive Datenbankanalyse der Jahre 2008 – 2013 mit HLH und mit kritischer Aortenklappenstenose (AS), die sich im Verlauf zu einem HLH entwickelt haben.

Ergebnisse: 72 Feten wurden eingeschlossen. Die Inzidenz der VCAC betrug 11,8 % (8 Fälle) und der EFE 33,3 % (24 Fälle). Fünf Fälle mit VCAC wiesen eine Mitralklappenstenose/Aortenklappenatresie (MS/AA, 62,5 %) und zwei Feten wiesen eine Mitralklappenatresie/Aortenklappenatresie (MA/AA, 25 %) auf. In einem Fall mit VCAC konnte keine Klassifikation erfolgen. EFE wurde vorwiegend in Fällen mit MS/AA, MA/AA und in Fällen mit AS und sich entwickelndem HLH diagnostiziert. Die 30 Tage Überlebensrate auf intention-to-treat Basis war 91,2 % (52/57 Neugeborenen, 2/59 mit compassionate care ohne Behandlungswunsch). Die Überlebensrate zwischen den anatomischen Subgruppen mit/ohne EFE und/oder VCAC war vergleichbar (91 %).

Folgerung: Die pränatale Diagnose einer VCAC bei HLH ist möglich. VCAC kommen vorwiegend in der anatomischen Subgruppe mit MS/AA vor. EFE ist eine häufige Begleiterscheinung. Die Überlebensrate zwischen den anatomischen Subgruppen war vergleichbar und dies sollte in der pränatalen Beratung Berücksichtigung finden.

Key words

prenatal diagnosis - fetal echocardiography - hypoplastic left heart

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