New Aspects in the Diagnosis and Therapy of Fetal Hypoplastic Left Heart Syndrome

Abstract

Fetal hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease with a lethal prognosis without postnatal therapeutic intervention or surgery. The aim of this article is to give a brief overview of new findings in the field of prenatal diagnosis and the therapy of HLHS. As cardiac output in HLHS children depends on the right ventricle (RV), prenatal assessment of fetal RV function is of interest to predict poor functional RV status before the RV becomes the systemic ventricle. Prenatal cardiac interventions such as fetal aortic valvuloplasty and non-invasive procedures such as maternal hyperoxygenation seem to be promising treatment options but will need to be evaluated with regard to long-term outcomes. Novel approaches such as stem cell therapy or neuroprotection provide important clues about the complexity of the disease. New aspects in diagnostics and therapy of HLHS show the potential of a targeted prenatal treatment planning. This could be used to optimize parental counseling as well as pre- and postnatal management of affected children.

Zusammenfassung

Das hypoplastische Linksherzsyndrom (HLHS) ist eine schwerwiegende angeborene Herzerkrankung. Ohne postnatale therapeutische Intervention oder chirurgischen Eingriff ist die Prognose fatal. Dieser Artikel gibt einen kurzen Überblick über neue Erkennntnisse für die pränatale Diagnostik und Therapie von Feten mit HLHS. Da die Herzleistung bei Kindern mit HLHS von der rechten Herzkammer (RV) abhängt, liefert die vorgeburtliche Evaluierung der fetalen RV-Funktion wichtige Informationen zur Prognose einer RV-Dysfunktion, bevor der RV zum Systemventrikel wird. Pränatale Eingriffe am Herzen, wie z. B. die fetale Aorten-Valvuloplastie, sowie nicht-invasive Eingriffe, wie die mütterliche Hyperoxygenierung, sind vielversprechende Behandlungsoptionen. Allerdings müssen diese Ansätze im Hinblick auf die Langzeitergebnisse noch weiter evaluiert werden. Neue Ansätze wie die Stammzelltherapie oder Neuroprotektion liefern wichtige Hinweise für die Komplexität der Erkrankung. Neue Aspekte bei der Diagnostik und Therapie von HLHS weisen auf das Potenzial einer zielgerichteten Planung der vor- und nachgeburtlichen Behandlung hin. Dies könnte zur Optimierung der Elternberatung und des prä- und postnatalen Managements betroffener Kinder führen.

Key words

fetal hypoplastic left heart syndrome - fetal cardiac function - fetal cardiac interventions

Schlüsselwörter

fetales hypoplastisches Linksherzsyndrom - fetale Herzfunktion - therapeutische Eingriffe am fetalen Herzen

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