Course and Outcome of Fetuses Suspected of Having Coarctation of the Aorta during Gestation

Zusammenfassung

Ziel: Bericht über den Schwangerschaftsverlauf und -ausgang bei Feten mit pränatalem Verdacht auf Aortenisthmusstenose. Material und Methoden: Retrospektive Observationsstudie über den Zeitraum von 1993 – 2005 an zwei tertiären Zentren. Ergebnisse: 96 Feten wurden untersucht, davon waren 52 Lebendgeburten. Von 52 Lebendgeburten hatten 34 eine Aortenisthmusstenose, die postnatal durch Echokardiografie bestätigt wurde (65,4 %), 13 Neugeborene hatten zusätzlich, pränatal diagnostizierte, kardiale Anomalien (Ventrikelseptumdefekt, Vorhofseptumdefekt Typ I, Aorten- und Pulmonalstenose, persistierende linke obere Hohlvene) und drei Neugeborene hatten eine Linksherzhypoplasie. Drei Neugeborene wiesen zusätzliche extrakardiale Anomalien auf, die pränatal diagnostiziert wurden (Hypospadie, Mikroophtalmie, Omphalozele, Zwerchfellhernie). 22 Neugeborene wurden operiert, 19 innerhalb der ersten zehn Lebenstage. Ein Neugeborenes entwickelte erst am 14. Lebenstag klinische Zeichen einer Aortenisthmusstenose. Die operative Mortalität betrug 3 / 22 (13,6 %) und diese war durch Frühgeburtlichkeit mitbeeinflusst. Die Überlebensrate auf der Basis intention-to-treat war 29 / 34 Neugeborenen mit bestätigter Aortenisthmusstenose (85,3 %). Zusammenfassung: Die Diagnose einer Aortenisthmusstenose beim Feten ist mit falsch positiven Befunden behaftet. Die Möglichkeit der intrauterinen Progression einer Hypoplasie der Linksherzstrukturen im Falle einer fetalen Aortenisthmusstenose mit der Entwicklung eines Erscheinungsbilds eines hypoplastischen Linksherzens sollte bedacht werden, und aus diesem Grund sind serielle antenatale Echokardiografien zu empfehlen.

Abstract

Purpose: To report the course and outcome of a group of fetuses with prenatal suspicion of coarctation of the aorta. Materials and Methods: Retrospective observational study in two tertiary fetal cardiology centers between 1993 – 2005. Results: 96 fetuses of whom 52 infants were born alive were studied. Of the 52 liveborn infants, 34 had coarctation of the aorta (65.4 %), thirteen had prenatally diagnosed additional cardiac anomalies (VSD, ASD, aortic and pulmonary stenosis, persistent left superior vena cava) and three were managed as having hypoplastic left heart syndrome. Three neonates had additional extracardiac malformations diagnosed prenatally. 22 neonates underwent surgery, nineteen within the first ten days of life. One neonate only developed clinical signs of coarctation on the fourteenth day of life. The early surgical mortality was three of 22 (13.6 %). The mortality was influenced by prematurity. The survival rate on the basis of intention-to-treat was twenty-nine of 34 neonates with confirmed coarctation (85.3 %). Conclusion: Coarctation of aorta during fetal life continues to be a difficult diagnosis. The potential of progressive hypoplasia of left heart structures during gestation in the case of fetal aortic isthmus stenosis with the development of a hypoplastic left heart should be kept in mind and therefore sequential echo-cardiography is recommended during gestation.

fetus - ultrasound - echocardiography

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