Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV)

Abstract

Purpose To assess the spectrum of associated cardiac anomalies, the intrauterine course, and postnatal outcome of fetuses with double inlet ventricle (DIV).

Methods Retrospective analysis of prenatal ultrasound of 35 patients with DIV diagnosed between 2003 and 2021 in two tertiary referral centers in Germany. All fetuses underwent fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports.

Results 33 cases of DIV were correctly diagnosed prenatally. 24 fetuses (72.7%) had a double inlet ventricle with dominant left (DILV), 7 (21.2%) with dominant right ventricular morphology (DIRV), and 2 cases (6%) with indeterminate morphology (DIIV). 4 (16.6%) were Holmes hearts. 5 of the 7 fetuses (71.4%) with DIRV had a double outlet right ventricle (DORV). Malposition of the great arteries was present in 84.8%. Chromosomal abnormalities were absent. Termination of pregnancy was performed in 8 cases (24.2%). 24 fetuses (72.7%) were live-born. 5 (20.8%) were female and 19 (79.2%) were male. The median gestational age at birth was 38+2.5 weeks. All but one child received univentricular palliation. The median follow-up time was 5.83 years with an adjusted survival rate of 91.6% (22 of 24 live-born children). There was one case of Fontan failure at 15.7 years.

Conclusion DIV remains a major cardiac malformation although both prenatal diagnostics and cardiac surgery have improved over the years. The course of pregnancy is commonly uneventful. All children need univentricular palliation. The children are slightly physically limited, develop a normal intellect, and attend school regularly.

Zusammenfassung

Ziel Beurteilung der assoziierten kardialen Anomalien, des intrauterinen und postnatalen Verlaufs von Feten mit doppeltem Einlassventrikel (DIV).

Methoden Retrospektive Analyse der pränatalen Befunde von 35 Patienten mit DIV zwischen 2003 und 2021 in 2 tertiären Referenzzentren in Deutschland. Bei allen Feten wurden eine fetale Echokardiografie und eine Feindiagnostik durchgeführt. Postnatale Follow-up-Daten wurden aus pädiatrischen Berichten entnommen.

Ergebnisse 33 Fälle von DIV wurden pränatal korrekt diagnostiziert. 24 Feten (72,7%) hatten einen DIV mit dominanter linker (DILV), 7 (21,2%) mit dominanter rechtsventrikulärer (DIRV) und 2 Fälle (6%) mit unbestimmter Morphologie (DIIV). 4 (16,6%) waren Holmes-Herzen. 5 der 7 Feten (71,4%) mit DIRV hatten einen DORV. Eine Malposition der großen Arterien lag in 84,8% vor. Aneuploidien waren nicht vorhanden. Ein Abbruch wurde in 8 Fällen (24,2%) durchgeführt. 24 Feten (72,7%) wurden lebend geboren, davon waren 5 (20,8%) weiblich und 19 (79,2%) männlich. Medianes Gestationsalter bei der Geburt betrug 38+2,5 Wochen. Bis auf ein Kind erhielten alle eine univentrikuläre Palliation. Das mediane Follow-up betrug 5,83 Jahre mit einer adjustierten Überlebensrate von 91,6% (22 von 24 Lebendgeborenen). Ein Fall von Fontan-Versagen mit 15,7 Jahren.

Schlussfolgerung DIV bleibt ein schwerer Herzfehler, auch wenn sich die pränatale Diagnostik und kardiochirurgische Palliation im Laufe der Jahre verbessert haben. Der Verlauf der Schwangerschaft ist meist problemlos. Alle Kinder erwartet eine univentrikuläre Palliation. Die Kinder sind körperlich leicht eingeschränkt, bei normalem Intellekt und gehen regelmäßig zur Schule.

Schlüsselwörter

Echokardiografie - Fetale Kardiologie - Pädiatrische Kardiologie - Double inlet ventricle - Pränatale Diagnostik

Keywords

Echocardiography - Fetal cardiology - Pediatric cardiology - Double inlet ventricle - Prenatal diagnosis

Publication History

Received: 05 January 2022

Accepted after revision: 29 April 2022

Article published online:
01 July 2022

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