Subscribe to RSS
DOI: 10.1055/s-0038-1670413
Prenatal diagnosis and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center
Publication History
Publication Date:
24 October 2018 (online)
Purpose:
To assess the spectrum of associated anomalies, the intrauterine course, the postnatal outcome and management of fetuses with DORV.
Methods:
All cases of DORV diagnosed prenatally over a period of 8 years were retrospectively collected in a single tertiary referral center in Germany. All additional prenatal findings were assessed and correlated with the outcome. The morphological variants of DORV and the accuracy of prenatal diagnosis was assessed.
Results:
46 cases of DORV were diagnosed prenatally. Mean gestational age at first diagnosis was 21+4 weeks (range, 13 – 37). 1 case had TGA with VSD postnatally and was excluded from the outcome. All 45 fetuses with DORV (100%) had major additional cardiac anomalies, 30 (66.7%) had extra-cardiac anomalies and 13 (28.9%) had chromosomal or syndromal anomalies. Due to their complex additional anomalies, 5 (11.1%) of our 45 fetuses were highly suspicious for non-chromosomal syndromes, although molecular diagnosis could not be provided. There were 17 terminations of pregnancy (37.8%), 2 (4.4%) intrauterine and 7 (15.6%) postnatal deaths. 19/22 (86.4%) live born children with an intention to treat were alive at last follow up. Mean follow up among survivors was 32 months (range, 2 – 72). 20/22 children underwent postnatal sugery, 8 (40.0%) achieved biventricular repair, 12 (60.0%) univentricular palliation and 2 recently born children are waiting for surgery. After surgery, 14/17 (82.4%) children were healthy without any limitations. Correct prenatal diagnosis of DORV was made in 26/27 (96.3%). If the relation of great arteries, position of VSD and additional cardiac and extra-cardiac anomalies are taken into account, the prenatal diagnosis was correct in 24/27 (88.9%) cases.
Conclusion:
DORV is a rare and often complex cardiac anomaly that can be diagnosed prenatally with high precision. DORV is frequently associated with major additional anomalies, leading to a high intrauterine and postnatal loss rate due to intrauterine terminations or declined postnatal therapy. Without these anomalies, prognosis is good, although 60% of children will achieve single ventricle palliation.