Subscribe to RSS
DOI: 10.1055/s-0038-1671112
Prenatal diagnosis and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center
Publication History
Publication Date:
20 September 2018 (online)
Purpose:
To assess all associated anomalies, the intrauterine course and postnatal outcome of fetuses with DORV. The morphological variants and accuracy of prenatal diagnosis were assessed.
Methods:
All cases of DORV diagnosed prenatally over a period of 8 years were retrospectively collected in a single tertiary referral center. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed.
Results:
46 cases of DORV were diagnosed prenatally. 1 case had TGA with VSD postnatally and was excluded from the outcome. All 45 fetuses with DORV had major additional cardiac anomalies, 30 (66.7%) had extra-cardiac anomalies, 13 (28.9%) had chromosomal or syndromal anomalies and 5 (11.1%) were highly suspicious for non-chromosomal syndromes.
There were 17 terminations (37.8%), 2 (4.4%) intrauterine and 7 (15.6%) postnatal deaths. 19 (42.2%) children survived. Mean follow up was 32 months (range, 2 – 72).
8 (40.0%) children achieved biventricular repair, 12 (60.0%) univentricular palliation and 2 are waiting for surgery. After surgery, 14/17 (82.4%) children were healthy without any limitations. In 96.3%, prenatal diagnosis of DORV was correct.
Conclusion:
DORV is a 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.
#