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Prenatal diagnosis and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center
20 September 2018 (online)
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.
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.
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.
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.