OBJECTIVES: To evaluate the prenatal features, associated anomalies and the postnatal management in fetuses with prenatally detected double-outlet right ventricle (DORV). METHODS: Retrospective analysis of prenatal, pediatric and cardiosurgical records for all fetuses with prenatally diagnosed DORV at a single tertiary referral center. RESULTS: 21 cases were detected prenatally with DORV (average gestational age 23(3/7) weeks). Fetal karyotyping was available in all cases revealing 3 cases with trisomy 18 and one fetus with trisomy 21. 10/21 (47.6%) cases had additional cardiac anomalies only, one fetus (4.8%) had extra-cardiac anomalies only. 8/21 cases (38.1%) presented with both additional cardiac- and extra-cardiac anomalies. A total of 13/21 (61.9%) fetuses were live-born. Average gestational age at delivery was 37(3/7) gestational weeks. There were four Rastelli maneuvers, one Fontan intervention and three children with resection of a coarctation of the aorta among the group of the surviving infants. CONCLUSIONS: Prenatal, sonographic detection of DORV is feasible. The majority of the cases shows a normal karyotype. Outcome strongly depends on the presence and severity of additional anomalies of the fetus. Surgical intervention in DORV can lead to a favorable outcome in simple DORV. DORV not aggravated by additional defects seems to be no contraindication for a vaginal delivery.
OBJECTIVES: To evaluate the prenatal features, associated anomalies and the postnatal management in fetuses with prenatally detected double-outlet right ventricle (DORV). METHODS: Retrospective analysis of prenatal, pediatric and cardiosurgical records for all fetuses with prenatally diagnosed DORV at a single tertiary referral center. RESULTS: 21 cases were detected prenatally with DORV (average gestational age 23(3/7) weeks). Fetal karyotyping was available in all cases revealing 3 cases with trisomy 18 and one fetus with trisomy 21. 10/21 (47.6%) cases had additional cardiac anomalies only, one fetus (4.8%) had extra-cardiac anomalies only. 8/21 cases (38.1%) presented with both additional cardiac- and extra-cardiac anomalies. A total of 13/21 (61.9%) fetuses were live-born. Average gestational age at delivery was 37(3/7) gestational weeks. There were four Rastelli maneuvers, one Fontan intervention and three children with resection of a coarctation of the aorta among the group of the surviving infants. CONCLUSIONS: Prenatal, sonographic detection of DORV is feasible. The majority of the cases shows a normal karyotype. Outcome strongly depends on the presence and severity of additional anomalies of the fetus. Surgical intervention in DORV can lead to a favorable outcome in simple DORV. DORV not aggravated by additional defects seems to be no contraindication for a vaginal delivery.