title: Prenatal detection and postnatal management of double outlet right ventricle (DORV) in 21 singleton pregnancies.
abstract: OBJECTIVE
  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.
authors.affiliation: Department of Prenatal Medicine, University of Schleswig-Holstein Medical Center , Luebeck, 23538, Germany. dhartge@gmail.com
authors.forename: David Rafael
authors.initials: DR
authors.lastname: Hartge
authors.forename: Linda
authors.initials: L
authors.lastname: Niemeyer
authors.forename: Roland
authors.initials: R
authors.lastname: Axt-Fliedner
authors.forename: Martin
authors.initials: M
authors.lastname: Krapp
authors.forename: Ulrich
authors.initials: U
authors.lastname: Gembruch
authors.forename: Ute
authors.initials: U
authors.lastname: Germer
authors.forename: Jan
authors.initials: J
authors.lastname: Weichert
mesh_terms: D002887:Chromosomes, Human, Pair 18; D004310:Double Outlet Right Ventricle / Q000000981:diagnostic imaging* / Q000235:genetics / Q000601:surgery; D004314:Down Syndrome / Q000150:complications / Q000235:genetics; D005260:Female; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D007621:Karyotyping; D008297:Male; D011247:Pregnancy; D011256:Pregnancy Outcome; D012189:Retrospective Studies; D014314:Trisomy / Q000235:genetics; D016216:Ultrasonography, Prenatal