title: Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes.
abstract: OBJECTIVE
  Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH.
  
  
  METHODS
  We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC).
  
  
  RESULTS
  Of initially 155 patients, 128 were liveborn (82.6%). Ten neonates (7.8%) were lost to follow-up, in three (2.5%) neonates, parents decided for compassionate care. Overall survival after prenatal diagnosis of functionally UVH was 67.1%, and 90.4% on an intention-to-treat basis. Survival after surgery reached 93.7%. The majority of deaths occurred within the group of dominant RV (10/74, 13.5%). High risk HLHS with restrictive foramen ovale was associated with the lowest survival rate (13/17, 76.5%) with significant difference compared to survival rate in dominant LV (40/41, 97.6%, p < 0.05).
  
  
  CONCLUSIONS
  These results should be explained to parents to ensure informed decisions and counselling. © 2016 John Wiley & Sons, Ltd.
authors.affiliation: University Hospital UKGM, Justus-Liebig University, Department of OB/GYN, Division of Prenatal Medicine, Giessen, Germany.
authors.forename: Aline
authors.initials: A
authors.lastname: Wolter
authors.affiliation: University Hospital UKGM, Justus-Liebig University, Department of OB/GYN, Division of Prenatal Medicine, Giessen, Germany.
authors.forename: Sina
authors.initials: S
authors.lastname: Nosbüsch
authors.affiliation: University Hospital UKGM, Justus-Liebig University, Department of OB/GYN, Division of Prenatal Medicine, Giessen, Germany.
authors.forename: Andreea
authors.initials: A
authors.lastname: Kawecki
authors.affiliation: University Hospital UKGM, Justus-Liebig University, Department of OB/GYN, Division of Prenatal Medicine, Giessen, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Degenhardt
authors.affiliation: University Hospital UKGM, Phillips University, Department of OB/GYN, Division of Prenatal Medicine, Marburg, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Enzensberger
authors.affiliation: University Hospital, Klinikum rechts der Isar, Technische Universität, Department of OB/GYN, München, Germany.
authors.forename: Oliver
authors.initials: O
authors.lastname: Graupner
authors.affiliation: University Hospital UKGM, Justus-Liebig University, Department of OB/GYN, Division of Prenatal Medicine, Giessen, Germany.
authors.forename: Carina
authors.initials: C
authors.lastname: Vorisek
authors.affiliation: Pediatric Heart Center, University Hospital, Justus-Liebig University, Division of Pediatric Heart Surgery, Giessen, Germany.
authors.forename: Hakan
authors.initials: H
authors.lastname: Akintürk
authors.affiliation: Pediatric Heart Center, University Hospital, Justus-Liebig University, Division of Pediatric Heart Surgery, Giessen, Germany.
authors.forename: Can
authors.initials: C
authors.lastname: Yerebakan
authors.affiliation: Pediatric Heart Center, University Hospital, Justus-Liebig University, Division of Pediatric Cardiology, Giessen, Germany.
authors.forename: Markus
authors.initials: M
authors.lastname: Khalil
authors.affiliation: Pediatric Heart Center, University Hospital, Justus-Liebig University, Division of Pediatric Cardiology, Giessen, Germany.
authors.forename: Dietmar
authors.initials: D
authors.lastname: Schranz
authors.affiliation: praenatal.de, Prenatal Medicine and Genetics, Köln, Germany.
authors.forename: Jochen
authors.initials: J
authors.lastname: Ritgen
authors.affiliation: praenatal.de, Prenatal Medicine and Genetics, Köln, Germany.
authors.forename: Rüdiger
authors.initials: R
authors.lastname: Stressig
authors.affiliation: University Hospital UKGM, Justus-Liebig University, Department of OB/GYN, Division of Prenatal Medicine, Giessen, Germany.
authors.forename: Roland
authors.initials: R
authors.lastname: Axt-Fliedner
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology*; D000293:Adolescent; D000328:Adult; D005260:Female; D005858:Germany / Q000453:epidemiology; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000401:mortality* / Q000601:surgery; D006343:Heart Septal Defects / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging; D059446:Heterotaxy Syndrome / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D007231:Infant, Newborn; D059012:Lost to Follow-Up; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D012189:Retrospective Studies; D015996:Survival Rate; D018785:Tricuspid Atresia / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D016216:Ultrasonography, Prenatal; D055815:Young Adult