title: Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects.
abstract: OBJECTIVE
  To investigate the perinatal outcome of cases with a prenatal diagnosis of single-ventricle cardiac defects, single ventricle being defined as a dominant right ventricle (RV) or left ventricle (LV), in which biventricular circulation was not possible.
  
  
  METHODS
  We reviewed patients with a prenatal diagnosis of single-ventricle cardiac defects, made at one institution between 1995 and 2008. Cases diagnosed with double-inlet LV, tricuspid atresia, pulmonary atresia with intact ventricular septum and severe RV hypoplasia and those with hypoplastic left heart syndrome (HLHS) were included in the study population. Patients with HLHS were identified prenatally as being standard risk or high risk (HLHS with highly restrictive or intact atrial septum, mitral stenosis with aortic atresia and/or LV coronary artery sinusoids). Patients with an address over 200 miles from the hospital, diagnosed with heterotaxy syndrome or referred for fetal intervention, were excluded.
  
  
  RESULTS
  We identified 312 cases of single-ventricle cardiac defect (208 dominant RV; 104 dominant LV) that were diagnosed prenatally. Most (96%) patients with a dominant RV had HLHS. Among the total 312 cases there were 98 (31%) elective terminations of pregnancy (TOP), 12 (4%) cases of spontaneous fetal demise, 12 (4%) cases lost to prenatal follow-up and 190 (61%) live births. Among the 199 patients that underwent fetal echocardiography before 24 weeks' gestation, there were 97 (49%) cases of elective TOP. There was no difference in prenatal outcome between those with a dominant RV and those with a dominant LV (P = 0.98). Of the 190 live births, five received comfort care. With an average of 7 years' follow-up (to obtain data on the Fontan procedure), transplantation-free survival was lower in those with a dominant RV than in those with a dominant LV (standard-risk HLHS odds ratio (OR), 3.0 (P = 0.01); high-risk HLHS OR, 8.8 (P < 0.001)).
  
  
  CONCLUSIONS
  The prenatal outcome of cases with single-ventricle cardiac defects was similar between those with a dominant RV and those with a dominant LV, however postnatal intermediate-term survival favored those with a dominant LV. High-risk HLHS identified prenatally was associated with the lowest transplantation-free survival.
authors.affiliation: Department of Pediatric/Congenital Cardiology, Massachusetts General Hospital for Children, Boston, MA, USA.|Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
authors.forename: R S
authors.initials: RS
authors.lastname: Beroukhim
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
authors.forename: K
authors.initials: K
authors.lastname: Gauvreau
authors.affiliation: Department of Pediatric/Congenital Cardiology, Massachusetts General Hospital for Children, Boston, MA, USA.|Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
authors.forename: O J
authors.initials: OJ
authors.lastname: Benavidez
authors.affiliation: Department of Cardiac Surgery, Boston Children's Hospital, Boston, MA, USA.
authors.forename: C W
authors.initials: CW
authors.lastname: Baird
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
authors.forename: T
authors.initials: T
authors.lastname: LaFranchi
authors.affiliation: Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
authors.forename: W
authors.initials: W
authors.lastname: Tworetzky
mesh_terms: D000025:Abortion, Eugenic / Q000706:statistics & numerical data; D000293:Adolescent; D000328:Adult; D005260:Female; D005313:Fetal Death; D005865:Gestational Age; D016027:Heart Transplantation / Q000401:mortality; D006352:Heart Ventricles / Q000002:abnormalities* / Q000000981:diagnostic imaging; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging*; D007231:Infant, Newborn; D008875:Middle Aged; D011247:Pregnancy; D018633:Pulmonary Atresia / Q000000981:diagnostic imaging*; D012189:Retrospective Studies; D018785:Tricuspid Atresia / Q000000981:diagnostic imaging*; D016216:Ultrasonography, Prenatal; D055815:Young Adult
keywords: congenital heart disease; fetal; hypoplastic left heart syndrome; outcomes; postnatal; prenatal; termination of pregnancy; tricuspid atresia