title: Surgical management of symptomatic neonates with Ebstein's anomaly: choice of operation.
abstract: OBJECTIVE
  Symptomatic neonates with Ebstein's anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset.
  
  
  METHODS
  A total of 32 consecutive symptomatic neonates with Ebstein's anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients' weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein's anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes' palliation and 2 Blalock-Taussig shunts. Six recent patients with Ebstein's anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair.
  
  
  RESULTS
  Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein's anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein's anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4).
  
  
  CONCLUSIONS
  Surgical management of neonates with Ebstein's anomaly remains challenging. For neonates with Ebstein's anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.
authors.affiliation: 1Division of Pediatric Cardiovascular Surgery,Le Bonheur Children's Hospital,University of Tennessee Health Science Center,Memphis,Tennessee,United States of America.
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Knott-Craig
authors.affiliation: 1Division of Pediatric Cardiovascular Surgery,Le Bonheur Children's Hospital,University of Tennessee Health Science Center,Memphis,Tennessee,United States of America.
authors.forename: Thittamaranahalli Kariyappa S
authors.initials: TK
authors.lastname: Kumar
authors.forename: Alejandro R
authors.initials: AR
authors.lastname: Arevalo
authors.affiliation: 2Divison of Pediatric Cardiology,Le Bonheur Children's Hospital,University of Tennessee Health Science Center,Memphis,Tennessee,United States of America.
authors.forename: Vijaya M
authors.initials: VM
authors.lastname: Joshi
mesh_terms: D019468:Disease Management; D004437:Ebstein Anomaly / Q000150:complications / Q000401:mortality* / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D018633:Pulmonary Atresia / Q000150:complications / Q000401:mortality* / Q000601:surgery*; D011664:Pulmonary Valve / Q000601:surgery; D014261:Tricuspid Valve / Q000601:surgery
keywords: neonate; pulmonary valve; right ventricle; surgical techniques; tricuspid valve