title: Repair of neonates and young infants with Ebstein's anomaly and related disorders.
abstract: BACKGROUND
  Severely symptomatic neonates and young infants with Ebstein's anomaly usually die without surgical intervention. The relative risks and benefits of single-ventricle palliation versus a two-ventricle repair are uncertain. In a recent series, 69% early survival with single-ventricle palliation was reported in 16 neonates with Ebstein's anomaly. Our institutional bias has been to do a two-ventricle repair in all such patients.
  
  
  METHODS
  We reviewed our entire surgical experience with a two-ventricle repair in the severely symptomatic neonate (n = 22) and young infant (n = 5). The indications for operation were ventilator dependence, severe cardiac failure, prostaglandin-dependent circulation, and gross cardiomegaly.
  
  
  RESULTS
  Between 1994 and 2006, 27 consecutive patients with Ebstein's anomaly underwent operation. Associated comorbidities included anatomic or functional pulmonary atresia (n = 18), ventricular septal defects (n = 3), small left ventricle (n = 3), hypoplastic branch pulmonary arteries (n = 3), previous cardiac surgery (n = 4), significant intracranial hemorrhage (n = 3), hepatic necrosis and renal insufficiency (n = 3), and malignant tachyarrhythmias (n = 4). Operations consisted of tricuspid valve repair (n = 23) or valve replacement (n = 2), Blalock-Taussig shunt only (n = 1), and bilateral pulmonary arterioplasty with bidirectional Glenn (n = 1). Hospital survival was 74%, and there have been no late deaths during a median follow-up period of 5.4 years (range, 0.2 to 12 years). Three patients required tricuspid valve replacement during the follow-up period. Late arrhythmia requiring medication is present in 1 patient. All patients are currently in New York Heart Association functional class I.
  
  
  CONCLUSIONS
  Two-ventricle repair currently has similar early survival compared with single-ventricle palliation. The advantages of a better physiologic repair can be anticipated for a longer follow-up period.
authors.affiliation: Department of Thoracic and Cardiovascular Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA. ckc@uab.edu
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Knott-Craig
authors.forename: Steven P
authors.initials: SP
authors.lastname: Goldberg
authors.forename: Edward D
authors.initials: ED
authors.lastname: Overholt
authors.forename: Edward V
authors.initials: EV
authors.lastname: Colvin
authors.forename: James K
authors.initials: JK
authors.lastname: Kirklin
mesh_terms: D004437:Ebstein Anomaly / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006343:Heart Septal Defects / Q000503:physiopathology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D020300:Intracranial Hemorrhages / Q000453:epidemiology; D008099:Liver / Q000473:pathology; D009336:Necrosis; D018579:Patient Selection; D012121:Respiration, Artificial; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000601:surgery