title: Complete repair of Ebstein anomaly in neonates and young infants: a 16-year follow-up.
abstract: OBJECTIVE
  The purpose of this study was to review the long-term outcome of patients with Ebstein anomaly who underwent complete repair as neonates and young infants.
  
  
  METHODS
  Between March 1994 and May 2010, 32 patients (23 neonates and 9 young infants) underwent surgery for Ebstein anomaly. Mean weight was 3.9 ± 2.0 kg (range, 1.9-8.6 kg). The Great Ormond Street Echocardiography score was greater than 1.5 in 22 of the 23 neonates and greater than 1.0 in all infants. All associated cardiac defects were repaired including pulmonary atresia in 15 and ventricular septal defect in 4. Primary outcome measures included (1) early and late survival, (2) freedom from reoperation, (3) durability of tricuspid valve repair, and (4) functional status.
  
  
  RESULTS
  Early survival was 78.1% (25/32). There was 1 late death. Fifteen-year survival estimate was 74% ± 8%. For patients with pulmonary atresia, early and late survival was 60% ± 12% (9/15) versus 94.1% (16/17) (P < .05), respectively; for those without pulmonary atresia, early and late survival was 60% ± 12% versus 85% ± 10 (P = .06), respectively. The mean follow-up was 5.9 ± 4.5 years (0.1-16 years). A biventricular repair was achieved in 90.6% (29/32) patients. Median preoperative tricuspid regurgitation was 4/4 and at late follow-up, 1/4. Freedom from reoperation at 15 years was 74% ± 10% for patients undergoing biventricular repair. All survivors are in New York Heart Association class I or II.
  
  
  CONCLUSIONS
  Biventricular repair of Ebstein anomaly in symptomatic neonates is feasible with good early and late survival, especially in those without pulmonary atresia. Tricuspid valve repair is durable, and functional status is excellent.
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, University of Tennessee Health Sciences Center/Le Bonheur Children's Hospital, Memphis, Tenn, USA. uboston@uthsc.edu
authors.forename: Umar S
authors.initials: US
authors.lastname: Boston
authors.forename: Steven P
authors.initials: SP
authors.lastname: Goldberg
authors.forename: Kent E
authors.initials: KE
authors.lastname: Ward
authors.forename: Edward D
authors.initials: ED
authors.lastname: Overholt
authors.forename: Thomas
authors.initials: T
authors.lastname: Spentzas
authors.forename: Thomas K
authors.initials: TK
authors.lastname: Chin
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Knott-Craig
mesh_terms: D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D004437:Ebstein Anomaly / Q000000981:diagnostic imaging / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D005500:Follow-Up Studies; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D012086:Reoperation; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014463:Ultrasonography; D014481:United States