title: Anatomic repair of Ebstein's malformation: lessons learned with cone reconstruction.
abstract: BACKGROUND
  Reproducible repair of Ebstein's malformation is challenging and numerous surgical techniques have been described. We reviewed our experience with the cone reconstruction.
  
  
  METHODS
  Between June 2007 and December 2011, 89 patients (47 female; 53%) underwent cone reconstruction (median age 19 years; range, 19 days to 68 years). Indication for operation was progressive cardiomegaly in 43 (48%), cyanosis in 29 (33%), and heart failure in 13 (15%). Prior tricuspid valve repair was performed in 12 patients (13%). Severe tricuspid regurgitation (TR) was present in 75 patients (84%).
  
  
  RESULTS
  All patients underwent cone reconstruction (360-degree leaflet tissue repair anchored at true annulus). Modifications included ringed annuloplasty in 57 patients (64%), leaflet augmentation in 28 patients (31%), and autologous chordae in 17 patients (19%). Bidirectional cavopulmonary anastomosis was performed in 21 patients (24%). Early mortality occurred in 1 patient (1%). Early reoperation for recurrent TR occurred in 12 patients (13%); re-repair was performed in 6 patients (50%), and 6 (50%) required replacement. Mean follow-up was 19.7 ± 24.7 months. There was no late mortality or reoperation. At follow-up, 72 patients (87%) had no or mild TR, 9 (11%) had moderate TR, and 2 patients (2%) had severe TR. Ringed annuloplasty was associated with less than moderate TR at dismissal (p = 0.01).
  
  
  CONCLUSIONS
  The learning curve for cone reconstruction is steep, but early mortality is low. Cone reconstruction with ringed annuloplasty results in less TR and should be used whenever possible. Longer follow-up is essential to determine late durability of cone reconstruction.
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA. dearani.joseph@mayo.edu
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
authors.forename: Sameh M
authors.initials: SM
authors.lastname: Said
authors.forename: Patrick W
authors.initials: PW
authors.lastname: O'Leary
authors.forename: Harold M
authors.initials: HM
authors.lastname: Burkhart
authors.forename: Roxann D
authors.initials: RD
authors.lastname: Barnes
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D006348:Cardiac Surgical Procedures / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014261:Tricuspid Valve / Q000601:surgery*; D055815:Young Adult