title: Case Series: Heart Transplantation After Fontan Operation-Single-Center Experience.
abstract: BACKGROUND
  Fontan failure (FF) occurs rarely. In patients with Fontan failure, heart transplantation is believed to be the most effective therapy. We review our experience in heart transplantations after the Fontan operation.
  
  
  METHODS
  From July 1987 to December 2014, 4 of 513 patients underwent orthotopic heart transplantation (OHT). Among them, 4 were due to FF. We reviewed these 4 cases via retrospective chart review. Clinical history, laboratory data, surgical technique, perioperative variables, and outcomes of long-term follow-up are presented herein. The primary outcomes were hospital mortality, 1-year-survival rate, and 4-year-survival rate. The secondary outcome is the improvement in patients with protein-losing enteropathy.
  
  
  RESULTS
  The hospital mortality rate was 0% in the 4 FF patients receiving OHT. No surgically related hemorrhage or infection was observed. The 1-year-survival rate was 100% (n = 4) and the 4-year-survival rate 50% (n = 2). One patient died of posttransplantation lymphoproliferative disorder. Hypoalbuminemia improved in 1 of 3 patients 4 months after OHT.
  
  
  CONCLUSIONS
  Despite technical challenges, heart transplantation can be performed successfully in patients with Fontan operation. However, protein-losing enteropathy might not be resolved quickly after heart transplantation.
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: S-N
authors.initials: SN
authors.lastname: Lin
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: S-C
authors.initials: SC
authors.lastname: Huang
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Y-S
authors.initials: YS
authors.lastname: Chen
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: N-H
authors.initials: NH
authors.lastname: Chih
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: C-H
authors.initials: CH
authors.lastname: Wang
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: N-K
authors.initials: NK
authors.lastname: Chou
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: H-Y
authors.initials: HY
authors.lastname: Yu
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: I-H
authors.initials: IH
authors.lastname: Wu
authors.affiliation: Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: C-T
authors.initials: CT
authors.lastname: Shun
authors.affiliation: Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan; Department of Surgery, Fu Jen Catholic University Hospital, and Fu Jen Catholic University College of Medicine, New Taipei City, Taiwan. Electronic address: wangp@ntu.edu.tw.
authors.forename: S-S
authors.initials: SS
authors.lastname: Wang
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D008297:Male; D012086:Reoperation; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D016896:Treatment Outcome