title: Heart transplantation after Fontan: Results from a surgical Fontan cohort.
abstract: We performed a retrospective review of outcomes after heart transplantation during long-term follow-up of a surgical cohort of 1138 Fontan patients who were followed at the Mayo Clinic. Follow-up information was obtained from medical records and a clinical questionnaire that was mailed to patients not known to be deceased at the initiation of the study. Forty-four of 1138 Fontan patients with initial or subsequent evaluation at Mayo had cardiac transplantation between 1988 and 2014 (mean age at transplantation was 23.2 ± 12 yr, median was 19.8 yr; mean interval between Fontan and transplantation was 13.0 ± 7.7 yr, median was 13.1 yr). Two patients had combined organ transplantation (one heart-lung, one heart-liver). Twelve of the 44 (27%) patients had PLE prior to transplantation. There was no difference in post-bypass Fontan pressures or incidence of late reoperations for AVV repair/replacement between transplanted and non-transplanted patients. There were 16 (36%) deaths in the transplantation cohort; seven occurred within 30 days of transplantation. Overall one, five, 10, and 15 yr post-transplantation survival was 80%, 72%, 69%, and 55%, respectively. Although this is a challenging group of patients, intermediate-term results suggest that cardiac transplantation remains a reasonable option for patients with a failed Fontan circulation.
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Kavitha N
authors.initials: KN
authors.lastname: Pundi
authors.affiliation: Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN, USA.
authors.forename: Krishna
authors.initials: K
authors.lastname: Pundi
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: David J
authors.initials: DJ
authors.lastname: Driscoll
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
authors.affiliation: Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA.
authors.forename: Zhuo
authors.initials: Z
authors.lastname: Li
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Sonja H
authors.initials: SH
authors.lastname: Dahl
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Bassem N
authors.initials: BN
authors.lastname: Mora
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.|Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
authors.forename: Patrick W
authors.initials: PW
authors.lastname: O'Leary
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN, USA.
authors.forename: Richard C
authors.initials: RC
authors.lastname: Daly
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.|Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
authors.forename: Frank
authors.initials: F
authors.lastname: Cetta
authors.affiliation: Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA.|Division of Cardiovascular Diseases, Mayo Clinic, Rochester, MN, USA.
authors.forename: Jonathan N
authors.initials: JN
authors.lastname: Johnson
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008137:Longitudinal Studies; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D012086:Reoperation; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D055815:Young Adult
keywords: Fontan procedure; protein-losing enteropathy; transplant