title: Cardiac transplantation in adults with congenital heart disease.
abstract: BACKGROUND
  Due to increasing success with repair or palliation in childhood, there is a rapidly growing population of adult patients with complex congenital heart disease who may require transplantation. There remains little data on outcomes of cardiac transplantation in this group.
  
  
  METHODS
  38 orthotopic cardiac transplants were performed in 37 patients (18 men) > or =18 years of age with congenital heart disease (CHD) from 1988 to 2009 in our institution. Outcomes were reviewed using medical records and transplant databases.
  
  
  RESULTS
  15 patients (41%) had univentricular and 22 (59%) biventricular physiology. The biggest group was transposition of the great arteries following atrial switch in eight patients (22%). Six (16%) had no previous surgical intervention. Mean age at transplant was 33.5 years (range 19.1-59.9 years). 11 patients (30%) required additional surgical procedures at transplant. 16 (43%) died, 12 early and 4 late deaths (1.8, 2.4, 2.7 and 7 years). Survival was 70% at 30 days, 68% at 1 year, 58% at 5 years and 53% at 10 and 15 years. Outcome improved in later eras with reduction in 30-day mortality from 50% to 18% and increase in 5-year survival from 50% to 69%. Two patients developed post-transplant lymphoproliferative disease. None required long-term renal replacement therapy. One patient was re-transplanted for cardiac allograft vasculopathy.
  
  
  CONCLUSIONS
  While operative mortality following cardiac transplantation for adult congenital heart disease is higher than for other diagnostic groups, long-term survival is good and comparable to patients without CHD. Disappointing early results are improved with increasing experience.
authors.affiliation: Department of Paediatric Cardiology, Freeman Hospital, Newcastle upon, UK. claire.irving@nuth.nhs.uk
authors.forename: C
authors.initials: C
authors.lastname: Irving
authors.forename: G
authors.initials: G
authors.lastname: Parry
authors.forename: J
authors.initials: J
authors.lastname: O'Sullivan
authors.forename: J H
authors.initials: JH
authors.lastname: Dark
authors.forename: R
authors.initials: R
authors.lastname: Kirk
authors.forename: D S
authors.initials: DS
authors.lastname: Crossland
authors.forename: M
authors.initials: M
authors.lastname: Chaudhari
authors.forename: M
authors.initials: M
authors.lastname: Griselli
authors.forename: J R L
authors.initials: JR
authors.lastname: Hamilton
authors.forename: A
authors.initials: A
authors.lastname: Hasan
mesh_terms: D000328:Adult; D002423:Cause of Death; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D016027:Heart Transplantation; D006801:Humans; D007165:Immunosuppression Therapy / Q000379:methods; D008297:Male; D008875:Middle Aged; D018579:Patient Selection; D011182:Postoperative Care / Q000379:methods; D011379:Prognosis; D012086:Reoperation; D016019:Survival Analysis; D016896:Treatment Outcome; D055815:Young Adult