title: Temporal trends and changing profile of adults with congenital heart disease undergoing heart transplantation.
abstract: OBJECTIVE
  To investigate the temporal trends in profile and outcomes of adults with congenital heart disease (ACHD) undergoing heart transplantation (HT).
  
  
  RESULTS
  Out of a multi-institutional series of 2257 HT from 1988 to 2012, 100 HT were performed in 97 ACHD. We evaluated clinical characteristics, underlying defect, surgical history, perioperative issues, and outcomes. We compared two eras: era 1 (1988-2005, n = 48) and era 2 (2006-2012, n = 49). Mean age at HT was 30.3 ± 10.5 years. Twenty-five patients (25.8%) had biventricular physiology with a systemic right ventricle and 43 patients (44%) had univentricular physiology. Adults with congenital heart disease severity were classified as great complexity (74.2%), moderate (21.7%), and simple (4.1%). During a median follow-up of 28.7 months [0-282], 44 patients died. Early mortality was high (34%; 95% CI 0.2536-0.4390). Survival was 63.9% at 1 year. The proportion of univentricular patients did not change. Biventricular patients with systemic right ventricle significantly increased in era 2 (16.7  vs. 34.7%, P = 0.04) due to increasing number of transposition of the great arteries with atrial switch. Although the proportion of great complexity ACHD did not change significantly in era 2 (81.6%  vs. and 66.7% in era 1, P = 0.09), ACHD recipients have more advanced disease, being more likely hospitalized (P = 0.03), receiving intravenous inotropes (P = 0.01), under assist devices (P = 0.04), or UNOS status 1 (P = 0.02) at the time of HT. Survival rates were comparable.
  
  
  CONCLUSIONS
  Despite a worse risk profile, mortality after HT in ACHD did not increase. Improving survival of complex CHD will probably amplify the proportion of complex ACHD recipients with more advanced disease.
authors.affiliation: Adult Congenital Heart Disease Unit, Cardiology Department, M3C, Reference Center for Complex Congenital Heart Diseases, European George Pompidou Hospital, AP-HP, Paris, France INSERM-UMRS 1138 Team 22, Cordeliers Research Center, Paris Descartes University, Paris, France sarah.cohen@aphp.fr.
authors.forename: Sarah
authors.initials: S
authors.lastname: Cohen
authors.affiliation: Marie Lannelongue Hospital, Pediatric and Congenital Cardiac Surgery, M3C, Reference Center for Complex Congenital Heart Diseases, Le Plessis Robinson, France.
authors.forename: Lucile
authors.initials: L
authors.lastname: Houyel
authors.affiliation: Department of Cardiovascular Surgery, European George Pompidou Hospital, AP-HP, Paris, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Guillemain
authors.affiliation: Cardio-Thoracic Surgery Unit and Pathology Department, La Pitié-Salpétrière Hospital, APHP, Paris, France.
authors.forename: Shaida
authors.initials: S
authors.lastname: Varnous
authors.affiliation: INSERM-UMRS 1138 Team 22, Cordeliers Research Center, Paris Descartes University, Paris, France Department of Medical Informatics and Public Health, European George Pompidou Hospital, AP-HP, Paris, France.
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Jannot
authors.affiliation: Pediatric Cardiology, M3C, Reference Center for Complex Congenital Heart Diseases, Necker Hospital for Sick Children, Paris, France Paris Descartes University, Paris, France.
authors.forename: Magalie
authors.initials: M
authors.lastname: Ladouceur
authors.affiliation: Pediatric Cardiology, M3C, Reference Center for Complex Congenital Heart Diseases, Necker Hospital for Sick Children, Paris, France Paris Descartes University, Paris, France.
authors.forename: Younes
authors.initials: Y
authors.lastname: Boudjemline
authors.affiliation: Pediatric Cardiology, M3C, Reference Center for Complex Congenital Heart Diseases, Necker Hospital for Sick Children, Paris, France Paris Descartes University, Paris, France.
authors.forename: Damien
authors.initials: D
authors.lastname: Bonnet
authors.affiliation: Adult Congenital Heart Disease Unit, Cardiology Department, M3C, Reference Center for Complex Congenital Heart Diseases, European George Pompidou Hospital, AP-HP, Paris, France.
authors.forename: Laurence
authors.initials: L
authors.lastname: Iserin
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D006330:Heart Defects, Congenital / Q000401:mortality / Q000601:surgery*; D016027:Heart Transplantation / Q000401:mortality / Q000639:trends*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010297:Paris / Q000453:epidemiology; D011446:Prospective Studies; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: Adult congenital heart disease; Heart failure; Heart transplantation; Mortality; Severity