Heart transplantation in arrhythmogenic right ventricular cardiomyopathy - Experience from the Nordic ARVC Registry

Research output: Contribution to journalArticle

Abstract

Objective: There is a paucity of data on heart transplantation (HTx) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), and specific recommendations on indications for listing ARVC patients for HTx are lacking. In order to delineate features pertinent to HTx assessment, we explored the pre-HTx characteristics and clinical history in a cohort of ARVC patients who received heart transplants. Methods: Data from 31 ARVC/HTx patients enrolled in the Nordic ARVC Registry, transplanted between 1988 and 2014 at a median age of 46. years (14-65), were compared with data from 152 non-transplanted probands with Definite ARVC according to 2010 Task Force Criteria from the same registry. Results: The HTx patients were younger at presentation, median 31 vs. 38. years (p = 0.001). There was no difference in arrhythmia-related events. The indication for HTx was heart failure in 28 patients (90%) and ventricular arrhythmias in 3 patients (10%). During median follow-up of 4.9. years (0.04-28), there was one early death and two late deaths. Survival was 91% at 5. years after HTx. Age at first symptoms under 35. years independently predicted HTx in our cohort (OR = 7.59, 95% CI 2.69-21.39, p <. 0.001). Conclusion: HTx in patients with ARVC is performed predominantly due to heart failure. This suggests that current 2016 International Society for Heart and Lung Transplantation heart transplant listing recommendations for other cardiomyopathies could be applicable in many cases when taking into account the haemodynamic consequences of right ventricular failure in conjunction with ventricular arrhythmia.

Details

Authors
  • Thomas Gilljam
  • Kristina H. Haugaa
  • Henrik K. Jensen
  • Anneli Svensson
  • Henning Bundgaard
  • Jim Hansen
  • Göran Dellgren
  • Finn Gustafsson
  • Hans Eiskjær
  • Arne K. Andreassen
  • Johan Sjögren
  • Thor Edvardsen
  • Anders G. Holst
  • Jesper Hastrup Svendsen
  • Pyotr G. Platonov
Organisations
External organisations
  • Sahlgrenska Academy
  • University of Oslo
  • Aarhus University Hospital
  • Linköping University
  • Copenhagen University Hospital
  • University of Copenhagen
  • Norwegian Radium Hospital
  • Skåne University Hospital
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Cardiac and Cardiovascular Systems

Keywords

  • Arrhythmogenic right ventricular cardiomyopathy, Heart transplantation
Original languageEnglish
Pages (from-to)201-206
JournalInternational Journal of Cardiology
Volume250
Publication statusPublished - 2018
Publication categoryResearch
Peer-reviewedYes