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

Gilljam, Thomas; Haugaa, Kristina H.; Jensen, Henrik K.; Svensson, Anneli; Bundgaard, Henning; Hansen, Jim; Dellgren, Göran; Gustafsson, Finn; Eiskjær, Hans and Andreassen, Arne K., et al. (2018) In International Journal of Cardiology 250. p.201-206
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... (More)

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.

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publication status
published
subject
keywords
Arrhythmogenic right ventricular cardiomyopathy, Heart transplantation
in
International Journal of Cardiology
volume
250
pages
201 - 206
publisher
Elsevier
external identifiers
  • scopus:85032343360
ISSN
0167-5273
DOI
10.1016/j.ijcard.2017.10.076
language
English
LU publication?
yes
id
98abd66a-cfe0-4f57-b6a3-00c89e1d1d64
date added to LUP
2017-11-15 14:12:55
date last changed
2018-01-07 12:25:56
@article{98abd66a-cfe0-4f57-b6a3-00c89e1d1d64,
  abstract     = {<p>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 &lt;. 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.</p>},
  author       = {Gilljam, Thomas and Haugaa, Kristina H. and Jensen, Henrik K. and Svensson, Anneli and Bundgaard, Henning and Hansen, Jim and Dellgren, Göran and Gustafsson, Finn and Eiskjær, Hans and Andreassen, Arne K. and Sjögren, Johan and Edvardsen, Thor and Holst, Anders G. and Svendsen, Jesper Hastrup and Platonov, Pyotr G.},
  issn         = {0167-5273},
  keyword      = {Arrhythmogenic right ventricular cardiomyopathy,Heart transplantation},
  language     = {eng},
  pages        = {201--206},
  publisher    = {Elsevier},
  series       = {International Journal of Cardiology},
  title        = {Heart transplantation in arrhythmogenic right ventricular cardiomyopathy - Experience from the Nordic ARVC Registry},
  url          = {http://dx.doi.org/10.1016/j.ijcard.2017.10.076},
  volume       = {250},
  year         = {2018},
}