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

TY - JOUR

T1 - Heart transplantation in arrhythmogenic right ventricular cardiomyopathy — Experience from the Nordic ARVC Registry

AU - Gilljam, Thomas

AU - Haugaa, Kristina H.

AU - Jensen, Henrik K.

AU - Svensson, Anneli

AU - Bundgaard, Henning

AU - Hansen, Jim

AU - Dellgren, Göran

AU - Gustafsson, Finn

AU - Eiskjær, Hans

AU - Andreassen, Arne K.

AU - Sjögren, Johan

AU - Edvardsen, Thor

AU - Holst, Anders G.

AU - Svendsen, Jesper Hastrup

AU - Platonov, Pyotr G.

PY - 2018

Y1 - 2018

N2 - 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.

AB - 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.

KW - Arrhythmogenic right ventricular cardiomyopathy

KW - Heart transplantation

U2 - 10.1016/j.ijcard.2017.10.076

DO - 10.1016/j.ijcard.2017.10.076

M3 - Journal article

C2 - 29107359

AN - SCOPUS:85032343360

SN - 0167-5273

VL - 250

SP - 201

EP - 206

JO - International Journal of Cardiology

JF - International Journal of Cardiology

ER -