Heart transplantations in the Nordic Arrhythmogenic Right Ventricular Cardiomyopathy Registry  
American College of Cardiology 2013  
PG Platonov; T Gilljam; AG Holst; KH Haugaa; T Edvardsen; R Borgquist; O Eschen; J Hansen; H Bundgaard; JH Svendsen  
Purpose: Arrythmogenic right ventricular cardiomyopathy (ARVC) is a progressive disease that in rare cases may lead to the need for heart transplantation (HTX). However, the knowledge concerning the patients characteristics and clinical outcome following heart transplantation in ARVC is limited to case reports or small patient series. We aimed to examine patient characteristics and clinical outcome in heart transplanted patients included in the Nordic ARVC Registry. Methods: Patients who underwent HTX (n=14, age 53±14 years, 10 male) were identified from the cohort of probands with definite ARVC by Task Force 2010 criteria enrolled in the registry in Denmark, Norway and Sweden (n=111, age 50±15 years, 69 male). The following clinical variables were reviewed: demographic characteristics and co-morbidities, competitive sports or vigorous exercise > 4 hours per week, diagnostic criteria and arrhythmic events defined as syncope, ventricular tachycardia (VT), aborted cardiac arrest (ACA) and appropriate ICD therapy that had occurred by inclusion in the registry. Data are presented as median [range]. Results: 1) Median age at HTX was 35 [18-58] years and duration of follow-up 4 [0-14] years. Two patients died 4 and 9 years after HTX from non-cardiac causes (at 48 and 61 year respectively). Prior to HTX, 3 patients survived aborted cardiac arrest, 4 received ICD for primary and 5 for secondary prevention of sudden death. 2) Among HTX-patients, females experienced their first arrhythmic event earlier than men (25 [14-40] vs 45 [33-56] years, p=0.014) and underwent both ICD implantation [26 [14-40] vs 47 [36-56], p=0.032) and HTX (33 [18-44] vs 52 [42-61], p=0.008) at a younger age. Female gender was also associated with earlier debut of arrhythmic events in the entire cohort of ARVC patients (39[14-64] vs 41 [21-75] years, p=0.034). Competitive athletes were overrepresented in the HTX-group (6/14) compared with the rest of the cohort (n=30/97, p=0.042). Conclusion: Patients who underwent HTX due to ARVC demonstrate excellent long-term survival in the Nordic ARVC registry. Female gender is associated with earlier disease onset and faster deterioration leading to HTX. Our data also indicate that vigorous exercise may be associated with disease progression.  
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