Prospective follow-up in various subtypes of cardiomyopathies: Insights from the EORP Cardiomyopathy Registry of the ESC

Abstract

The EORP Cardiomyopathy Registry is a prospective, observational, multinational registry of consecutive patients with cardiomyopathies. The objective of this report is to describe the outcomes at one year of follow-up of adult patients (> 18 years old) enrolled in the registry. A total of 3208 patients [median age: 55.0 (43.0;64.0) years, males: 65.1%] were recruited at baseline. Follow-up data at 1 year were obtained in 2,713 patients (84.6%), including 1420 with hypertrophic (HCM), 1,105 dilated (DCM), 128 arrhythmogenic right ventricular (ARVC) and 60 restrictive cardiomyopathy (RCM) ( Fig. 1 ). Improvement of symptoms (NYHA, chest pain, syncope) was globally observed over time ( P < 0.001 for each). Additional invasive therapeutics were performed during follow-up: implantation of ICD (primary prevention) ( N = 109 patients, 5.2%), pacemaker ( N = 28, 1.2%), heart transplant ( N = 30, 1,1%), ablation for atrial or ventricular arrhythmia (0.5% & 0.1%). The proportion of patients with history of AF increased from baseline to FU in 3.6% (from 28.2% to 31.8%). ICD therapy at 1 year was delivered more frequently in ARVC then in DCM, HCM and RCM (11.4%, 9.0%; 8.1%, 0% respectively for primary prevention). Major cardiovascular events (MACE) occurred in 29.3% of RCM, 10.5% of DCM, 7.9% of ARVC and 5.3% of HCM. MACE were globally higher in index patients compared to relatives (10.8% vs. 4.4%, P < 0.001). When considering geographical areas, MACE were higher in East Europe (13.1%) and lower in South Europe (5.3%) (univariate); heart transplant was higher in West Europe (2.40%) and lower in South Europe (0.25%) (univariate). Despite symptomatic improvement in most cases, there is still a significant burden of arrhythmic and heart failure events in patients with cardiomyopathies. Outcomes were different not only according to cardiomyopathy subtypes but also in relatives versus index patients.