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.