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

. 2021 Mar 15 ; 7 (2) : 134-142.

Language English Country Great Britain, England Media print

Document type Journal Article, Research Support, Non-U.S. Gov't

Grant support
MR/T005181/1 Medical Research Council - United Kingdom
MR/T024062/1 Medical Research Council - United Kingdom

AIMS: The European Society of Cardiology (ESC) European Observational Research Programme (EORP) Cardiomyopathy Registry is a prospective multinational registry of consecutive patients with cardiomyopathies. The objective of this report is to describe the short-term outcomes of adult patients (≥18 years old). METHODS AND RESULTS: Out of 3208 patients recruited, follow-up data at 1 year were obtained in 2713 patients (84.6%) [1420 with hypertrophic (HCM); 1105 dilated (DCM); 128 arrhythmogenic right ventricular (ARVC); and 60 restrictive (RCM) cardiomyopathies]. Improvement of symptoms (dyspnoea, chest pain, and palpitations) was globally observed over time (P < 0.05 for each). Additional invasive procedures were performed: prophylactic implantation of implantable cardioverter-defibrillator (ICD) (5.2%), pacemaker (1.2%), heart transplant (1.1%), ablation for atrial or ventricular arrhythmia (0.5% and 0.1%). Patients with atrial fibrillation increased from 28.7% to 32.2% of the cohort. Ventricular arrhythmias (VF/ventricular tachycardias) in ICD carriers (primary prevention) at 1 year were more frequent in ARVC, then in DCM, HCM, and RCM (10.3%, 8.2%, 7.5%, and 0%, respectively). Major cardiovascular events (MACE) occurred in 29.3% of RCM, 10.5% of DCM, 5.3% of HCM, and 3.9% of ARVC (P < 0.001). MACE were more frequent in index patients compared to relatives (10.8% vs. 4.4%, P < 0.001), more frequent in East Europe centres (13.1%) and least common in South Europe (5.3%) (P < 0.001). Subtype of cardiomyopathy, geographical region, and proband were predictors of MACE on multivariable analysis. CONCLUSIONS: Despite symptomatic improvement, patients with cardiomyopathies remain prone to major clinical events in the short term. Outcomes were different not only according to cardiomyopathy subtypes but also in relatives vs. index patients, and according to European regions.

2nd Department of Medicine Department of Cardiovascular Medicine 1st Faculty of Medicine Charles University and General University Hospital Prague Czech Republic

Assistance Publique Hôpitaux de Paris Hôpital Européen Georges Pompidou Cardiology Department Sorbonne Paris Cité INSERM U970 Paris France

Centre for Inherited Cardiovascular Diseases Great Ormond Street Hospital Great Ormond Street London WC1N 3JH UK

Department of Cardiology and Structural Heart Diseases School of Medicine in Katowice Medical University of Silesia Ziolowa Street 45 47 40 635 Katowice Poland

Department of Cardiology Odense University Hospital 5000 Odense C Denmark

Department of Cardiology University of Bologna S Orsola Malpighi Hospital Via Giuseppe Massarenti 9 40138 Bologna Italy

Department of Cardiology Zagazig University Hospital Zagazig Egypt

Division of Cardiology Department of Cardiological Thoracic and Vascular Sciences University of Padova via N Giustiniani 2 35100 Padova Italy

EURObservational Research Programme European Society of Cardiology 2035 Route des colles CS 80179 Biot 06903 Sophia Antipolis Cedex France

Fondazione IRCCS Policlinico San Matteo Viale Camillo Golgi 19 27100 Pavia Italy

GVM Care and Research E S Health Science Foundation Maria Cecilia Hospital Via Corriera 1 48010 Cotignola Italy

Heart Failure Center University of Belgrade School of Medicine Belgrade University Medical Center Koste Todorovica 8 11 000 Belgrade Serbia

Inherited Cardiac Disease Unit Complejo Hospitalario Universitario de A Coruña Calle As Xubias 84 15006 A Coruña Spain

Inherited Cardiac Disease Unit Hospital Clínico Universitario Virgen de la Arrixaca Ctra Murcia Cartagena s n El Palmar 30120 Murcia Spain

Inherited Cardiac Disease Unit Hospital General Universitario Gregorio Marañón 28007 Madrid Spain

Serviço de Cardiologia Hospital Garcia de Orta E P E Av Torrado da Silva 2805 267 Almada Portugal

Sorbonne Université Centre de Référence des Maladies Cardiaques Héréditaires Assistance Publique Hôpitaux de Paris ICAN Inserm UMR1166 Hôpital Pitié Salpêtrière Paris France

University College London and Inherited Cardiac Diseases Unit St Bartholomew's Hospital West Smithfield Paul O'Gorman Building 72 Huntley St London WC1E 6AG UK

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