Juan R Gimeno1, Perry M Elliott2, Luigi Tavazzi3, Michal Tendera4, Juan P Kaski5, Cecile Laroche6, Roberto Barriales-Villa7, Petar Seferovic8, Elena Biagini9, Eloisa Arbustini10, Luis R Lopes11, Ales Linhart12, Jens Mogensen13, Albert Hagege14, Maria A Espinosa15, Aly Saad16, Aldo P Maggioni3,6, Alida L P Caforio17, Philippe H Charron18. 1. Inherited Cardiac Disease Unit, Hospital Clínico Universitario Virgen de la Arrixaca, Ctra. Murcia-Cartagena s/n. El Palmar, 30120 Murcia, Spain. 2. 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. 3. GVM Care and Research, E.S. Health Science Foundation, Maria Cecilia Hospital, Via Corriera, 1, 48010 Cotignola, Italy. 4. Department of Cardiology and Structural Heart Diseases, School of Medicine in Katowice, Medical University of Silesia, Ziolowa Street 45/47, 40-635 Katowice, Poland. 5. Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK. 6. EURObservational Research Programme, European Society of Cardiology, 2035 Route des colles, CS 80179 Biot, 06903 Sophia-Antipolis Cedex, France. 7. Inherited Cardiac Disease Unit, Complejo Hospitalario Universitario de A Coruña, Calle As Xubias, 84, 15006 A Coruña, Spain. 8. Heart Failure Center, University of Belgrade School of Medicine, Belgrade University Medical Center, Koste Todorovica 8, 11 000, Belgrade, Serbia. 9. Department of Cardiology, University of Bologna/S. Orsola-Malpighi Hospital, Via Giuseppe Massarenti, 9, 40138 Bologna, Italy. 10. Fondazione IRCCS Policlinico San Matteo, Viale Camillo Golgi, 19, 27100 Pavia, Italy. 11. Serviço de Cardiologia, Hospital Garcia de Orta. E:P.E. Av. Torrado da Silva, 2805-267 Almada, Portugal. 12. Second Department of Medicine - Department of Cardiovascular Medicine, 1st Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic. 13. Department of Cardiology, Odense University Hospital, 5000 Odense C, Denmark. 14. Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Cardiology Department, Sorbonne Paris Cité, INSERM U970, Paris, France. 15. Inherited Cardiac Disease Unit, Hospital General Universitario Gregorio Marañón, 28007 Madrid, Spain. 16. Department of Cardiology, Zagazig University Hospital, Zagazig, Egypt. 17. Division of Cardiology, Department of Cardiological Thoracic and Vascular Sciences, University of Padova, via N Giustiniani 2, 35100 Padova, Italy. 18. Sorbonne Université, Centre de Référence des Maladies Cardiaques Héréditaires, Assistance Publique-Hôpitaux de Paris, ICAN, Inserm, UMR1166, Hôpital Pitié-Salpêtrière, Paris, France.
Abstract
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. Published on behalf of the European Society of Cardiology. All rights reserved.
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. Published on behalf of the European Society of Cardiology. All rights reserved.
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