Philippe Charron1,2, Perry M Elliott3, Juan R Gimeno4,5, Alida L P Caforio6, Juan Pablo Kaski7, Luigi Tavazzi8, Michal Tendera9, Carole Maupain1, Cécile Laroche10, Pawel Rubis11, Ruxandra Jurcut12, Leonardo Calò13, Tiina M Heliö14, Gianfranco Sinagra15, Marija Zdravkovic16, Aušra Kavoliuniene17, Stephan B Felix18,19, Jacek Grzybowski20, Maria-Angela Losi21, Folkert W Asselbergs22,23, José Manuel García-Pinilla5,24, Joel Salazar-Mendiguchia25, Katarzyna Mizia-Stec26, Aldo P Maggioni10,27. 1. Centre de Référence des Maladies Cardiaques Héréditaires, Assistance Publique-Hôpitaux de Paris, ICAN, Hôpital Pitié-Salpêtrière, 47 Bvd de l'hôpital, 75013 Paris, France. 2. Université Versailles Saint Quentin & AP-HP, CESP, INSERM U1018, Hôpital Ambroise Paré, 9, Avenue Charles de Gaulle, 92100 Boulogne-Billancourt, France. 3. University College London and Inherited Cardiac Diseases Unit, Barts Heart Centre, St Bartholomew's Hospital, West Smithfield, EC1A 7BE London, UK. 4. Cardiac Department, Hospital Universitario Virgen de la Arrixaca, Ctra. Murcia-Cartagena s/n, 30120 El Palmar Murcia, Spain. 5. CIBER in Cardiovascular Diseases, Instituto Carlos III, Av. de Monforte de Lemos 5, 28029 Madrid, Spain. 6. Division of Cardiology, Department of Cardiological Thoracic and Vascular Sciences, University of Padova, via N Giustiniani 2, 35100 Padova, Italy. 7. Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, Great Ormond Street, WC1N 3JH London, UK. 8. GVM Care and Research, E.S. Health Science Foundation, Maria Cecilia Hospital, Via Corriera, 1, 48010 Cotignola, Italy. 9. Department of Cardiology and Structural Heart Diseases, School of Medicine in Katowice, Medical University of Silesia, Ziolowa Street 45/47, 40-635 Katowice, Poland. 10. EURObservational Research Programme, European Society of Cardiology, 2035 Route des colles, CS 80179 Biot, 06903 Sophia-Antipolis Cedex, France. 11. Department of Cardiac and Vascular Diseases, John Paul II Hospital, Institute of Cardiology, Jagiellonian University Medical College, Pradnicka street 80, 31-202 Krakow, Poland. 12. Institute of Emergency for Cardiovascular Diseases "Prof.dr.C.C.Iliescu", UMF "Carol Davila", Sos. Fundeni 258, 22328 Bucharest, Romania. 13. Policlinico Casilino, U.O. Cardiologia, Via Casilina, 1049, 00169 Roma, Italy. 14. Heart and Lung Center, Helsinki University Hospital and University of Helsinki, Haartmaninkatu 4, 00290 Helsinki, Finland. 15. Cardiovascular Department, Center for Cardiomyopathies, Azienda Sanitaria Universitaria Integrata, University of Trieste, Via P. Valdoni 7, 34100 Trieste, Italy. 16. University Hospital Medical Center Bezanijska kosa, Faculty of Medicine, University of Belgrade, Dragise Brasovana 13/8, 11077 Belgrade, Serbia. 17. Lithuanian University of Health Sciences, Eiveniu Str. 2, 50009 Kaunas, Lithuania. 18. Department for Internal Medicine B, University Medicine Greifswald, Ferdinand-Sauerbruch Strasse, 17475 Greifswald, Germany. 19. DZHK (German Centre for Cardiovascular Research), partner site Greifswald, Greifswald, Germany. 20. Department of Cardiomyopathies, Institute of Cardiology, Alpejska 42, 04-628 Warsaw, Poland. 21. Department of Advanced Biomedical Sciences, University Federico II of Naples, Via S. Pansini 5, 80131 Naples, Italy. 22. Department of Cardiology, Division Heart & Lungs, UMC Utrecht, 3508 GA Utrecht, The Netherlands. 23. Institute of Cardiovascular Science, Faculty of Population Health Sciences, University College London, Gower Street, WC1E 6BT London, UK. 24. Heart failure and familial cardiomyopathies division, Cardiology department, Hospital Universitario Virgen de la Victoria, IBIMA, Campus Universitario Teatinos, 29010 Málaga, Spain. 25. Cardiomyopathy, Heart Failure and Transplant Program, Hospital Universistari de Bellvitge, Heart Diseases Institute, Av. Feixa Llarga s/n, 08907 L'Hospitalet de Llobregat, Barcelona, Spain. 26. First Department of Cardiology, School of Medicine in Katowice, Medical University of Silesia, Ochojec, Ziolowa Street 45/47, 40-635 Katowice, Poland. 27. ANMCO Research Center, Via La Marmora, 34 50121 Firenze, Italy.
Abstract
Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. Methods and results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). Conclusion: By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.
Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry. Methods and results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001). Conclusion: By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.
Authors: Stefan Neubauer; Paul Kolm; Carolyn Y Ho; Raymond Y Kwong; Milind Y Desai; Sarahfaye F Dolman; Evan Appelbaum; Patrice Desvigne-Nickens; John P DiMarco; Matthias G Friedrich; Nancy Geller; Andrew R Harper; Petr Jarolim; Michael Jerosch-Herold; Dong-Yun Kim; Martin S Maron; Jeanette Schulz-Menger; Stefan K Piechnik; Kate Thomson; Cheng Zhang; Hugh Watkins; William S Weintraub; Christopher M Kramer Journal: J Am Coll Cardiol Date: 2019-11-12 Impact factor: 24.094
Authors: Maria Angela Losi; Massimo Imbriaco; Grazia Canciello; Filomena Pacelli; Carlo Di Nardo; Raffaella Lombardi; Raffaele Izzo; Costantino Mancusi; Andrea Ponsiglione; Serena Dell'Aversana; Alberto Cuocolo; Giovanni de Simone; Bruno Trimarco; Emanuele Barbato Journal: J Cardiovasc Transl Res Date: 2019-09-05 Impact factor: 4.132
Authors: Antoine Bondue; Eloisa Arbustini; Anna Bianco; Michele Ciccarelli; Dana Dawson; Matteo De Rosa; Nazha Hamdani; Denise Hilfiker-Kleiner; Benjamin Meder; Adelino F Leite-Moreira; Thomas Thum; Carlo G Tocchetti; Gilda Varricchi; Jolanda Van der Velden; Roddy Walsh; Stephane Heymans Journal: Cardiovasc Res Date: 2018-08-01 Impact factor: 10.787
Authors: Juan R Gimeno; Perry M Elliott; Luigi Tavazzi; Michal Tendera; Juan P Kaski; Cecile Laroche; Roberto Barriales-Villa; Petar Seferovic; Elena Biagini; Eloisa Arbustini; Luis R Lopes; Ales Linhart; Jens Mogensen; Albert Hagege; Maria A Espinosa; Aly Saad; Aldo P Maggioni; Alida L P Caforio; Philippe H Charron Journal: Eur Heart J Qual Care Clin Outcomes Date: 2021-03-15
Authors: Katarzyna Mizia-Stec; Philippe Charron; Juan Ramon Gimeno Blanes; Perry Elliott; Juan Pablo Kaski; Aldo P Maggioni; Luigi Tavazzi; Michał Tendera; Stephan B Felix; Fernando Dominguez; Natalia Ojrzynska; Maria-Angela Losi; Giuseppe Limongelli; Roberto Barriales-Villa; Petar M Seferovic; Elena Biagini; Maciej Wybraniec; Cecile Laroche; Alida L P Caforio Journal: Eur Heart J Cardiovasc Imaging Date: 2021-06-22 Impact factor: 6.875