TY - JOUR
T1 - The Cardiomyopathy Registry of the EURObservational Research Programme of the European Society of Cardiology
T2 - baseline data and contemporary management of adult patients with cardiomyopathies
AU - Charron, Philippe
AU - Elliott, Perry M
AU - Gimeno, Juan R
AU - Caforio, Alida L P
AU - Kaski, Juan Pablo
AU - Tavazzi, Luigi
AU - Tendera, Michal
AU - Maupain, Carole
AU - Laroche, Cécile
AU - Rubis, Pawel
AU - Jurcut, Ruxandra
AU - Calò, Leonardo
AU - Heliö, Tiina M
AU - Sinagra, Gianfranco
AU - Zdravkovic, Marija
AU - Kavoliuniene, Aušra
AU - Felix, Stephan B
AU - Grzybowski, Jacek
AU - Losi, Maria-Angela
AU - Asselbergs, Folkert W
AU - García-Pinilla, José Manuel
AU - Salazar-Mendiguchia, Joel
AU - Mizia-Stec, Katarzyna
AU - Maggioni, Aldo P
N1 - Funding Information:
Conflict of interest: P.C. reports personal fees from Boehringer, Novartis, Amicus, and MyoKardia, non-financial support from Genzyme, grants and personal fees from Sanofi and Shire, personal fees and nonfinancial support from Servier, outside the submitted work. P.E. reports grants and personal fees from Sanofi Genzyme, personal fees from Pfizer, MyoKardia, and Shire, outside the submitted work. L.T. reports personal fees from Servier, CVIE Therapeutics, and Cardiorentis, outside the submitted work. M.T. reports personal fees from Bayer, Kowa, Janssen-Cilag, Perfuse Group, Servier, Celyad, grants from EU Framework Program VII andPolish National Center for Research and Development, outside the submitted work. A.P.M. reports personal fees from Novartis, Cardiorentis, Bayer and Fresenius, outside the submitted work. T.M.H. reports having worked as clinical consultant at Blueprint Genetics and being Member of Sanofi Genzyme Fabry advisory board in Finland,
Publisher Copyright:
© The Author 2017.
PY - 2018/5/21
Y1 - 2018/5/21
N2 - 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.
AB - 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.
KW - Journal Article
KW - Restrictive
KW - Cardiomyopathy
KW - Hypertrophic
KW - Registry
KW - Dilated
KW - Arrhythmogenic right ventricular
KW - Prospective Studies
KW - Age Factors
KW - Humans
KW - Middle Aged
KW - Arrhythmogenic Right Ventricular Dysplasia/diagnosis
KW - Male
KW - Cardiomyopathy, Hypertrophic/diagnosis
KW - Magnetic Resonance Imaging
KW - Cardiomyopathy, Restrictive/diagnosis
KW - Defibrillators
KW - Europe/epidemiology
KW - Cardiomyopathy, Dilated/diagnosis
KW - Adult
KW - Female
KW - Registries
KW - Cardiomyopathies/diagnosis
KW - Disease Management
UR - http://www.scopus.com/inward/record.url?scp=85043587731&partnerID=8YFLogxK
U2 - 10.1093/eurheartj/ehx819
DO - 10.1093/eurheartj/ehx819
M3 - Article
C2 - 29378019
SN - 0195-668X
VL - 39
SP - 1784
EP - 1793
JO - European Heart Journal
JF - European Heart Journal
IS - 20
ER -