TY - JOUR
T1 - European cardiomyopathy pilot registry
T2 - EURObservational research programme of the European society of cardiology
AU - on behalf of the EORP Cardiomyopathy Registry Pilot Investigators
AU - Elliott, Perry
AU - Charron, Philippe
AU - Blanes, Juan Ramon Gimeno
AU - Tavazzi, Luigi
AU - Tendera, Michal
AU - Konté, Marème
AU - Laroche, Cécile
AU - Maggioni, Aldo P.
AU - Anastasakis, Aris
AU - Arbustini, Eloisa
AU - Asselbergs, Folkert W.
AU - Axelsson, Anna
AU - Brito, Dulce
AU - Caforio, Alida L.P.
AU - Carr-White, Gerald
AU - Czekaj, Agata
AU - Damy, Thibaud
AU - Devoto, Emmanuela
AU - Favalli, Valentina
AU - Findlay, Iain
AU - Garcia-Pavia, Pablo
AU - Hagège, Albert
AU - Heliö, Tiina
AU - Iliceto, Sabino
AU - Isnard, Richard
AU - Jansweijer, Joeri A.
AU - Limongelli, Giuseppe
AU - Linhart, Ales
AU - Cuenca, David López
AU - Mansencal, Nicolas
AU - McKeown, Pascal
AU - Mogensen, Jens
AU - Mohiddin, Saidi A.
AU - Monserrat, Lorenzo
AU - Olivotto, Iacopo
AU - Rapezzi, Claudio
AU - Rigopoulos, A. G.
AU - Rosmini, Stefania
AU - Pfeiffer, Barbara
AU - Wicks, Eleanor
AU - Podzimkova, J.
AU - Kuchynka, P.
AU - Palecek, T.
AU - Bundgaard, H.
AU - Thune, J. J.
AU - Kumme, A.
AU - Due Vestergaard, L.
AU - Wilde, A.A.M.
AU - Pinto, Y.
AU - Van Der Heijden, J. F.
PY - 2016
Y1 - 2016
N2 - Aims: Cardiomyopathies are a heterogeneous group of disorders associated with premature death due to ventricular arrhythmia or heart failure. The purpose of this study was to examine the characteristics of patients enrolled in the pilot phase of the EURObservational Research Programme (EORP) cardiomyopathy registry. Methods and results: Between 1 December 2012 and 30 November 2013, four cardiomyopathy phenotypes were studied: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). Twenty-seven centres in 12 countries participated; 1115 patients were enrolled. The commonest cardiomyopathy was HCM (n = 681), followed by DCM (n = 346), ARVC (n = 59), and RCM (n = 29); 423 patients (46.4% of those reported) had familial disease; and 56 (5.0%) had rare disease phenocopies. Median age at enrolment and diagnosis was 54 [interquartile range (IQR), 42-64] and 46 years (IQR, 32-58), respectively; fewer patients with ARVC and more with RCM were diagnosed in the upper age quartile (P, 0.0001). There was a male predominance for all cardiomyopathies except RCM (P = 0.0023). Most patients were in New York Heart Association functional class I (n = 813) at enrolment; 139 (12.5%) reported syncope, most frequently in ARVC (P = 0.0009). Five hundred and seven (45.5%) patients underwent cardiac magnetic resonance imaging, 117 (10.6%) endomyocardial biopsy, and 462 (41.4%) genetic testing with a causative mutation reported in 236 individuals (51.1%). 1026 patients (92.0%) were receiving drug therapy; 316 (28.3%) had received an implantable cardioverter defibrillator (highest proportion in ARVC, P, 0.0001). Conclusion: This pilot study shows that services for patients with cardiomyopathy are complex, requiring access to a large range of invasive and non-invasive investigations and involvement of multidisciplinary teams. Treatment regimens are equally multifaceted and show that patients are likely to need long-term follow-up in close liaison with expert centres.
AB - Aims: Cardiomyopathies are a heterogeneous group of disorders associated with premature death due to ventricular arrhythmia or heart failure. The purpose of this study was to examine the characteristics of patients enrolled in the pilot phase of the EURObservational Research Programme (EORP) cardiomyopathy registry. Methods and results: Between 1 December 2012 and 30 November 2013, four cardiomyopathy phenotypes were studied: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). Twenty-seven centres in 12 countries participated; 1115 patients were enrolled. The commonest cardiomyopathy was HCM (n = 681), followed by DCM (n = 346), ARVC (n = 59), and RCM (n = 29); 423 patients (46.4% of those reported) had familial disease; and 56 (5.0%) had rare disease phenocopies. Median age at enrolment and diagnosis was 54 [interquartile range (IQR), 42-64] and 46 years (IQR, 32-58), respectively; fewer patients with ARVC and more with RCM were diagnosed in the upper age quartile (P, 0.0001). There was a male predominance for all cardiomyopathies except RCM (P = 0.0023). Most patients were in New York Heart Association functional class I (n = 813) at enrolment; 139 (12.5%) reported syncope, most frequently in ARVC (P = 0.0009). Five hundred and seven (45.5%) patients underwent cardiac magnetic resonance imaging, 117 (10.6%) endomyocardial biopsy, and 462 (41.4%) genetic testing with a causative mutation reported in 236 individuals (51.1%). 1026 patients (92.0%) were receiving drug therapy; 316 (28.3%) had received an implantable cardioverter defibrillator (highest proportion in ARVC, P, 0.0001). Conclusion: This pilot study shows that services for patients with cardiomyopathy are complex, requiring access to a large range of invasive and non-invasive investigations and involvement of multidisciplinary teams. Treatment regimens are equally multifaceted and show that patients are likely to need long-term follow-up in close liaison with expert centres.
KW - Arrhythmogenic right ventricular
KW - Cardiomyopathy
KW - Dilated
KW - Hypertrophic
KW - Registry
KW - Restrictive
UR - http://www.scopus.com/inward/record.url?scp=84964575491&partnerID=8YFLogxK
U2 - https://doi.org/10.1093/eurheartj/ehv497
DO - https://doi.org/10.1093/eurheartj/ehv497
M3 - Article
C2 - 26409010
SN - 0195-668X
VL - 37
SP - 164
EP - 173
JO - European Heart journal
JF - European Heart journal
IS - 2
ER -