European Cardiomyopathy Pilot Registry: EURObservational Research Programme of the European Society of Cardiology
Key result
HCM dominates a European cardiomyopathy registry where 92% of patients receive medical therapy.
- n=1,115
Why the study?
Cardiomyopathies are a heterogeneous group of disorders associated with premature death from ventricular arrhythmia or heart failure, prompting examination of characteristics in the pilot phase of the EORP registry.
Key Points
- This study aimed to examine the characteristics of patients enrolled in the pilot phase of the cardiomyopathy registry.
- Studied four cardiomyopathy phenotypes: hypertrophic, dilated, arrhythmogenic right ventricular, and restrictive cardiomyopathy.
- Utilized data from 1115 patients enrolled across 27 centers in 12 countries between December 2012 and November 2013.
- Conducted assessments including cardiac MRI, endomyocardial biopsy, and genetic testing.
- Hypertrophic cardiomyopathy was the most common phenotype with 681 cases (61%), followed by dilated (346), arrhythmogenic (59), and restrictive (29).
- 46.4% of patients had familial disease, and 5.0% had rare disease phenocopies.
- 92.0% of patients were on drug therapy; 28.3% had received an implantable cardioverter defibrillator.
Study Design
Type
Observational (n=1,115)
Multicenter
Yes
PICO
P
Population
1,115 patients with hypertrophic, dilated, arrhythmogenic right ventricular, or restrictive cardiomyopathy enrolled across 27 centers in 12 countries.
E
Exposure / Comparator
Cardiomyopathy
O
Primary Outcome
Patient characteristics and clinical management
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Key expert perspectives
Captured external expert commentary on this paper, strongest first. Original sources are linked where available.
“This is the first time that we have a snapshot of practice in cardiomyopathies across different European countries with diverse healthcare systems. The literature is dominated by studies from a small number of large centres and none of the research has been so representative of Europe as a whole.”
Implication
Highlights European cardiomyopathy phenotypes and therapy patterns; leaves open phenotype-specific outcome associations for prospective study.
Abstract
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.
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Cite This Study
Elliott et al. (2015) conducted an observational in Cardiomyopathies (n=1,115). Cardiomyopathy was evaluated on Patient characteristics and clinical management. Among 1,115 patients in a European cardiomyopathy registry, hypertrophic cardiomyopathy was most common (n=681), 92.0% received drug therapy, and 28.3% had an implantable defibrillator.