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Multinational Registry on Cardiomyopathies
Study Questions:
What are characteristics of patients with a diagnosis of hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM)?
Methods:
The EURObservational Research Programme (EORP) Cardiomyopathy Registry was created by the European Society of Cardiology Working Group on Myocardial and Pericardial Disease to collect information on patients with a diagnosis of cardiomyopathy, more specifically four different subtypes: HCM, DCM, ARVC, and RCM. This prospective observational multicenter registry enrolled 3,208 patients (HCM [n = 1,739], DCM [n = 1,260], ARVC [n = 143], and RCM [n = 66]). A pilot phase of the registry was created to validate the structure and quality of the data in adults only. A long-term phase was then created to look at adults and pediatric patients with cardiomyopathies, as well as patients with suspected or biopsy-proven myocarditis.
Results:
The median age at enrollment was 55 years and there was male predominance for all cardiomyopathies except RCM (p < 0.001). The most common diagnosis was HCM (54.2%), DCM (39.3%), ARVC (4.4%), and RCM (2.1%). A familial disease was seen in 38.9%; the highest proportion was seen in HCM (48.5%) and ARVC (40.6%). A history of ventricular tachycardia was seen most in patients with ARVC (39.2%) and least in RCM (1.5%). Different imaging modalities were used for different subtypes where magnetic resonance imaging was used the most in patients with ARVC and the least in DCM. Goal-directed medical therapy was assessed and beta-blockers were used consistently (80.6% in all patients). Compared with the long-term phase, patients in the pilot phase had a more frequent rate of familial disease (p < 0.001) and more frequently received defibrillators. Of the four geographical areas included in the study, Southern Europe saw a higher prevalence of familial disease (p < 0.001), which was more frequently diagnosed through family screening (p < 0.001).
Conclusions:
This is the first multinational European registry in cardiomyopathies. The registry helps provide a basis for the need of a multidisciplinary approach for patients with a diagnosis of cardiomyopathy.
Perspective:
This registry demonstrates that physicians still use a range of diagnostic tests to assess cardiomyopathies and to help guide treatment. The registry also helps solidify the prevalence of inherited cardiomyopathies and the importance of referring relatives for evaluation as well. The registry also helped demonstrate geographical differences in the prevalence and management of these cardiomyopathies based on more expert centers seeing more familial disease.
Keywords: Arrhythmogenic Right Ventricular Dysplasia, Biopsy, Cardiomyopathies, Cardiomyopathy, Dilated, Cardiomyopathy, Hypertrophic, Cardiomyopathy, Restrictive, Defibrillators, Diagnostic Tests, Routine, Heart Failure, Magnetic Resonance Imaging, Myocarditis, Myocardium, Registries, Tachycardia, Ventricular
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