Differences between familial and sporadic dilated cardiomyopathy : ESC EORP Cardiomyopathy & Myocarditis registry

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Cardiomyopathy Myocarditis , Asselbergs , F W , Sammani , A , Elliott , P , Heliö , T & Charron , P 2021 , ' Differences between familial and sporadic dilated cardiomyopathy : ESC EORP Cardiomyopathy & Myocarditis registry ' , ESC Heart Failure , vol. 8 , no. 1 , pp. 95-105 . https://doi.org/10.1002/ehf2.13100

Title: Differences between familial and sporadic dilated cardiomyopathy : ESC EORP Cardiomyopathy & Myocarditis registry
Author: Cardiomyopathy Myocarditis; Asselbergs, Folkert W.; Sammani, Arjan; Elliott, Perry; Heliö, Tiina; Charron, Philippe
Other contributor: University of Helsinki, HUS Heart and Lung Center




Date: 2021-02
Language: eng
Number of pages: 11
Belongs to series: ESC Heart Failure
ISSN: 2055-5822
DOI: https://doi.org/10.1002/ehf2.13100
URI: http://hdl.handle.net/10138/325859
Abstract: Aims Dilated cardiomyopathy (DCM) is a complex disease where genetics interplay with extrinsic factors. This study aims to compare the phenotype, management, and outcome of familial DCM (FDCM) and non-familial (sporadic) DCM (SDCM) across Europe. Methods and results Patients with DCM that were enrolled in the prospective ESC EORP Cardiomyopathy & Myocarditis Registry were included. Baseline characteristics, genetic testing, genetic yield, and outcome were analysed comparing FDCM and SDCM; 1260 adult patients were studied (238 FDCM, 707 SDCM, and 315 not disclosed). Patients with FDCM were younger (P <0.01), had less severe disease phenotype at presentation (P <0.02), more favourable baseline cardiovascular risk profiles (P Conclusions We observed that FDCM and SDCM have significant differences at baseline but similar short-term prognosis. Whether modification of associated cardiovascular risk factors provide opportunities for treatment remains to be investigated. Our results also show a prevalent role of genetics in FDCM and a non-marginal yield in SDCM although genetic testing is largely neglected in SDCM. Limited genetic testing and heterogeneity in panels provides a scaffold for improvement of guideline adherence.
Subject: Dilated cardiomyopathy
Sporadic
Familial
Genetic
Prognosis
Europe
EUROBSERVATIONAL RESEARCH-PROGRAM
FREQUENCY
PILOT
3121 General medicine, internal medicine and other clinical medicine
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