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

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dc.contributor.authorAsselbergs, F. W.
dc.contributor.authorSammani, A.
dc.contributor.authorElliott, P.
dc.contributor.authorGimeno, J. R.
dc.contributor.authorTavazzi, L.
dc.contributor.authorTendera, M.
dc.contributor.authorKaski, J. P.
dc.contributor.authorMaggioni, A. P.
dc.contributor.authorRubis, P. P.
dc.contributor.authorJurcut, R.
dc.contributor.authorHeliö, T.
dc.contributor.authorCalò, L.
dc.contributor.authorSinagra, G.
dc.contributor.authorZdravkovic, M.
dc.contributor.authorOlivotto, I.
dc.contributor.authorKavoliūnienė, A.
dc.contributor.authorLaroche, C.
dc.contributor.authorCaforio, A.
dc.contributor.authorCharron, P.
dc.contributor.authorCardiomyopathy & Myocarditis Registry Investigators Group
dc.date.accessioned2021-09-07T08:47:06Z
dc.date.available2021-09-07T08:47:06Z
dc.date.issued2021-02
dc.descriptionScholarly articleen_US
dc.description.abstractAims: 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 ≤ 0.007), and less medication use (P ≤ 0.042). Outcome at 1 year was similar and predicted by NYHA class (HR 0.45; 95% CI [0.25-0.81]) and LVEF per % decrease (HR 1.05; 95% CI [1.02-1.08]. Throughout Europe, patients with FDCM received more genetic testing (47% vs. 8%, P < 0.01) and had higher genetic yield (55% vs. 22%, P < 0.01). 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.en_US
dc.description.sponsorshipAbbott Vascular Int. (2011–2021), Amgen Cardiovascular (2009–2018), AstraZeneca (2014–2021), Bayer AG (2009–2018), Boehringer Ingelheim (2009–2019), Boston Scientific (2009–2012), The Bristol Myers Squibb and Pfizer Alliance (2011–2019), Daiichi Sankyo Europe GmbH (2011–2020), The Alliance Daiichi Sankyo Europe GmbH and Eli Lilly and Company (2014–2017), Edwards (2016–2019), Gedeon Richter Plc. (2014–2016), Menarini Int. Op. (2009–2012), MSD‐Merck & Co. (2011–2014), Novartis Pharma AG (2014–2020), ResMed (2014–2016), Sanofi (2009–2011), SERVIER (2009–2021), and Vifor (2019–2022).en_US
dc.identifier.citationAsselbergs FW, Sammani A, Elliott P, et al. Differences between familial and sporadic dilated cardiomyopathy: ESC EORP Cardiomyopathy & Myocarditis registry. ESC Heart Fail. 2021;8(1):95-105. doi:10.1002/ehf2.13100en_US
dc.identifier.otherDOI: 10.1002/ehf2.13100
dc.identifier.urihttps://ir.unilag.edu.ng/handle/123456789/9562
dc.language.isoenen_US
dc.publisherWiley Periodicals, Inc.en_US
dc.subjectDilated cardiomyopathyen_US
dc.subjectEuropeen_US
dc.subjectFamilialen_US
dc.subjectGeneticen_US
dc.subjectPrognosisen_US
dc.subjectSporadicen_US
dc.subjectResearch Subject Categories::MEDICINEen_US
dc.titleDifferences between familial and sporadic dilated cardiomyopathy: ESC EORP Cardiomyopathy & Myocarditis registryen_US
dc.typeArticleen_US
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