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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorMARSTRAND, Peter
dc.contributor.authorHAN, Larry
dc.contributor.authorDAY, Sharlene M.
dc.contributor.authorOLIVOTTO, Iacopo
dc.contributor.authorASHLEY, Euan A.
dc.contributor.authorMICHELS, Michelle
dc.contributor.authorPEREIRA, Alexandre C.
dc.contributor.authorWITTEKIND, Samuel G.
dc.contributor.authorHELMS, Adam
dc.contributor.authorSABERI, Sara
dc.contributor.authorJACOBY, Daniel
dc.contributor.authorWARE, James S.
dc.contributor.authorCOLAN, Steven D.
dc.contributor.authorSEMSARIAN, Christopher
dc.contributor.authorINGLES, Jodie
dc.contributor.authorLAKDAWALA, Neal K.
dc.contributor.authorHO, Carolyn Y.
dc.identifier.citationCIRCULATION, v.141, n.17, p.1371-1383, 2020
dc.description.abstractBackground: The term ""end stage"" has been used to describe hypertrophic cardiomyopathy (HCM) with left ventricular systolic dysfunction (LVSD), defined as occurring when left ventricular ejection fraction is <50%. The prognosis of HCM-LVSD has reportedly been poor, but because of its relative rarity, the natural history remains incompletely characterized. Methods: Data from 11 high-volume HCM specialty centers making up the international SHaRe Registry (Sarcomeric Human Cardiomyopathy Registry) were used to describe the natural history of patients with HCM-LVSD. Cox proportional hazards models were used to identify predictors of prognosis and incident development. Results: From a cohort of 6793 patients with HCM, 553 (8%) met the criteria for HCM-LVSD. Overall, 75% of patients with HCM-LVSD experienced clinically relevant events, and 35% met the composite outcome (all-cause death [n=128], cardiac transplantation [n=55], or left ventricular assist device implantation [n=9]). After recognition of HCM-LVSD, the median time to composite outcome was 8.4 years. However, there was substantial individual variation in natural history. Significant predictors of the composite outcome included the presence of multiple pathogenic/likely pathogenic sarcomeric variants (hazard ratio [HR], 5.6 [95% CI, 2.3-13.5]), atrial fibrillation (HR, 2.6 [95% CI, 1.7-3.5]), and left ventricular ejection fraction <35% (HR, 2.0 [95% CI, 1.3-2.8]). The incidence of new HCM-LVSD was approximate to 7.5% over 15 years. Significant predictors of developing incident HCM-LVSD included greater left ventricular cavity size (HR, 1.1 [95% CI, 1.0-1.3] and wall thickness (HR, 1.3 [95% CI, 1.1-1.4]), left ventricular ejection fraction of 50% to 60% (HR, 1.8 [95% CI, 1.2, 2.8]-2.8 [95% CI, 1.8-4.2]) at baseline evaluation, the presence of late gadolinium enhancement on cardiac magnetic resonance imaging (HR, 2.3 [95% CI, 1.0-4.9]), and the presence of a pathogenic/likely pathogenic sarcomeric variant, particularly in thin filament genes (HR, 1.5 [95% CI, 1.0-2.1] and 2.5 [95% CI, 1.2-5.1], respectively). Conclusions: HCM-LVSD affects approximate to 8% of patients with HCM. Although the natural history of HCM-LVSD was variable, 75% of patients experienced adverse events, including 35% experiencing a death equivalent an estimated median time of 8.4 years after developing systolic dysfunction. In addition to clinical features, genetic substrate appears to play a role in both prognosis (multiple sarcomeric variants) and the risk for incident development of HCM-LVSD (thin filament variants).eng
dc.description.sponsorshipMyoKardia, Inc
dc.description.sponsorshipNational Institutes of HealthUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [R01 GRANT11572784, 1P50HL112349, 1U01HL117006]
dc.description.sponsorshipDanish Heart Foundation [17-R115-A7532-22065]
dc.description.sponsorshipElite Research Travel Grant from the Danish Ministry of Higher Education and Science
dc.description.sponsorshipFUKAP Foundation
dc.description.sponsorshipTorben og Alice Frimodts Fond
dc.description.sponsorshipAugustinus fonden
dc.description.sponsorshipKnud Hojgaards fond
dc.description.sponsorshipItalian Ministry of HealthMinistry of Health, Italy [RF-2013-02356787, NET-2011-02347173]
dc.description.sponsorshipMedical Research Council (United Kingdom)Medical Research Council UK (MRC)
dc.description.sponsorshipNational Health and Medical Research Council Practitioner FellowshipNational Health and Medical Research Council of Australia [1154992]
dc.description.sponsorshipTuscany Registry of Sudden Cardiac Death project (FAS-Salute 2014, Regione Toscana)
dc.description.sponsorshipWellcome TrustWellcome Trust [107469/Z/15/Z]
dc.subjectheart failureeng
dc.subjectventricular dysfunctioneng
dc.subject.otherend-stage phaseeng
dc.titleHypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction Insights From the SHaRe Registryeng
dc.rights.holderCopyright LIPPINCOTT WILLIAMS & WILKINSeng
dc.subject.wosCardiac & Cardiovascular Systemseng
dc.subject.wosPeripheral Vascular Diseaseeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng, Peter:Brigham & Womens Hosp, Cardiovasc Div, 75 Francis St, Boston, MA 02115 USA; Univ Hosp Copenhagen, Herlev Gentofte Hosp, Dept Cardiol, Copenhagen, Denmark, Larry:Harvard TH Chan Sch Publ Hlth, Dept Biostat, Boston, MA USA, Sharlene M.:Univ Penn, Dept Med, Philadelphia, PA 19104 USA, Iacopo:Careggi Univ Hosp, Cardiomyopathy Unit, Florence, Italy, Euan A.:Stanford Ctr Inherited Heart Dis, Stanford, CA USA, Michelle:Erasmus MC, Thoraxctr, Dept Cardiol, Rotterdam, Netherlands, Samuel G.:Cincinnati Childrens Hosp Med Ctr, Heart Inst, Cincinnati, OH 45229 USA, Adam:Univ Michigan, Dept Internal Med, Ann Arbor, MI 48109 USA, Sara:Univ Michigan, Dept Internal Med, Ann Arbor, MI 48109 USA, Daniel:Yale Univ, New Haven, CT USA, James S.:Imperial Coll London, Natl Heart & Lung Inst, London, England; Imperial Coll London, Royal Brompton Cardiovasc Res Ctr, London, England, Steven D.:Boston Childrens Hosp, Dept Cardiol, Boston, MA USA, Christopher:Univ Sydney, Agnes Ginges Ctr Mol Cardiol, Centenary Inst, Sydney, NSW, Australia, Jodie:Univ Sydney, Agnes Ginges Ctr Mol Cardiol, Centenary Inst, Sydney, NSW, Australia, Neal K.:Brigham & Womens Hosp, Cardiovasc Div, 75 Francis St, Boston, MA 02115 USA, Carolyn Y.:Brigham & Womens Hosp, Cardiovasc Div, 75 Francis St, Boston, MA 02115 USA
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LIM/13 - Laboratório de Genética e Cardiologia Molecular

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