Please use this identifier to cite or link to this item: https://observatorio.fm.usp.br/handle/OPI/36268
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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.date.accessioned2020-06-01T15:01:38Z-
dc.date.available2020-06-01T15:01:38Z-
dc.date.issued2020
dc.identifier.citationCIRCULATION, v.141, n.17, p.1371-1383, 2020
dc.identifier.issn0009-7322
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/36268-
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.language.isoeng
dc.publisherLIPPINCOTT WILLIAMS & WILKINSeng
dc.relation.ispartofCirculation
dc.rightsopenAccesseng
dc.subjectcardiomyopathyeng
dc.subjecthypertrophiceng
dc.subjectgeneticseng
dc.subjectheart failureeng
dc.subjectprognosiseng
dc.subjectventricular dysfunctioneng
dc.subject.otherend-stage phaseeng
dc.subject.otherclinical-significanceeng
dc.subject.otheramerican-collegeeng
dc.subject.othertask-forceeng
dc.subject.otherprevalenceeng
dc.subject.otherassociationeng
dc.subject.otherimpairmenteng
dc.subject.otherguidelineseng
dc.subject.otherdiagnosiseng
dc.subject.otherspectrumeng
dc.titleHypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction Insights From the SHaRe Registryeng
dc.typearticleeng
dc.rights.holderCopyright LIPPINCOTT WILLIAMS & WILKINSeng
dc.identifier.doi10.1161/CIRCULATIONAHA.119.044366
dc.identifier.pmid32228044
dc.subject.wosCardiac & Cardiovascular Systemseng
dc.subject.wosPeripheral Vascular Diseaseeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
hcfmusp.author.externalMARSTRAND, Peter:Brigham & Womens Hosp, Cardiovasc Div, 75 Francis St, Boston, MA 02115 USA; Univ Hosp Copenhagen, Herlev Gentofte Hosp, Dept Cardiol, Copenhagen, Denmark
hcfmusp.author.externalHAN, Larry:Harvard TH Chan Sch Publ Hlth, Dept Biostat, Boston, MA USA
hcfmusp.author.externalDAY, Sharlene M.:Univ Penn, Dept Med, Philadelphia, PA 19104 USA
hcfmusp.author.externalOLIVOTTO, Iacopo:Careggi Univ Hosp, Cardiomyopathy Unit, Florence, Italy
hcfmusp.author.externalASHLEY, Euan A.:Stanford Ctr Inherited Heart Dis, Stanford, CA USA
hcfmusp.author.externalMICHELS, Michelle:Erasmus MC, Thoraxctr, Dept Cardiol, Rotterdam, Netherlands
hcfmusp.author.externalWITTEKIND, Samuel G.:Cincinnati Childrens Hosp Med Ctr, Heart Inst, Cincinnati, OH 45229 USA
hcfmusp.author.externalHELMS, Adam:Univ Michigan, Dept Internal Med, Ann Arbor, MI 48109 USA
hcfmusp.author.externalSABERI, Sara:Univ Michigan, Dept Internal Med, Ann Arbor, MI 48109 USA
hcfmusp.author.externalJACOBY, Daniel:Yale Univ, New Haven, CT USA
hcfmusp.author.externalWARE, James S.:Imperial Coll London, Natl Heart & Lung Inst, London, England; Imperial Coll London, Royal Brompton Cardiovasc Res Ctr, London, England
hcfmusp.author.externalCOLAN, Steven D.:Boston Childrens Hosp, Dept Cardiol, Boston, MA USA
hcfmusp.author.externalSEMSARIAN, Christopher:Univ Sydney, Agnes Ginges Ctr Mol Cardiol, Centenary Inst, Sydney, NSW, Australia
hcfmusp.author.externalINGLES, Jodie:Univ Sydney, Agnes Ginges Ctr Mol Cardiol, Centenary Inst, Sydney, NSW, Australia
hcfmusp.author.externalLAKDAWALA, Neal K.:Brigham & Womens Hosp, Cardiovasc Div, 75 Francis St, Boston, MA 02115 USA
hcfmusp.author.externalHO, Carolyn Y.:Brigham & Womens Hosp, Cardiovasc Div, 75 Francis St, Boston, MA 02115 USA
hcfmusp.description.beginpage1371
hcfmusp.description.endpage1383
hcfmusp.description.issue17
hcfmusp.description.volume141
hcfmusp.origemWOS
hcfmusp.origem.idWOS:000531420200003
hcfmusp.origem.id2-s2.0-85085066622
hcfmusp.publisher.cityPHILADELPHIAeng
hcfmusp.publisher.countryUSAeng
hcfmusp.relation.referenceAlfares AA, 2015, GENET MED, V17, P880, DOI [10.1038/gim.2014.205, 10.1038/gim.2015.16]eng
hcfmusp.relation.referenceBiagini E, 2005, J AM COLL CARDIOL, V46, P1543, DOI 10.1016/j.jacc.2005.04.062eng
hcfmusp.relation.referenceBiagini E, 2014, AM J CARDIOL, V114, P769, DOI 10.1016/j.amjcard.2014.05.065eng
hcfmusp.relation.referenceElliott PM, 2014, EUR HEART J, V35, P2733, DOI 10.1093/eurheartj/ehu284eng
hcfmusp.relation.referenceFernandez A, 2011, AM J CARDIOL, V108, P548, DOI 10.1016/j.amjcard.2011.03.083eng
hcfmusp.relation.referenceGalati G, 2016, CIRC-HEART FAIL, V9, DOI 10.1161/CIRCHEARTFAILURE.116.003090eng
hcfmusp.relation.referenceGersh BJ, 2011, CIRCULATION, V124, P2761, DOI 10.1161/CIR.0b013e318223e230eng
hcfmusp.relation.referenceHarris KM, 2006, CIRCULATION, V114, P216, DOI 10.1161/CIRCULATIONAHA.105.583500eng
hcfmusp.relation.referenceHo CY, 2018, CIRCULATION, V138, P1387, DOI 10.1161/CIRCULATIONAHA.117.033200eng
hcfmusp.relation.referenceMeghji Z, 2019, JAMA CARDIOL, V4, P237, DOI 10.1001/jamacardio.2019.0084eng
hcfmusp.relation.referenceNeubauer S, 2019, J AM COLL CARDIOL, V74, P2333, DOI 10.1016/j.jacc.2019.08.1057eng
hcfmusp.relation.referenceNguyen A, 2019, ANN THORAC SURG, V108, P723, DOI 10.1016/j.athoracsur.2019.03.026eng
hcfmusp.relation.referenceOlivotto I, 2010, AM J CARDIOL, V106, P261, DOI 10.1016/j.amjcard.2010.03.020eng
hcfmusp.relation.referenceParbhudayal RY, 2019, INT J CARDIOVAS IMAG, V35, P1089, DOI 10.1007/s10554-019-01563-3eng
hcfmusp.relation.referencePasqualucci D, 2015, CIRC-HEART FAIL, V8, P1014, DOI 10.1161/CIRCHEARTFAILURE.114.001843eng
hcfmusp.relation.referencePeduzzi P, 1995, J CLIN EPIDEMIOL, V48, P1503, DOI 10.1016/0895-4356(95)00048-8eng
hcfmusp.relation.referenceRichards S, 2015, GENET MED, V17, P405, DOI 10.1038/gim.2015.30eng
hcfmusp.relation.referenceSemsarian C, 2015, J AM COLL CARDIOL, V65, P1249, DOI 10.1016/j.jacc.2015.01.019eng
hcfmusp.relation.referenceSPIRITO P, 1987, AM J CARDIOL, V60, P123, DOI 10.1016/0002-9149(87)90998-2eng
hcfmusp.relation.referenceThaman R, 2005, HEART, V91, P920, DOI 10.1136/hrt.2003.031161eng
hcfmusp.relation.referenceTURINA J, 1986, EUR HEART J, V7, P685, DOI 10.1093/oxfordjournals.eurheartj.a062123eng
hcfmusp.relation.referenceWoo A, 2005, CIRCULATION, V111, P2033, DOI 10.1161/01.CIR.0000162460.36735.71eng
dc.description.indexMEDLINEeng
dc.identifier.eissn1524-4539
hcfmusp.citation.scopus41-
hcfmusp.scopus.lastupdate2022-04-29-
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