Cardiac Remodeling in Subclinical Hypertrophic CardiomyopathyThe VANISH Randomized Clinical Trial
dc.contributor | Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP | |
dc.contributor.author | VISSING, Christoffer Rasmus | |
dc.contributor.author | RAJA, Anna Axelsson | |
dc.contributor.author | DAY, Sharlene M. | |
dc.contributor.author | RUSSELL, Mark W. | |
dc.contributor.author | ZAHKA, Kenneth | |
dc.contributor.author | LEVER, Harry M. | |
dc.contributor.author | PEREIRA, Alexandre C. | |
dc.contributor.author | COLAN, Steven D. | |
dc.contributor.author | MARGOSSIAN, Renee | |
dc.contributor.author | MURPHY, Anne M. | |
dc.contributor.author | CANTER, Charles | |
dc.contributor.author | BACH, Richard G. | |
dc.contributor.author | WHEELER, Matthew T. | |
dc.contributor.author | ROSSANO, Joseph W. | |
dc.contributor.author | OWENS, Anjali T. | |
dc.contributor.author | BENSON, Lee | |
dc.contributor.author | MESTRONI, Luisa | |
dc.contributor.author | TAYLOR, Matthew R. G. | |
dc.contributor.author | PATEL, Amit R. | |
dc.contributor.author | WILMOT, Ivan | |
dc.contributor.author | THRUSH, Philip | |
dc.contributor.author | SOSLOW, Jonathan H. | |
dc.contributor.author | BECKER, Jason R. | |
dc.contributor.author | SEIDMAN, Christine E. | |
dc.contributor.author | LAKDAWALA, Neal K. | |
dc.contributor.author | CIRINO, Allison L. | |
dc.contributor.author | MCMURRAY, John J. V. | |
dc.contributor.author | MACRAE, Calum A. | |
dc.contributor.author | SOLOMON, Scott D. | |
dc.contributor.author | BUNDGAARD, Henning | |
dc.contributor.author | ORAV, E. John | |
dc.contributor.author | HO, Carolyn Y. | |
dc.contributor.groupauthor | Valsartan Attenuating Dis Evolutio | |
dc.date.accessioned | 2024-04-05T19:46:26Z | |
dc.date.available | 2024-04-05T19:46:26Z | |
dc.date.issued | 2023 | |
dc.description.abstract | Importance Valsartan has shown promise in attenuating cardiac remodeling in patients with early-stage sarcomeric hypertrophic cardiomyopathy (HCM). Genetic testing can identify individuals at risk of HCM in a subclinical stage who could benefit from therapies that prevent disease progression.Objective To explore the potential for valsartan to modify disease development, and to characterize short-term phenotypic progression in subclinical HCM.Design, Setting, and Participants The multicenter, double-blind, placebo-controlled Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy (VANISH) randomized clinical trial was conducted from April 2014 to July 2019 at 17 sites in 4 countries (Brazil, Canada, Denmark, and the US), with 2 years of follow-up. The prespecified exploratory VANISH cohort studied here included sarcomere variant carriers with subclinical HCM and early phenotypic manifestations (reduced E ' velocity, electrocardiographic abnormalities, or an increased left ventricular [LV] wall thickness [LVWT] to cavity diameter ratio) but no LV hypertrophy (LVH). Data were analyzed between March and December 2022.Interventions Treatment with placebo or valsartan (80 mg/d for children weighing <35 kg, 160 mg/d for children weighing >= 35 kg, or 320 mg/d for adults aged >= 18 years).Main Outcomes and Measures The primary outcome was a composite z score incorporating changes in 9 parameters of cardiac remodeling (LV cavity volume, LVWT, and LV mass; left atrial [LA] volume; E ' velocity and S ' velocity; and serum troponin and N-terminal prohormone of brain natriuretic peptide levels).Results This study included 34 participants, with a mean (SD) age of 16 (5) years (all were White). A total of 18 participants (8 female [44%] and 10 male [56%]) were randomized to valsartan and 16 (9 female [56%] and 7 male [44%]) were randomized to placebo. No statistically significant effects of valsartan on cardiac remodeling were detected (mean change in composite z score compared with placebo: -0.01 [95% CI, -0.29 to 0.26]; P = .92). Overall, 2-year phenotypic progression was modest, with only a mild increase in LA volume detected (increased by 3.5 mL/m(2) [95% CI, 1.4-6.0 mL/m(2)]; P = .002). Nine participants (26%) had increased LVWT, including 6 (18%) who developed clinically overt HCM. Baseline LA volume index (LAVI; 35 vs 28 mL/m(2); P = .01) and average interventricular septum thickness (8.5 vs 7.0 mm; P = .009) were higher in participants who developed HCM.Conclusions and Relevance In this exploratory cohort, valsartan was not proven to slow progression of subclinical HCM. Minimal changes in markers of cardiac remodeling were observed, although nearly one-fifth of patients developed clinically overt HCM. Transition to disease was associated with greater baseline interventricular septum thickness and LAVI. These findings highlight the importance of following sarcomere variant carriers longitudinally and the critical need to improve understanding of factors that drive disease penetrance and progression. | eng |
dc.description.index | MEDLINE | |
dc.description.index | PubMed | |
dc.description.index | Scopus | |
dc.description.index | Dimensions | |
dc.description.index | WoS | |
dc.description.sponsorship | NIH [1P50HL112349] | |
dc.identifier.citation | JAMA CARDIOLOGY, v.8, n.11, 2023 | |
dc.identifier.doi | 10.1001/jamacardio.2023.2808 | |
dc.identifier.eissn | 2380-6591 | |
dc.identifier.issn | 2380-6583 | |
dc.identifier.uri | https://observatorio.fm.usp.br/handle/OPI/59295 | |
dc.language.iso | eng | |
dc.publisher | AMER MEDICAL ASSOC | eng |
dc.relation.ispartof | Jama Cardiology | |
dc.rights | restrictedAccess | eng |
dc.rights.holder | Copyright AMER MEDICAL ASSOC | eng |
dc.subject.other | disease | eng |
dc.subject.other | penetrance | eng |
dc.subject.other | evolution | eng |
dc.subject.other | death | eng |
dc.subject.other | risk | eng |
dc.subject.wos | Cardiac & Cardiovascular Systems | eng |
dc.title | Cardiac Remodeling in Subclinical Hypertrophic CardiomyopathyThe VANISH Randomized Clinical Trial | eng |
dc.type | article | eng |
dc.type.category | original article | eng |
dc.type.version | publishedVersion | eng |
dspace.entity.type | Publication | |
hcfmusp.affiliation.country | Dinamarca | |
hcfmusp.affiliation.country | Escócia | |
hcfmusp.affiliation.country | Canadá | |
hcfmusp.affiliation.country | Estados Unidos | |
hcfmusp.affiliation.countryiso | us | |
hcfmusp.affiliation.countryiso | dk | |
hcfmusp.affiliation.countryiso | ca | |
hcfmusp.affiliation.countryiso | gb | |
hcfmusp.author.external | VISSING, Christoffer Rasmus:Rigshosp, Copenhagen Univ Hosp, Dept Cardiol, Copenhagen, Denmark; Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.author.external | RAJA, Anna Axelsson:Rigshosp, Copenhagen Univ Hosp, Dept Cardiol, Copenhagen, Denmark | |
hcfmusp.author.external | DAY, Sharlene M.:Univ Penn, Perelman Sch Med, Dept Med, Div Cardiovasc Med, Philadelphia, PA USA | |
hcfmusp.author.external | RUSSELL, Mark W.:Univ Michigan, Ann Arbor, MI USA | |
hcfmusp.author.external | ZAHKA, Kenneth:Cleveland Clin Fdn, Cleveland, OH USA | |
hcfmusp.author.external | LEVER, Harry M.:Cleveland Clin Fdn, Cleveland, OH USA | |
hcfmusp.author.external | COLAN, Steven D.:Boston Childrens Hosp, Dept Cardiol, Boston, MA USA | |
hcfmusp.author.external | MARGOSSIAN, Renee:Boston Childrens Hosp, Dept Cardiol, Boston, MA USA | |
hcfmusp.author.external | MURPHY, Anne M.:Johns Hopkins Univ, Div Pediat Cardiol, Sch Med, Dept Pediat, Baltimore, MD USA | |
hcfmusp.author.external | CANTER, Charles:Washington Univ, Sch Med, St. Louis, MO USA | |
hcfmusp.author.external | BACH, Richard G.:Washington Univ, Sch Med, St. Louis, MO USA | |
hcfmusp.author.external | WHEELER, Matthew T.:Stanford Univ, Dept Med, Sch Med, Div Cardiovasc Med, Stanford, CA USA | |
hcfmusp.author.external | ROSSANO, Joseph W.:Childrens Hosp Philadelphia, Philadelphia, PA USA | |
hcfmusp.author.external | OWENS, Anjali T.:Univ Penn, Perelman Sch Med, Dept Med, Div Cardiovasc Med, Philadelphia, PA USA | |
hcfmusp.author.external | BENSON, Lee:Toronto Hosp Sick Children, Toronto, ON, Canada | |
hcfmusp.author.external | MESTRONI, Luisa:Univ Colorado Anschutz Med Campus, Dept Neurol & Neurosurg, Aurora, CO USA | |
hcfmusp.author.external | TAYLOR, Matthew R. G.:Univ Colorado Anschutz Med Campus, Dept Neurol & Neurosurg, Aurora, CO USA | |
hcfmusp.author.external | PATEL, Amit R.:Univ Virginia Hlth Syst, Dept Med, Cardiovasc Div, Charlottesville, VA USA | |
hcfmusp.author.external | WILMOT, Ivan:Cincinnati Childrens Hosp, Med Ctr, Heart Inst, Cincinnati, OH USA | |
hcfmusp.author.external | THRUSH, Philip:Ann & Robert H Lurie Childrens Hosp Chicago, Chicago, IL USA | |
hcfmusp.author.external | SOSLOW, Jonathan H.:Vanderbilt Univ, Med Ctr, Nashville, TN USA | |
hcfmusp.author.external | BECKER, Jason R.:Univ Pittsburgh, Div Cardiol, Sch Med, Pittsburgh, PA USA; UPMC, Pittsburgh, PA USA | |
hcfmusp.author.external | SEIDMAN, Christine E.:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA; Howard Hughes Med Inst, Chevy Chase, MD USA | |
hcfmusp.author.external | LAKDAWALA, Neal K.:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.author.external | CIRINO, Allison L.:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.author.external | MCMURRAY, John J. V.:Univ Glasgow, British Heart Fdn Cardiovasc Res Ctr, Glasgow City, Scotland | |
hcfmusp.author.external | MACRAE, Calum A.:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.author.external | SOLOMON, Scott D.:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.author.external | BUNDGAARD, Henning:Rigshosp, Copenhagen Univ Hosp, Dept Cardiol, Copenhagen, Denmark | |
hcfmusp.author.external | ORAV, E. John:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.author.external | HO, Carolyn Y.:Harvard Med Sch, Brigham & Womens Hosp, Boston, MA USA | |
hcfmusp.citation.scopus | 1 | |
hcfmusp.contributor.author-fmusphc | ALEXANDRE DA COSTA PEREIRA | |
hcfmusp.description.issue | 11 | |
hcfmusp.description.volume | 8 | |
hcfmusp.origem | WOS | |
hcfmusp.origem.dimensions | pub.1163881687 | |
hcfmusp.origem.pubmed | 37672268 | |
hcfmusp.origem.scopus | 2-s2.0-85172304468 | |
hcfmusp.origem.wos | WOS:001085840800001 | |
hcfmusp.publisher.city | CHICAGO | eng |
hcfmusp.publisher.country | USA | eng |
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hcfmusp.scopus.lastupdate | 2024-04-12 | |
relation.isAuthorOfPublication | 415ce7ca-65c1-4699-b6f4-19dae8b03849 | |
relation.isAuthorOfPublication.latestForDiscovery | 415ce7ca-65c1-4699-b6f4-19dae8b03849 |