دورية أكاديمية

Baseline Characteristics of the VANISH Cohort.

التفاصيل البيبلوغرافية
العنوان: Baseline Characteristics of the VANISH Cohort.
المؤلفون: Axelsson Raja A; Copenhagen University Hospital Rigshospitalet, Denmark (A.A.R., H.B.)., Shi L; New England Research Institutes, Watertown, MA (L.S.)., Day SM; University of Michigan, Ann Arbor (S.M.D., M.R.)., Russell M; University of Michigan, Ann Arbor (S.M.D., M.R.)., Zahka K; Cleveland Clinic, OH (K.Z., H.L.)., Lever H; Cleveland Clinic, OH (K.Z., H.L.)., Colan SD; Boston Children's Hospital, MA (S.D.C., R.M.)., Margossian R; Boston Children's Hospital, MA (S.D.C., R.M.)., Hall EK; Yale University, New Haven, CT (E.K.H.)., Becker J; Vanderbilt University Medical Center, Nashville, TN (J.B.)., Jefferies JL; Cincinnati Children's Hospital Medical Center, OH (J.L.J.)., Patel AR; University of Chicago, IL (A.R.P.)., Choudhury L; Northwestern University, Chicago, IL (L.C.)., Murphy AM; Johns Hopkins University School of Medicine, Baltimore, MD (A.M.M.)., Canter C; Washington University School of Medicine, St. Louis, MO (C.C., R.B.)., Bach R; Washington University School of Medicine, St. Louis, MO (C.C., R.B.)., Taylor M; University of Colorado Anschutz Medical Campus, Aurora (M.T., L.M.)., Mestroni L; University of Colorado Anschutz Medical Campus, Aurora (M.T., L.M.)., Wheeler MT; Stanford University School of Medicine, Palo Alto, CA (M.T.W.)., Benson L; Toronto Hospital for Sick Children, ON, Canada (L.B.)., Owens AT; University of Pennsylvania Perelman School of Medicine, Philadelphia (A.T.O.)., Rossano J; Children's Hospital of Philadelphia, PA (J.R., K.Y.L.)., Lin KY; Children's Hospital of Philadelphia, PA (J.R., K.Y.L.)., Pahl E; Ann & Robert H. Lurie Children's Hospital of Chicago, IL (E.P.)., Pereira AC; Heart Institute, University of São Paulo Medical School (Instituto do Coração), Brazil (A.C.P.)., Bundgaard H; Copenhagen University Hospital Rigshospitalet, Denmark (A.A.R., H.B.)., Lewis GD; Massachusetts General Hospital, Boston (G.D.L.)., Vargas JD; MedStar Georgetown University Hospital, National Institutes of Health, Bethesda, MD (J.D.V.)., Cirino AL; Brigham and Women's Hospital, Boston, MA (A.L.C., C.A.M., S.D.S., E.J.O., E.B., C.Y.H.)., McMurray JJV; University of Glasgow, Glasgow, UK (J.J.V.M.)., MacRae CA; Brigham and Women's Hospital, Boston, MA (A.L.C., C.A.M., S.D.S., E.J.O., E.B., C.Y.H.)., Solomon SD; Brigham and Women's Hospital, Boston, MA (A.L.C., C.A.M., S.D.S., E.J.O., E.B., C.Y.H.)., Orav EJ; Brigham and Women's Hospital, Boston, MA (A.L.C., C.A.M., S.D.S., E.J.O., E.B., C.Y.H.)., Braunwald E; Brigham and Women's Hospital, Boston, MA (A.L.C., C.A.M., S.D.S., E.J.O., E.B., C.Y.H.)., Ho CY; Brigham and Women's Hospital, Boston, MA (A.L.C., C.A.M., S.D.S., E.J.O., E.B., C.Y.H.).
المصدر: Circulation. Heart failure [Circ Heart Fail] 2019 Dec; Vol. 12 (12), pp. e006231. Date of Electronic Publication: 2019 Dec 09.
نوع المنشور: Journal Article; Multicenter Study; Randomized Controlled Trial; Research Support, N.I.H., Extramural
اللغة: English
بيانات الدورية: Publisher: Lippincott Williams & Wilkins Country of Publication: United States NLM ID: 101479941 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1941-3297 (Electronic) Linking ISSN: 19413289 NLM ISO Abbreviation: Circ Heart Fail Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Hagerstown, MD : Lippincott Williams & Wilkins
مواضيع طبية MeSH: Mutation*, Angiotensin II Type 1 Receptor Blockers/*therapeutic use , Cardiomyopathy, Hypertrophic/*drug therapy , Sarcomeres/*genetics , Valsartan/*therapeutic use, Adolescent ; Adult ; Angiotensin II Type 1 Receptor Blockers/adverse effects ; Brazil ; Canada ; Cardiomyopathy, Hypertrophic/diagnosis ; Cardiomyopathy, Hypertrophic/genetics ; Cardiomyopathy, Hypertrophic/physiopathology ; Child ; Denmark ; Disease Progression ; Double-Blind Method ; Female ; Genetic Predisposition to Disease ; Humans ; Male ; Middle Aged ; Phenotype ; Recovery of Function ; Time Factors ; Treatment Outcome ; United States ; Valsartan/adverse effects ; Young Adult
مستخلص: Background: The VANISH trial (Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy) targeted young sarcomeric gene mutation carriers with early-stage hypertrophic cardiomyopathy (HCM) to test whether valsartan can modify disease progression. We describe the baseline characteristics of the VANISH cohort and compare to previous trials evaluating angiotensin receptor blockers.
Methods: Applying a randomized, double-blinded, placebo-controlled design, 178 participants with nonobstructive HCM (age, 23.3±10.1 years; 61% men) were randomized in the primary cohort and 34 (age, 16.5±4.9 years; 50% men) in the exploratory cohort of sarcomeric mutation carriers without left ventricular hypertrophy.
Results: In the primary cohort, maximal left ventricular wall thickness was 17±4 mm for adults and Z score 7.0±4.5 for children. Nineteen percent had late gadolinium enhancement on cardiac magnetic resonance. Mean peak oxygen consumption was 33 mL/kg per minute, and 92% of participants were New York Heart Association functional class I. New York Heart Association class II was associated with older age, MYH7 variants, and more prominent imaging abnormalities. Six previous trials of angiotensin receptor blockers in HCM enrolled a median of 24 patients (range, 19-133) with mean age of 51.2 years; 42% of patients were in New York Heart Association class ≥II, and sarcomeric mutations were not required.
Conclusions: The VANISH cohort is much larger, younger, less heterogeneous, and has less advanced disease than prior angiotensin receptor blocker trials in HCM. Participants had relatively normal functional capacity and mild HCM features. New York Heart Association functional class II symptoms were associated with older age, more prominent imaging abnormalities, and MYH7 variants, suggesting both phenotype and genotype contribute to disease manifestations.
Clinical Trial Registration: URL: https://www.clinicaltrials.gov. Unique identifier: NCT01912534.
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معلومات مُعتمدة: P20 HL101408 United States HL NHLBI NIH HHS; P50 HL112349 United States HL NHLBI NIH HHS
فهرسة مساهمة: Keywords: angiotension receptor blocker; cardiomyopathy, hypertrophic; randomized controlled trial
سلسلة جزيئية: ClinicalTrials.gov NCT01912534
المشرفين على المادة: 0 (Angiotensin II Type 1 Receptor Blockers)
80M03YXJ7I (Valsartan)
تواريخ الأحداث: Date Created: 20191210 Date Completed: 20200616 Latest Revision: 20201209
رمز التحديث: 20221213
مُعرف محوري في PubMed: PMC7219518
DOI: 10.1161/CIRCHEARTFAILURE.119.006231
PMID: 31813281
قاعدة البيانات: MEDLINE
الوصف
تدمد:1941-3297
DOI:10.1161/CIRCHEARTFAILURE.119.006231