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Quality of Life and Exercise Capacity in Early Stage and Subclinical Hypertrophic Cardiomyopathy: A Secondary Analysis of the VANISH Trial.
Ireland, Catherine G; Burstein, Danielle S; Day, Sharlene M; Axelsson Raja, Anna; Russell, Mark W; Zahka, Kenneth G; Pereira, Alexandre; Canter, Charles E; Bach, Richard G; Wheeler, Matthew T; Rossano, Joseph W; Owens, Anjali T; Bundgaard, Henning; Mestroni, Luisa; Taylor, Matthew R G; Patel, Amit R; Wilmot, Ivan; Soslow, Jonathan H; Becker, Jason R; Giverts, Ilya; Orav, E John; Claggett, Brian; Lin, Kimberly Y; Ho, Carolyn Y.
Afiliación
  • Ireland CG; Brigham and Women's Hospital, Harvard Medical School, Boston, MA (C.G.I., E.J.O., B.C., C.Y.H.).
  • Burstein DS; University of Vermont Larner School of Medicine, Burlington (D.S.B.).
  • Day SM; University of Pennsylvania Perelman School of Medicine, Philadelphia (S.M.D., A.T.O.).
  • Axelsson Raja A; Copenhagen University Hospital, Rigshospitalet, Denmark (A.A.R., H.B.).
  • Russell MW; University of Michigan, Ann Arbor (M.W.R.).
  • Zahka KG; Cleveland Clinic Foundation, OH (K.G.Z.).
  • Pereira A; Heart Institute, University of Sao Paulo Medical School, Brazil (A.P.).
  • Canter CE; Washington University School of Medicine, St. Louis, MO (C.E.C., R.G.B.).
  • Bach RG; Washington University School of Medicine, St. Louis, MO (C.E.C., R.G.B.).
  • Wheeler MT; Stanford University School of Medicine, Palo Alto, CA (M.T.W.).
  • Rossano JW; Children's Hospital of Philadelphia, PA (J.W.R., K.Y.L.).
  • Owens AT; University of Pennsylvania Perelman School of Medicine, Philadelphia (S.M.D., A.T.O.).
  • Bundgaard H; Copenhagen University Hospital, Rigshospitalet, Denmark (A.A.R., H.B.).
  • Mestroni L; University of Colorado Anschutz Medical Campus, Aurora (L.M., M.R.G.T.).
  • Taylor MRG; University of Colorado Anschutz Medical Campus, Aurora (L.M., M.R.G.T.).
  • Patel AR; University of Virginia, Charlottesville (A.R.P.).
  • Wilmot I; Cincinnati Children's Hospital Medical Center, OH (I.W.).
  • Soslow JH; Vanderbilt University Medical Center, Nashville, TN (J.H.S.).
  • Becker JR; University of Pittsburgh, PA (J.R.B.).
  • Giverts I; Massachusetts General Hospital, Boston (I.G.).
  • Orav EJ; Brigham and Women's Hospital, Harvard Medical School, Boston, MA (C.G.I., E.J.O., B.C., C.Y.H.).
  • Claggett B; Brigham and Women's Hospital, Harvard Medical School, Boston, MA (C.G.I., E.J.O., B.C., C.Y.H.).
  • Lin KY; Children's Hospital of Philadelphia, PA (J.W.R., K.Y.L.).
  • Ho CY; Brigham and Women's Hospital, Harvard Medical School, Boston, MA (C.G.I., E.J.O., B.C., C.Y.H.).
Circ Heart Fail ; 17(8): e011663, 2024 Aug.
Article en En | MEDLINE | ID: mdl-39087355
ABSTRACT

BACKGROUND:

The health-related quality of life (HRQOL) and cardiopulmonary exercise testing (CPET) performance of individuals with subclinical and early stage hypertrophic cardiomyopathy (HCM) have not been systematically studied. Improved understanding will inform the natural history of HCM and factors influencing well-being.

METHODS:

VANISH trial (Valsartan for Attenuating Disease Evolution in Early Sarcomeric HCM) participants with early stage sarcomeric HCM (primary analysis cohort) and subclinical HCM (sarcomere variant without left ventricular hypertrophy comprising the exploratory cohort) who completed baseline and year 2 HRQOL assessment via the pediatric quality of life inventory and CPET were studied. Metrics correlating with baseline HRQOL and CPET performance were identified. The impact of valsartan treatment on these measures was analyzed in the early stage cohort.

RESULTS:

Two hundred participants were included 166 with early stage HCM (mean age, 23±10 years; 40% female; 97% White; and 92% New York Heart Association class I) and 34 subclinical sarcomere variant carriers (mean age, 16±5 years; 50% female; and 100% White). Baseline HRQOL was good in both cohorts, although slightly better in subclinical HCM (composite pediatric quality of life score 84.6±10.6 versus 90.2±9.8; P=0.005). Both cohorts demonstrated mildly reduced functional status (mean percent predicted peak oxygen uptake 73±16 versus 78±12 mL/kg per minute; P=0.18). Percent predicted peak oxygen uptake and peak oxygen pulse correlated with HRQOL. Valsartan improved physical HRQOL in early stage HCM (adjusted mean change in pediatric quality of life score +4.1 versus placebo; P=0.01) but did not significantly impact CPET performance.

CONCLUSIONS:

Functional capacity can be impaired in young, healthy people with early stage HCM, despite New York Heart Association class I status and good HRQOL. Peak oxygen uptake was similarly decreased in subclinical HCM despite normal left ventricular wall thickness and excellent HRQOL. Valsartan improved physical pediatric quality of life scores but did not significantly impact CPET performance. Further studies are needed for validation and to understand how to improve patient experience. REGISTRATION URL https//www.clinicaltrials.gov; Unique identifier NCT01912534.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Calidad de Vida / Cardiomiopatía Hipertrófica / Tolerancia al Ejercicio / Prueba de Esfuerzo / Valsartán Límite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: Circ Heart Fail Asunto de la revista: ANGIOLOGIA / CARDIOLOGIA Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Calidad de Vida / Cardiomiopatía Hipertrófica / Tolerancia al Ejercicio / Prueba de Esfuerzo / Valsartán Límite: Adolescent / Adult / Child / Female / Humans / Male Idioma: En Revista: Circ Heart Fail Asunto de la revista: ANGIOLOGIA / CARDIOLOGIA Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos