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Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction: Insights From the SHaRe Registry

Circulation (New York, N.Y.), 2020-04, Vol.141 (17), p.1371-1383 [Peer Reviewed Journal]

2020 by the American College of Cardiology Foundation and the American Heart Association, Inc. ;2020 The Authors. 2020 ;ISSN: 0009-7322 ;EISSN: 1524-4539 ;DOI: 10.1161/CIRCULATIONAHA.119.044366 ;PMID: 32228044

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  • Title:
    Hypertrophic Cardiomyopathy With Left Ventricular Systolic Dysfunction: Insights From the SHaRe Registry
  • Author: Marstrand, Peter ; Han, Larry ; Day, Sharlene M ; Olivotto, Iacopo ; Ashley, Euan A ; Michels, Michelle ; Pereira, Alexandre C ; Wittekind, Samuel G ; Helms, Adam ; Saberi, Sara ; Jacoby, Daniel ; Ware, James S ; Colan, Steven D ; Semsarian, Christopher ; Ingles, Jodie ; Lakdawala, Neal K ; Ho, Carolyn Y
  • Subjects: Adult ; Cardiomyopathy, Hypertrophic - diagnosis ; Cardiomyopathy, Hypertrophic - diagnostic imaging ; Cardiomyopathy, Hypertrophic - epidemiology ; Cardiomyopathy, Hypertrophic - physiopathology ; Female ; Humans ; Incidence ; Male ; Middle Aged ; Original s ; Prognosis ; Registries ; Risk Factors ; Ventricular Dysfunction, Left - diagnosis ; Ventricular Dysfunction, Left - diagnostic imaging ; Ventricular Dysfunction, Left - epidemiology ; Ventricular Dysfunction, Left - physiopathology
  • Is Part Of: Circulation (New York, N.Y.), 2020-04, Vol.141 (17), p.1371-1383
  • Description: BACKGROUND: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 ≈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 ≈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).
  • Publisher: United States: by the American College of Cardiology Foundation and the American Heart Association, Inc
  • Language: English
  • Identifier: ISSN: 0009-7322
    EISSN: 1524-4539
    DOI: 10.1161/CIRCULATIONAHA.119.044366
    PMID: 32228044
  • Source: GFMER Free Medical Journals
    MEDLINE
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