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  • Health status of cardiac genetic disease patients and their at-risk relatives

    Author(s)
    Ingles, Jodie
    Yeates, Laura
    Hunt, Lauren
    McGaughran, Julie
    Scuffham, Paul A
    Atherton, John
    Semsarian, Christopher
    Griffith University Author(s)
    Scuffham, Paul A.
    Hunt, Lauren
    Year published
    2013
    Metadata
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    Abstract
    Background: Health status is an important outcome measure that incorporates multiple dimensions of health, including symptoms, functional status, and psychosocial factors. While health status has been shown to be a predictor for hospital readmission, morbidity and mortality in the heart failure setting, there are limited data in cardiac genetic disease. We examined health status in a number of cardiac genetic disease groups compared to the general Australian population. Methods: A total of 409 individuals were assessed. Individualswith inherited cardiomyopathies [hypertrophic cardiomyopathy (HCM), familial dilated cardiomyopathy ...
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    Background: Health status is an important outcome measure that incorporates multiple dimensions of health, including symptoms, functional status, and psychosocial factors. While health status has been shown to be a predictor for hospital readmission, morbidity and mortality in the heart failure setting, there are limited data in cardiac genetic disease. We examined health status in a number of cardiac genetic disease groups compared to the general Australian population. Methods: A total of 409 individuals were assessed. Individualswith inherited cardiomyopathies [hypertrophic cardiomyopathy (HCM), familial dilated cardiomyopathy (FDC), arrhythmogenic right ventricular cardiomyopathy (ARVC)] and primary arrhythmogenic disorders [long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT)], as well as their first-degree relatives, completed the Medical Outcomes Survey Short Form-36 (SF-36). The physical andmental component scores (PCS andMCS) and SF-6D utility scorewere assessed. Results: Patients with HCM (pb0.001), FDC (pb0.05), and CPVT (pb0.05) were found to have a significantly lower PCS, while patients with LQTS (pb0.01) had a lower MCS. Individuals at risk of HCM (pb0.0001) and genotype positive-phenotype negative HCM patients (pb0.01) both had a higher PCS and utility scores compared to the clinically affected HCMpopulation. Individuals at risk of LQTS had significantly higher PCS than those with a clinical diagnosis of LQTS (pb0.05) and similarly individuals at risk of FDC had significantly higher PCS than FDC patients (pb0.05). In HCM, female gender (p=0.002), presence of co-morbidities (pb0.0001) and higher NYHA functional class (pb0.0001) were predictors of a lower PCS. Conclusions: Patients with a clinical diagnosis of a genetic heart disease have an impaired health status, related to both physical andmental function. Clinical management strategies in such patient groups need to consider health status as an important outcome measure.
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    Journal Title
    International Journal of Cardiology
    Volume
    165
    Issue
    3
    DOI
    https://doi.org/10.1016/j.ijcard.2011.08.083
    Subject
    Medical and Health Sciences not elsewhere classified
    Cardiorespiratory Medicine and Haematology
    Public Health and Health Services
    Publication URI
    http://hdl.handle.net/10072/45407
    Collection
    • Journal articles

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