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- W2953511786 abstract "Background: Cardiomyopathy is common in the setting of muscular dystrophy (MD). As life expectancy has improved with treatment of neuromuscular-associated respiratory failure, patients surviving into adulthood are at risk of developing cardiomyopathy. However, MD-associated cardiomyopathy is often underdiagnosed and undertreated. Methods and results: We conducted a retrospective review of patients seen at the St Vincent's Neuromuscular Cardiology and Heart Failure Clinics between February 2018–February 2019. Twenty six patients were identified, with diagnoses of Duchenne MD (n = 18), Becker MD (n = 3), limb girdle MD (n = 2), congenital MD (n = 2) and Emery Dreifuss MD (n = 1). Most patients were male (n = 24, 84.6%) and non-ambulant, with mean age 24.7 ± 7.1 years. Transthoracic echocardiogram had been performed in almost all patients (n = 24, 93.2%) in the past 12 months. A left ventricular ejection fraction (LVEF) ≤55% was identified in a third of patients (n = 8, 34.8%). More severe LV dysfunction with LVEF ≤40% was identified in 2 patients (7.7%). The majority of patients were prescribed an ACE inhibitor (n = 22, 84.6%), while nearly half of the cohort were prescribed a beta blocker (n = 12, 46.1%). Analysis of global longitudinal strain was performed in only a small proportion of patients (n = 5, 4.2%) due to limited image quality in this patient cohort. Conclusion: Muscular dystrophy is associated with significant cardiomyopathy, which may shorten life expectancy. This unique population can benefit from careful cardiac surveillance with echocardiography to detect cardiomyopathy and allow early pharmacological treatment." @default.
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- W2953511786 date "2019-01-01" @default.
- W2953511786 modified "2023-09-27" @default.
- W2953511786 title "Prevalence and Management of Cardiomyopathy in Adult Patients with Muscular Dystrophies" @default.
- W2953511786 doi "https://doi.org/10.1016/j.hlc.2019.06.122" @default.
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