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The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population.
J Card Fail
October 2018
National Institute of Neurologic Diseases and Stroke, all of the National Institutes of Health, Bethesda, Maryland.
Background: Identification of a subclinical cardiomyopathy in pediatric patients with Friedreich's ataxia (FA) has not been well-described.
Methods: We performed echocardiography (Echo), cardiac magnetic resonance imaging (cMRI), and neurologic assessment in a cross-sectional analysis of 48 genetically confirmed FA subjects aged 9-17 years with moderate neurologic impairment but without a cardiovascular history. Echo- and cMRI-determined left ventricular mass were indexed (LVMI) to height in grams/m.