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The Subclinical Cardiomyopathy of Friedreich's Ataxia in a Pediatric Population

Identification of a subclinical cardiomyopathy in a pediatric patients with Friedreich's ataxia (FA) has not been well-described. This group therefore 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. They found that echo showed that left ventricle measurements exceeded age-based normal values in 85% of subjects and cMRI-determined that these measurements correlated with depression of both diastolic and systolic tissue Doppler velocity as well as increased early diastolic Doppler flow velocity/tissue velocity ratio, a marker of elevated left ventricle filling pressure. Similar associations were found with echo left ventricle mass. Depressed left ventricle relaxation and increased left ventricle stiffness were observed in 88% and 71% of subjects despite a normal left ventricle ejection fraction in almost all cases. concentric remodeling and concentric hypertrophy were present in 40% and 44% of the study group. The group concludes that a subclinical hypertrophic cardiomyopathy is common in pediatric FA patients and cardiac hypertrophy is associated with both diastolic and systolic dysfunction.

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