Heart disease in Friedreich's ataxia: a clinical and echocardiographic study.

Twelve patients with Friedreich's ataxia (FA) were evaluated clinically and echocardiographically for evidence of heart disease. Electrocardiographic and echocardiographic abnormal findings were discovered in eight (67%) and seven (58%) children, respectively. A high incidence of cardiac involvement is well known in FA cases. Although the patient number in the present study is small, the findings are consistent with those in the literature. The most common pathology was asymmetric septal hypertrophy (ASH), followed by concentric left ventricular hypertrophy (CLVH) and dilated cardiomyopathy (DC).