HEART-DISEASE IN FRIEDREICHS ATAXIA - A CLINICAL AND ECHOCARDIOGRAPHIC STUDY

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