Myocardial perfusion in Friedreich's ataxia: assessment by dipyridamole thallium-201 imaging

In order to assess myocardial perfusion in patients with Friedreich's ataxia, we carried out dipyridamole-thallium imaging in 13 cardiologically asymptomatic patients (aged 16 to 39; mean age 24) with various degrees of left ventricular hypertrophy; all showed electrocardiographic ST and/or T wave abnormalities before scintigraphy. After dosing with dipyridamole-201 thallium, we found: a completely reversible perfusion defect in the apical and a partially reversible defect in the posterolateral wall of the left ventricle in 1 case; a partially reversible perfusion defect in the anterior and posterior walls of the left ventricle in 1 case; and a persistent anterolateral perfusion defect in 1 case. The remaining 10 patients showed no scintigraphic abnormalities. The fact that only 1 of our patients showed a completely reversible perfusion defect, possibly consistent with regional myocardial ischemia, suggests that ischemia does not play a major role in the pathogenesis of the cardiac involvement in Friedreich's ataxia.