The heart in Friedreich's ataxia

Seventeen patients with Freidreich's ataxia were investigated for cardiac abnormalities using non-invasive methods. Clinical examination, routine chest radiographs and electrocardiograms were a poor guide to underlying heart involvement but using continuous ambulatory electrocardiographic monitoring, echocardiography and isotope ventriculography all patients were found to have abnormalities, even when the neurological signs were minimal.