Progression of hypertrophic into a dilated left ventricle in Friedreich's ataxia

G Ital Cardiol. 1988 Jul;18(7):615-8.


The authors describe the case of a girl who developed Friedreich's ataxia at, approximately, the age of 7, with evidence of cardiac involvement being detected by electrocardiography and echocardiography at a later date. Cardiac function was moderately impaired and remained unchanged for a number of years, during which a picture of hypertrophic left ventricle seemed to be firmly established. Later still, however, the cardiac situation shifted gradually toward a hypokinetic form of the disease, with a progressive thinning of the interventricular septum and posterior wall of the left ventricle, associated enlargement of the ventricular chambers and increasingly severe hypokinesia leading to repeated episodes of heart failure.

Publication types

  • Case Reports

MeSH terms

  • Cardiomyopathy, Dilated / diagnosis
  • Cardiomyopathy, Dilated / etiology*
  • Cardiomyopathy, Hypertrophic / complications*
  • Cardiomyopathy, Hypertrophic / diagnosis
  • Child
  • Echocardiography
  • Electrocardiography
  • Female
  • Friedreich Ataxia / complications*
  • Humans