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Echocardiographic Findings in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

H.F. Gattiker
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
A. Davignon
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
A. Bozio
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
J. Batlle-Diaz
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
G. Geoffroy
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
B. Lemieux
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
A. Barbeau*
Affiliation:
Centre Hospitalier l'Université de Sherbrooke; the Hôpital Ste-Justine; and the Clinical Research Institute of Montreal
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, H2W IR7 Quebec, Canada
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Summary:

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Echocardiographic examination of 21 patients with Friedreich's ataxia (age 7 to 28 years) showed cardiac abnormalities in 90% of the cases. They were characterized by varying degrees of septal hypertrophy in 81%, left ventricular free wall hypertrophy in 61%, and a slight reduction of left ventricular internal dimension in 57% of the cases. Asymmetric septal hypertrophy (ASH) with a septal/left ventricular free wall ratio of over 1.3 was found in 29% of the cases, and systolic anterior motion (SAM) of the mitral valve in three patients. Two other patients showed evidence of a different type of cardiomyopathy with marked symmetric left ventricular hypertrophy and marked left ventricular enlargement.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Copyright
Copyright © Canadian Neurological Sciences Federation 1976

References

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