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Hypertrophic Cardiomyopathy in Friedreich's Ataxia: Symmetric or Asymmetric?

Published online by Cambridge University Press:  18 September 2015

A. Pasternac
Affiliation:
Montreal Heart Institute, the University of Montreal and the Department of Neurogenetics, the Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada
R. Król
Affiliation:
Montreal Heart Institute, the University of Montreal and the Department of Neurogenetics, the Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada
R. Petitclerc
Affiliation:
Montreal Heart Institute, the University of Montreal and the Department of Neurogenetics, the Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada
C. Harvey
Affiliation:
Montreal Heart Institute, the University of Montreal and the Department of Neurogenetics, the Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada
E. Andermann
Affiliation:
Montreal Heart Institute, the University of Montreal and the Department of Neurogenetics, the Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada
A. Barbeau*
Affiliation:
Montreal Heart Institute, the University of Montreal and the Department of Neurogenetics, the Montreal Neurological Hospital and Institute, McGill University, Montreal, Quebec, Canada
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada H2W 1R7
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Summary:

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We evaluated 15 patients with Friedreich's ataxia (FA) to define the incidence of myocardial involvement and the type of cardiomyopathy observed.

All patients with FA had either ECG, vectocardiographic or echocardiography abnormalities, suggesting some degree of myocardial involvement. In contrast to reports indicating that asymmetric septal hypertrophy (ASH), often obstructive, is associated with FA, symmetric, concentric hypertrophic cardiomyopathy (SCH) was the predominant abnormality (sixty-seven percent of patients). Echocardiograms should be performed periodically in all FA patients since this technique allows the detection of cardiac hypertrophy.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Copyright
Copyright © Canadian Neurological Sciences Federation 1980

References

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