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Hemodynamic Findings in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

M. Cote
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
A. Davignon
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
G. Elias
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
A. Solignac
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
G. Geoffroy
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
B. Lemieux
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
A. Barbeau*
Affiliation:
Centre Hospitalier de l'Université de Sherbrooke; the Hôpital Ste-Justine, Montreal; the Hôpital Maisonneuve-Rosemont, the Hôpital Hôtel-Dieu de Montreal; and the Clinical Research Institute of Montreal
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, H2W 1R7 Quebec, Canada
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Summary:

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Thirteen patients with classical Friedreich's ataxia underwent cardiac catheterization with recordings of retrograde cardiac pressures, measurements of cardiac output and calculation of the left ventricular volumes and mass. The cardiomyopathy in Friedreich's ataxia falls into the hypertrophic group of cardiomyopathies with decreased compliance of ventricular myocardium, varying degrees of concentric and asymmetric hypertrophy and outflow tract obstruction. Although there is no clear parallel between the degree of abnormal hemodynamic findings and the degree of neurological impairment, severely handicapped patients may present a diffusely hypertrophied and hypokinetic left ventricular myocardium.

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

References

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