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Hemodynamic Findings in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

M. Cote ,
A. Davignon ,
G. Elias ,
A. Solignac ,
G. Geoffroy ,
B. Lemieux  and
A. Barbeau
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
Information
Canadian Journal of Neurological Sciences , Volume 3 , Issue 4 , November 1976 , pp. 333 - 336
Copyright
Copyright © Canadian Neurological Sciences Federation 1976

References

REFERENCES

Blass, J.P., Kark, R.A.P., and Menon, N.K. (1976). Low activities of the pyruvate and oxoglutarate dehydrogenase complexes in five patients with Friedreich’s ataxia. N.E.J.M., 295, 62–67.CrossRefGoogle ScholarPubMed
Boehm, T.M., Dickerson, R.B., and Glasser, S.P. (1970). Hypertrophic subaortic stenosis occurring in a patient with Friedreich’s ataxia. Amer. J. Med. Sci., 260, 279–284.Google Scholar
Boyer, S.H., Chisholm, A.W., and McKusick, V.A. (1962). Cardiac aspects of Friedreich’s ataxia. Circulation, 25, 493–505.CrossRefGoogle ScholarPubMed
Cote, M., and Elias, G. (1972). Hemodynamic and myocardial metabolism abnormalities in Friedreich’s ataxia. Amer. J. Cardiol., 33, 132 (Abstract).CrossRefGoogle Scholar
Gabriel, B., Pinsard, N., Gerard, R., and Louchet, E. (1974). Association d’une cardiomyopathie et d’une dégénérescence spi no-cé rébelle use (maladie de Friedreich). A propos d’une observation. Pédiatrie, 29, 367–377.Google Scholar
Gach, J.V., Andriange, M., and Franck, G. (1971). Hypertrophie obstructive cardiomyopathy and Friedreich’s ataxia. Report of a case and review of literature. Amer. J. Cardiol., 27, 436–441.CrossRefGoogle Scholar
Ivemark, B., and Thoren, C. (1964). The pathology of the heart in Friedreich’s ataxia – Changes in coronary arteries and myocardium. Acta Med. Scand., 175, 227–237.CrossRefGoogle ScholarPubMed
Kennedy, J.W., Trenholme, S.E., and Kasser, I.S. (1970). Left ventricular volume and mass from single plane cine-angiogram. A comparison of anteroposterior and right anterior oblique methods. Amer. Heart J., 80, 343–352.CrossRefGoogle Scholar
Moore, A.A.D. and Lambert, E.C. (1968). Cardiomyopathy associated with musuclar and neuromuscular disease. In: Paediatric Cardiology, The C. V. Mosby Co.Google Scholar
Ruschhaupt, D.G., Thilenius, O.G., and Cassels, D.E. (1972). Friedreich’s ataxia with idiopathic hypertrophic subaortic stenosis. Amer. Heart J., 84, 95–102.CrossRefGoogle ScholarPubMed
Soulie, P., Vernant, P., Gaudeau, S., Calesto, G., Joly, F., Bouchard, F., and Forman, I. (1966). Le coeur dans la maladie de Friedreich. Etude hémodynamique droite et gauche. Malattie Cardiovascolari, 7, 369–386.Google Scholar
Thoren, C. (1964). Cardiomyopathy in Friedreich’s ataxia with studies of cardiovascular and respiratory functions. Acta Paediat. (Stockholm), 53 (Suppl.), 153.Google Scholar