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Echocardiographic Findings in Friedreich's Ataxia

Published online by Cambridge University Press:  18 September 2015

H.F. Gattiker ,
A. Davignon ,
A. Bozio ,
J. Batlle-Diaz ,
G. Geoffroy ,
B. Lemieux  and
A. Barbeau
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
Information
Canadian Journal of Neurological Sciences , Volume 3 , Issue 4 , November 1976 , pp. 329 - 332
Copyright
Copyright © Canadian Neurological Sciences Federation 1976

References

REFERENCES

Boyer, S.H., Chisholm, A.W., and McKusick, V.A. (1962). Cardiac aspects of Friedreich’s ataxia. Circulation, 25, 493–505.CrossRefGoogle ScholarPubMed
Feigenbaum, H. (1972). Clinical applications of echocardiography. Progr. Car-diovasc. Dis., 14, 531–558.CrossRefGoogle ScholarPubMed
Henry, W.L., Clark, C.E., and Epstein, S.E. (1973). Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation, 47, 225–233.CrossRefGoogle ScholarPubMed
Lalani, L.V., and Lee, S.J.K. (1976). Clinical echocardiography – An overview. Canad, Med. Ass. J., 114, 46–54.Google ScholarPubMed
Popp, R.L., Wolfe, S.B., Hirata, T., and Feigenbaum, H. (1969). Estimation of right and left ventricular size by ultrasound. Amer. J. Cardiol., 24, 523.CrossRefGoogle ScholarPubMed
Smith, E.R., Flemington, C.S., Heffernan, L.P., and Sangalang, V.E. (1975). Echocardiography in Friedreich’s ataxia. Clin. Res., 23, No. 3.Google Scholar