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Echocardiographic Findings in Friedreich's Ataxia

  • H.F. Gattiker (a1), A. Davignon (a1), A. Bozio (a1), J. Batlle-Diaz (a1), G. Geoffroy (a1), B. Lemieux (a1) and A. Barbeau (a1)...

Summary:

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.

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Copyright

Corresponding author

Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, H2W IR7 Quebec, Canada

References

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Boyer, S.H., Chisholm, A.W., and McKusick, V.A. (1962). Cardiac aspects of Friedreich’s ataxia. Circulation, 25, 493505.
Feigenbaum, H. (1972). Clinical applications of echocardiography. Progr. Car-diovasc. Dis., 14, 531558.
Henry, W.L., Clark, C.E., and Epstein, S.E. (1973). Echocardiographic identification of the pathognomonic anatomic abnormality of IHSS. Circulation, 47, 225233.
Lalani, L.V., and Lee, S.J.K. (1976). Clinical echocardiography – An overview. Canad, Med. Ass. J., 114, 4654.
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.
Smith, E.R., Flemington, C.S., Heffernan, L.P., and Sangalang, V.E. (1975). Echocardiography in Friedreich’s ataxia. Clin. Res., 23, No. 3.

Echocardiographic Findings in Friedreich's Ataxia

  • H.F. Gattiker (a1), A. Davignon (a1), A. Bozio (a1), J. Batlle-Diaz (a1), G. Geoffroy (a1), B. Lemieux (a1) and A. Barbeau (a1)...

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