Hostname: page-component-78c5997874-mlc7c Total loading time: 0 Render date: 2024-11-14T05:06:14.614Z Has data issue: false hasContentIssue false

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
Rights & Permissions [Opens in a new window]

Summary:

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.

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

REFERENCES

Abbasi, A.S., MacAlpin, R.N.. Eber, L.M. and Pearce, M.L. (1973). Left ventricular hypertrophy diagnosed by echocardiography. N. Engl. J. Med. 289: 118120.CrossRefGoogle ScholarPubMed
Barbeau, A. (1979). Friedrich’s ataxia 1979; an overview. Can. J. Neurol. Sci. 6: 311319.CrossRefGoogle ScholarPubMed
Boyer, S.H., Chisholm, A.W. and McKusick, V.A. (1962). Cardiac aspects of Friedreich’s ataxia. Circulation 25: 493505.CrossRefGoogle ScholarPubMed
Côté, MDavignon, A., Pecko-Drouin, K., Solignac, A., Geoffroy, G., Lemieux, B. and Barbeau, A. (1976). Cardiological signs and symptoms in Friedreich’s ataxia. Can. J. Neurol. Sci. 3: 319321.CrossRefGoogle ScholarPubMed
Gach, J.VAdriange, M. and Franck, G. (1971). Hypertrophic obstructive cardio-myopathy and Friedreich’s ataxia. Report of a case and review of literature. Am. J. Cardiol. 27: 436441.Google Scholar
Goodwin, J.F. (1974). Prospects and predictions for the cardiomyopathies. Circula-tion 50: 210219.CrossRefGoogle ScholarPubMed
Henry, W.L., Clark, CE. and Epstein, S.E. (1973). Asymmetric septal hypertrophy. Echocardiographic identification of the pathognomonic anatomic abnormality of the 1HSS. Circulation 47: 225233.CrossRefGoogle Scholar
Hewer, R.L. (1969). The heart in Friedreich’s ataxia. Brit. Heart J. 31: 514.CrossRefGoogle ScholarPubMed
Huxtable, R. (1978). Cardiac pharmacology and cardiomyopathy in Friedreich’s ataxia. Can. J. Neurol. Sci. 5: 8391.CrossRefGoogle ScholarPubMed
Kisslo, J., Von Ramm, O.T. and Thurstone, F.L. (1976). Cardiac imaging using a phased array ultrasound system. II. Clinical technique and application. Circulation 53: 262267.CrossRefGoogle ScholarPubMed
Maron, B.J., Gottdiener, IS., Roberts, W.C., Henry, W.L., Savage, D.D. and Epstein, S.E. (1978). Left ventricular outflow tract obstruction due to systolic anterior motion of the anterior mitral leaflet in patients with concentric left ventricular hypertrophy. Circulation 57: 527533.CrossRefGoogle ScholarPubMed
Mintz, G.D., Kotler, M.N., Segal, B.L. and Parry, W.R. (1978). Systolic anterior motion of the mitral valve in the absence of asymmetric septal hypertrophy. Circulation 57: 256263.CrossRefGoogle ScholarPubMed
Pasternac, A, Tubau, J., Cousineau, D. and De Champlain, J. (1979). Increased plasma catecholamines in symptomatic mitral valve prolapse. Circulation 60 (Suppl. 2): 11156.Google Scholar
Pernot, C. (1979). La myocardiopathie de la maladie de Friedreich. A propos d’une série de 17 observations. Arch. Fr. Pediatr. 36: 218.Google Scholar
Polani, P.E. and Moynahan, EJ. (1972). Progressive cardiomyopathic lentiginosis. Q. J. Med. 41: 205.Google ScholarPubMed
Rosing, D.R., Kent, K.M., Borer, I.S., Seides, S.F., Maron, B.J. and Epstein, S.E. (1979a). Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy. Circulation 60: 12011207.CrossRefGoogle Scholar
Rosing, D R., Kent, K M., Maron, B.J. and Epstein, S.E. (1979b). Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy. II. Effects on exercise capacity and symptomatic status. Circulation 60: 12081213.CrossRefGoogle Scholar
Ruschhaupt, D.G., Thilenius, O.G. and Cassels, D E. (1973). Friedreich’s ataxia associated with idiopathic hypertrophic subaortic stenosis. Am. Heart J. 84: 95102.CrossRefGoogle Scholar
Smith, E.R., Sangalang, V.E., Heffernan, L.P., Welch, J.P. and Flemington, C.S. (1977). Hypertrophic cardiomyopathy: the heart disease of Friedreich’s ataxia. Am. Heart J. 94: 428434.CrossRefGoogle ScholarPubMed
Thoren, C. (1964). Cardiomyopathy in Friedreich’s ataxia: with studies of cardiovascular and respiratory functions. Acta Paediat. 53 (Suppl.153): 153.CrossRefGoogle Scholar
Troesch, M., Hirzel, H.O., Jenni, R. and Krayenbuhl, H.P. (1979). Reduction of septal thickness following verapamil in patients with asymmetric septal hypertrophy. Circulation 60 (Suppl.2): 11155.Google Scholar