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Septal myectomy for hypertrophic obstructive cardiomyopathy in Friedreich’s ataxia

Published online by Cambridge University Press:  17 February 2015

Heather N. Anderson ,
Harold M. Burkhart  and
Jonathan N. Johnson
Heather N. Anderson
Affiliation:
Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, United States of America
Harold M. Burkhart
Affiliation:
Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota, United States of America
Jonathan N. Johnson*
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, Mayo Clinic, Rochester, Minnesota, United States of America
*
Correspondence to: Dr J. N. Johnson, MD, FACC, FASE, FAAP, Department of Medicine, Division of Cardiovascular Disease, Mayo Clinic, Gonda 6-138SW, First Street SW, Rochester, MN 55905, United States of America. Tel: 507 266 0676; Fax: 507 284 3968; E-mail: johnson.jonathan@mayo.edu
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Abstract

Hypertrophic cardiomyopathy associated with Friedreich’s ataxia is progressive, and there are few, if any, effective treatments available at present. This case report describes a Friedreich’s ataxia patient who had a septal myectomy for the management of hypertrophic cardiomyopathy with improved symptoms over a 7-year period. This suggests that septal myectomy may be a viable option to relieve symptoms and interrupt progression of heart disease in appropriately selected Friedreich’s ataxia patients.

Keywords

Friedreich’s ataxiahypertrophic cardiomyopathyseptal myectomy
Type
Brief Reports
Information
Cardiology in the Young , Volume 26 , Issue 1 , January 2016 , pp. 175 - 178
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Copyright
© Cambridge University Press 2015 

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References

1. Harding, AE. Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features. Brain: J Neurol 1981; 104: 589–620.Google Scholar
2. Schulz, JB, Boesch, S, Bürk, K, et al. Diagnosis and treatment of Friedreich ataxia: a European perspective. Nature reviews Neurology 2009; 5: 222–234.Google Scholar
3. Durr, A, Cossee, M, Agid, Y, et al. Clinical and genetic abnormalities in patients with Friedreich’s ataxia. N Engl J Med 1996; 335: 1169–1175.Google Scholar
4. Kipps, A, Alexander, M, Colan, SD, et al. The longitudinal course of cardiomyopathy in Friedreich's ataxia during childhood. Pediatr Cardiol 2009; 30: 306–310.Google Scholar
5. Hausse, AO, Aggoun, Y, Bonnet, D, et al. Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia. Heart 2002; 87: 346–349.Google Scholar
6. Lagedrost, SJ, Sutton, MSJ, Cohen, MS, et al. Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA). Am Heart J 2011; 161: 639.e1–645.e1.Google Scholar
7. Kearney, M, Orrell, RW, Fahey, M, et al. Antioxidants and other pharmacological treatments for Friedreich ataxia. Cochrane Database Syst Rev 2012; 4: CD007791.Google Scholar
8. Maron, BJ, Maron, MS. Hypertrophic cardiomyopathy. Lancet 2013; 381: 242–255.Google Scholar
9. Maron, BJ, McKenna, WJ, Danielson, GK, et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J Am Coll Cardiol 2003; 42: 1687–1713.Google Scholar
10. Gersh, BJ, Maron, BJ, Bonow, RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Circulation 2011; 124: e783–e831.Google Scholar