Hostname: page-component-cd9895bd7-p9bg8 Total loading time: 0 Render date: 2024-12-26T22:49:27.643Z Has data issue: false hasContentIssue false
  • English
  • Français

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
Get access Rights & Permissions [Opens in a new window]

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
Check for updates
Copyright
© Cambridge University Press 2015 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

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