Friedreich’s Ataxia: a review from a cardiology perspective

Review Article

Abstract

Background

Neuromuscular disorders are not among the common causes of cardiomyopathy in the general population; however, cardiomyopathy is known to occur in several neuromuscular disorders including Friedreich’s Ataxia (FA). In patients with neuromuscular disorders, concomitant cardiac involvement contributes significantly to morbidity and mortality and often leads to premature death.

Methods

An extensive literature search of Medline and Pubmed was conducted to include all published reports on cardiac involvement in FA. Secondary articles were identified from key paper reference listings.

Conclusion

Hypertrophic cardiomyopathy is a cardinal feature of FA; therefore all FA patients should be screened for cardiomyopathy. A cardiac examination, ECG and ECHO are advised at diagnosis, and also on the development of any cardiac symptoms. Treatment is determined by the presence of symptoms, the presence of left ventricular outflow gradient and the sudden death risk. Institution of aggressive medical therapy early in the course of the disease may help improve quality of life and provide survival benefit.

Keywords

Friedreich’s Ataxia Cardiomyopathy Congestive cardiac failure Sudden cardiac death Arrhythmias 

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Copyright information

© Royal Academy of Medicine in Ireland 2011

Authors and Affiliations

  1. 1.Cardiac Arrhythmia Service, St Vincent’s University HospitalElm ParkIreland
  2. 2.UCLA Cardiac Arrhythmia Center, David Geffen School of Medicine at UCLALos AngelesUSA

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