Treatment of Friedreich Ataxia in Heart Failure with Reduced Left Ventricular Ejection Fraction

Cardiomyopathy is a recognised complication of Friedreich ataxia, and a subset of patients develop heart failure with reduced left ventricular ejection fraction — a situation that calls for dedicated cardiac management alongside the neurological aspects of the disease.

Clinical scenario

Individual with Friedreich ataxia presenting with heart failure and reduced left ventricular ejection fraction. Current guidelines conditionally recommend managing the cardiac component according to established heart failure standards, while accounting for the patient's overall neurological and functional status.

Treatment approach (partial)

For patients with Friedreich ataxia and heart failure with reduced ejection fraction, advanced cardiac interventions — including device-based therapies — may be considered based on both cardiac status and overall health.

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References

We conditionally recommend treating individuals with Friedreich ataxia with a reduced left ventricular ejection fraction with medications according to current American Heart Association/American College of Cardiology heart failure guidelines (2013 & 2017 update).

Advanced heart failure therapies such as a left ventricular assist device, implantable cardioverter-defibrillator, biventricular pacemaker and heart transplantation should be considered for individuals with Friedreich ataxia and heart failure due to a reduced left ventricular ejection fraction, based on consideration of both their cardiac and overall health status.

DOI: 10.1186/s13023-022-02568-3

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