Key result
A female patient with a cardiac variant of Fabry disease developed sustained ventricular tachycardia and dilated cardiomyopathy requiring oral amiodarone and a cardiac defibrillator implant.
Case Report (n=1)
Highlights that female patients with Fabry disease can develop severe cardiac manifestations including dilated cardiomyopathy and sustained ventricular tachycardia, challenging the notion of a universally good prognosis.
Female Fabry patients may develop VT and DCM; challenges benign prognosis assumption and leaves open systematic risk assessment.
Women with Fabry disease are considered to have a good prognosis. Here we report the clinical history of a female patient with severe Fabry disease before and after referral to our hospital. She was diagnosed to have a cardiac variant of Fabry disease since a left ventricular endomyocardial biopsy revealed specific features of this disease and that cardiac involvement was the sole manifestation. She developed sustained ventricular tachycardia and dilated cardiomyopathy requiring oral administration of amiodarone and a cardiac defibrillator implant.
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Igawa et al. (2005) conducted a case report in Fabry disease (n=1). Amiodarone and cardiac defibrillator implant was evaluated. A female patient with a cardiac variant of Fabry disease developed sustained ventricular tachycardia and dilated cardiomyopathy requiring oral amiodarone and a cardiac defibrillator implant.
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