Abstract
Fabry disease is an X-linked multisystem disorder caused by deficiency of the α-galactosidase A enzyme. Cardiovascular manifestations include hypertension, coronary disease, arrhythmias, valvular abnormalities, heart failure, and sudden death. Bradycardia and conduction system abnormalities are related initially to abnormal accumulation of glycolipids in the lysosomes of conduction tissues. Hypertrophy and eventual fibrosis provides a substrate for persistent conduction abnormalities and ventricular arrhythmias. Sudden cardiac death can be related to bradyarrhythmias or tachycardias. Enzyme replacement therapy can improve cardiac function and clinical outcomes. Pacemakers or defibrillators are important in the treatment of patients with Fabry disease who are at risk for arrhythmias.
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