title: Arrhythmias in Fabry cardiomyopathy.
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.
authors.affiliation: Section of Advanced Heart Failure and Transplant Cardiology, Division of Cardiovascular Diseases, University of Alabama at Birmingham, 1900 University Boulevard, THT 321, Birmingham, AL 35294, USA. Electronic address: dacharya@uab.edu.
authors.forename: Deepak
authors.initials: D
authors.lastname: Acharya
authors.affiliation: Section of Electrophysiology, Division of Cardiovascular Diseases, University of Alabama at Birmingham, Faculty Office Tower, Room 930, 1530 3rd Avenue South, Birmingham, AL 35294-3400, USA.
authors.forename: Harish
authors.initials: H
authors.lastname: Doppalapudi
authors.affiliation: Section of Advanced Heart Failure and Transplant Cardiology, Division of Cardiovascular Diseases, University of Alabama at Birmingham, 1900 University Boulevard, THT 321, Birmingham, AL 35294, USA.
authors.forename: José A
authors.initials: JA
authors.lastname: Tallaj
mesh_terms: D001145:Arrhythmias, Cardiac; D009202:Cardiomyopathies; D000795:Fabry Disease; D006801:Humans
keywords: Arrhythmia; Bradycardia; Cardiomyopathy; Defibrillator; Fabry disease; Pacemaker; Tachycardia