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Arrhythmias in Fabry cardiomyopathy
Deepak Acharya1, Peter Robertson, G Neal Kay
1Division of Cardiovascular Diseases, University of Alabama at Birmingham, Birmingham, Alabama, USA. dacharya@cardiology.uab.edu
Clinical Cardiology
|August 14, 2012
Summary
Significant ventricular arrhythmias are uncommon in Fabry cardiomyopathy patients on enzyme replacement therapy (ERT). However, pacing utilization is high, and sudden cardiac death may be linked to bradycardia in this population.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Fabry cardiomyopathy is associated with a high incidence of ventricular arrhythmias.
- This study investigates the occurrence of significant arrhythmias in patients with Fabry cardiomyopathy.
Purpose of the Study:
- To evaluate the incidence of significant arrhythmias in a cohort of patients diagnosed with Fabry cardiomyopathy.
- To understand the role of arrhythmias in morbidity and mortality associated with Fabry cardiomyopathy.
Main Methods:
- Retrospective chart review of 19 patients with known Fabry cardiomyopathy.
- Analysis of device interrogation reports for patients with implantable devices.
- Review of electrocardiograms and monitoring data for patients without implantable devices.
Main Results:
- Nine out of 19 patients (47%) had implantable devices for indications including bradycardia and ventricular tachycardia.
- Patients with implantable cardioverter-defibrillators (ICDs) had lower heart rates pre-implant compared to those without devices.
- High pacing utilization was observed, with some patients requiring >95% pacing.
- Sudden cardiac death occurred in one patient without an implanted device.
Conclusions:
- Significant ventricular arrhythmias appear uncommon in Fabry cardiomyopathy patients receiving enzyme replacement therapy (ERT).
- High utilization of pacing suggests underlying conduction abnormalities.
- Bradycardia may be a contributing factor to sudden cardiac death in Fabry cardiomyopathy.
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