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Arrhythmias in Fabry cardiomyopathy
Deepak Acharya1, Harish Doppalapudi2, José A Tallaj1
1Section 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.
Cardiac Electrophysiology Clinics
|May 24, 2015
Summary
Fabry disease, a genetic disorder, causes severe cardiac issues like arrhythmias and heart failure due to enzyme deficiency. Enzyme replacement therapy and devices like pacemakers improve outcomes for affected patients.
Area of Science:
- Cardiology
- Genetics
- Metabolic Disorders
Background:
- Fabry disease is an X-linked genetic disorder resulting from α-galactosidase A enzyme deficiency.
- This deficiency leads to glycolipid accumulation, impacting multiple organ systems, notably the cardiovascular system.
Purpose of the Study:
- To outline the cardiovascular manifestations of Fabry disease.
- To discuss the mechanisms underlying cardiac dysfunction in Fabry disease.
- To review current therapeutic strategies for managing cardiac complications.
Main Methods:
- Review of existing literature on Fabry disease and its cardiac effects.
- Analysis of pathophysiological mechanisms of glycolipid accumulation in cardiac tissues.
- Evaluation of treatment outcomes for enzyme replacement therapy and device implantation.
Main Results:
- Cardiovascular issues in Fabry disease include hypertension, coronary artery disease, arrhythmias, valvular problems, heart failure, and sudden death.
- Bradycardia and conduction abnormalities stem from glycolipid buildup in cardiac conduction tissues.
- Cardiac hypertrophy and fibrosis create a basis for persistent arrhythmias and potential sudden cardiac death.
Conclusions:
- Fabry disease presents significant cardiovascular risks, including life-threatening arrhythmias and heart failure.
- Enzyme replacement therapy offers potential for improved cardiac function and clinical outcomes.
- Pacemakers and defibrillators are crucial for managing arrhythmia risk in patients with Fabry disease.
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