Related Experiment Video
Updated: May 18, 2026

Electrophysiological Assessment of Murine Atria with High-Resolution Optical Mapping
Published on: February 22, 2018
Electrophysiological findings in Fabry cardiomyopathy: mapping the maze of risk stratification
Jin Li1, Arne Warth, Philipp Schnabel
1Department of Cardiology, University of Heidelberg, Heidelberg, Germany.
Insights
Anderson-Fabry disease can cause cardiac hypertrophy and arrhythmias, even with preserved left ventricular function. This case highlights the need for better risk assessment in Fabry cardiomyopathy.
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Anderson-Fabry disease is a rare genetic disorder.
- Cardiac involvement, including hypertrophy and arrhythmias, is common.
- Current risk stratification for sudden cardiac death may be insufficient in these patients.
Abstract:
We report a case of Anderson-Fabry disease in a young man presenting with cardiac hypertrophy and asymptomatic non-sustained ventricular tachycardia. The patient was referred for evaluation of implantable cardioverter/defibrillator therapy. Assessment of left ventricular ejection fraction is considered the gold standard for identifying patients at risk of sudden cardiac death. However, this patient's left ventricular function was preserved. Electrophysiological study did not reveal inducible arrhythmia or cardiac conduction abnormalities. Review of the literature indicates limited knowledge on the electrophysiology of Fabry cardiomyopathy and highlights the need for optimized risk stratification strategies.
Related Concept Videos
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Acute Coronary Syndrome III: Diagnostic Studies
Cardiomyopathy II: Dilated Cardiomyopathy
Mechanism of Cardiac Arrhythmias
