Ventricular tachycardia ablation in late-onset phenotype Fabry disease: a case report
Abstract
Background: Fabry disease is an X-linked lysosomal storage disorder that can cause progressive cardiomyopathy, myocardial fibrosis, conduction disease, and malignant ventricular arrhythmias. Ventricular tachycardia (VT) ablation in advanced Fabry cardiomyopathy is challenging, particularly in patients with extensive scar and left ventricular assist device (LVAD) support.
Case summary: A 71-year-old man with late-onset Fabry disease, confirmed by endomyocardial biopsy showing myelin body accumulation, reduced α-galactosidase A activity, and elevated plasma Gb3, developed severe left ventricular dysfunction, extensive late gadolinium enhancement, LVAD implantation, and complete atrioventricular block requiring dual-chamber pacemaker implantation. He presented with recurrent palpitations and near-syncope. Device interrogation revealed multiple VT episodes, including one episode degenerating into ventricular fibrillation, and surface electrocardiography documented wide-complex tachycardia with right bundle branch block morphology and an inferior axis. An electrophysiological study was performed using an antegrade transseptal left ventricular approach. Intracardiac echocardiography was used to delineate the LV cavity and LVAD position, facilitating safe catheter navigation and mapping while avoiding interference with the LVAD. Endocardial mapping demonstrated diffuse late potentials, low-voltage substrate, and deceleration zones involving the basal-to-mid-inferolateral left ventricle. Substrate modification targeting these abnormal electrograms eliminated inducible ventricular premature complexes. No recurrent VT episodes were detected on subsequent pacemaker interrogation.
Discussion: This case highlights the complex arrhythmogenic substrate of late-stage Fabry cardiomyopathy and the utility of intracardiac echocardiography-guided mapping in patients with LVAD support. Catheter ablation may provide short-term arrhythmia control, but in advanced Fabry disease, its role remains largely palliative.
Related
- Case Report: Upper septal fascicular ventricular tachycardia mimicking supraventricular tachycardia: diagnostic pitfalls and mapping-guided ablation
- Global Advances in Ventricular Tachycardia Ablation
- Transatrial air-assisted epicardial ventricular tachycardia ablation for hypertrophic cardiomyopathy and management of postprocedural right atrial thrombus.
- Thirty years of percutaneous epicardial access: reflections on the evolution of ventricular tachycardia substrate mapping and ablation
- Successful Catheter Ablation of Basal Crux Ventricular Tachycardia with Right Bundle Branch Block Morphology in a Patient with Dilated Cardiomyopathy
BibTeX
@article{tran2026ventricular,
title = {{Ventricular tachycardia ablation in late-onset phenotype Fabry disease: a case report}},
author = {Khuong Dang Tran and Hendyono Lim and Phuoc Huu Nguyen and Chin-Yu Lin},
journal = {European Heart Journal. Case Reports},
year = {2026},
month = oct,
doi = {10.1093/ehjcr/ytag746},
url = {https://doi.org/10.1093/ehjcr/ytag746},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Ventricular tachycardia ablation in late-onset phenotype Fabry disease: a case report
AU - Khuong Dang Tran
AU - Hendyono Lim
AU - Phuoc Huu Nguyen
AU - Chin-Yu Lin
PY - 2026
DA - 2026/10/12
JO - European Heart Journal. Case Reports
DO - 10.1093/ehjcr/ytag746
UR - https://doi.org/10.1093/ehjcr/ytag746
AB - Background: Fabry disease is an X-linked lysosomal storage disorder that can cause progressive cardiomyopathy, myocardial fibrosis, conduction disease, and malignant ventricular arrhythmias. Ventricular tachycardia (VT) ablation in advanced Fabry cardiomyopathy is challenging, particularly in patients with extensive scar and left ventricular assist device (LVAD) support. Case summary: A 71-year-old man with late-onset Fabry disease, confirmed by endomyocardial biopsy showing myelin body accumulation, reduced α-galactosidase A activity, and elevated plasma Gb3, developed severe left ventricular dysfunction, extensive late gadolinium enhancement, LVAD implantation, and complete atrioventricular block requiring dual-chamber pacemaker implantation. He presented with recurrent palpitations and near-syncope. Device interrogation revealed multiple VT episodes, including one episode degenerating into ventricular fibrillation, and surface electrocardiography documented wide-complex tachycardia with right bundle branch block morphology and an inferior axis. An electrophysiological study was performed using an antegrade transseptal left ventricular approach. Intracardiac echocardiography was used to delineate the LV cavity and LVAD position, facilitating safe catheter navigation and mapping while avoiding interference with the LVAD. Endocardial mapping demonstrated diffuse late potentials, low-voltage substrate, and deceleration zones involving the basal-to-mid-inferolateral left ventricle. Substrate modification targeting these abnormal electrograms eliminated inducible ventricular premature complexes. No recurrent VT episodes were detected on subsequent pacemaker interrogation. Discussion: This case highlights the complex arrhythmogenic substrate of late-stage Fabry cardiomyopathy and the utility of intracardiac echocardiography-guided mapping in patients with LVAD support. Catheter ablation may provide short-term arrhythmia control, but in advanced Fabry disease, its role remains largely palliative.
SN - 2514-2119
ER -