Non-Pulmonary Vein Substrate Ablation of Recurrent Atrial Fibrillation in the Patient With Fabry Disease

Koumei Onuki1, Kenichi Hiroshima1, Akihiro Isotani1

  • 1Department of Cardiology Kokura Memorial Hospital Kitakyushu Japan.

Clinical Case Reports
|September 24, 2025
PubMed

Insights

Fabry disease (FD) causes progressive heart muscle degeneration. In patients with atrial fibrillation (AF), non-pulmonary vein substrate ablation may be beneficial due to specific left atrial electrical patterns.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Genetics

Background:

  • Fabry disease (FD) is a rare genetic disorder.
  • FD leads to progressive myocardial degeneration, affecting heart function.
  • Atrial fibrillation (AF) is a common complication in FD patients.

Purpose of the Study:

  • To investigate the electrophysiological substrate in the left atrium of FD patients with AF.
  • To evaluate the potential efficacy of non-pulmonary vein (non-PV) substrate ablation in this population.

Main Methods:

  • Electroanatomical mapping of the left atrium.
  • Analysis of voltage and potential fractionation in FD patients with AF.
  • Comparison with control groups (if applicable).

Main Results:

  • FD patients with AF exhibit distinct patterns, including limited low-voltage areas.
  • Presence of fractionated potentials in the left atrium of FD patients with AF.
  • These findings suggest an arrhythmogenic substrate beyond pulmonary veins.

Conclusions:

  • The specific left atrial substrate in FD patients with AF warrants further investigation.
  • Non-pulmonary vein substrate ablation may be a viable therapeutic strategy for AF management in FD.
  • Targeting non-PV areas could improve outcomes for FD patients experiencing AF.

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