# Arrhythmias and clinical outcomes in Fabry disease with cardiac and renal involvement

## TL;DR

This study examines how heart and kidney involvement in Fabry disease affects cardiovascular outcomes and arrhythmias.

## Contribution

The study identifies left ventricular wall thickness as an independent predictor of cardiovascular risk in Fabry disease.

## Key findings

- Patients with co-occurring heart and kidney involvement had the highest cardiovascular event rates.
- Left ventricular maximum wall thickness was an independent predictor of poor outcomes.
- Estimated glomerular filtration rate was not a significant predictor in multivariate analysis.

## Abstract

Both cardiomyopathy and nephropathy represent hallmark manifestations of Fabry disease (FD), evidence remains limited regarding cardiovascular risk stratification based on organ-specific involvement patterns. In this retrospective cohort study of FD patients from the First Affiliated Hospital of Zhejiang University, we evaluated associations between cardiac/renal involvement and major cardiovascular events, including: (1) cardiovascular mortality, (2) new-onset severe heart failure, (3) incident atrial fibrillation, and (4) significant arrhythmia requiring device implantation. Patients were stratified into four phenotypic groups: non-affected (24.1%, n = 20), cardiac-only (24.1%, n = 20), renal-only (18.1%, n = 15), and co-affected (33.7%, n = 28). During a median follow-up of 39 months, 36.1% (n = 30) reached the primary endpoint. The cohort demonstrated substantial arrhythmia burden with significant intergroup differences (p = 0.028). Univariate Cox analysis revealed both left ventricular maximum wall thickness (LVMWT; HR: 1.176, 95% CI: 1.050–1.336, p = 0.010) and estimated glomerular filtration rate (eGFR; HR: 0.978, 95% CI: 0.962–0.992, p < 0.001) as continuous variables were significantly associated with outcomes. However, multivariate analysis confirmed only LVMWT remained an independent predictor (p = 0.010), while eGFR lost its significance (p = 0.100). These findings demonstrate that organ involvement patterns exert a critical influence on cardiovascular prognosis in patients with FD, providing a framework for refined risk stratification.

The online version contains supplementary material available at 10.1186/s13023-025-04079-3.