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<p>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%, <i>n</i> = 20), cardiac-only (24.1%, <i>n</i> = 20), renal-only (18.1%, <i>n</i> = 15), and co-affected (33.7%, <i>n</i> = 28). During a median follow-up of 39 months, 36.1% (<i>n</i> = 30) reached the primary endpoint. The cohort demonstrated substantial arrhythmia burden with significant intergroup differences (<i>p</i> = 0.028). Univariate Cox analysis revealed both left ventricular maximum wall thickness (LVMWT; HR: 1.176, 95% CI: 1.050–1.336, <i>p</i> = 0.010) and estimated glomerular filtration rate (eGFR; HR: 0.978, 95% CI: 0.962–0.992, <i>p</i> &lt; 0.001) as continuous variables were significantly associated with outcomes. However, multivariate analysis confirmed only LVMWT remained an independent predictor (<i>p</i> = 0.010), while eGFR lost its significance (<i>p</i> = 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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月5日

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  • Xiang Yin,
  • Zhuonan Song,
  • Xiangjie Sun,
  • Xiaogang Guo,
  • Tianxin Ye,
  • Fangcong Yu,
  • Hui Yan,
  • Xiaosheng Hu

摘要

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.