International journal of cardiology · 2025
Multimodality imaging assessment of cardiac involvement in classic and late-onset Anderson-Fabry disease.
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Fan Jiali, Zhao Ru, Wang Jun, Zhu Jingfen, Kong Jie, Sun Sijia, Wang Yu, Lin Jia, Liu Yumeng, Jiang Tingbo, Ma Changsheng, Wang Heng, Zhou Bingyuan
Аннотация
BACKGROUND: Anderson-Fabry disease (AFD) manifests in two primary phenotypes-classic and late-onset-that share some clinical features but also exhibit distinct differences. Although both subtypes involve the heart, direct comparisons of their two-dimensional speckle tracking echocardiographic (2D-STE), atrioventricular coupling and comprehensive electrocardiographic (ECG) characteristics remain limited. Therefore, the aim of this study was to utilize multimodality imaging assessment to compare cardiac involvement in classic and late-onset AFD.
METHODS: This retrospective study included 80 AFD patients (40 classic, 40 late-onset). Echocardiography, ECG and cardiac magnetic resonance (CMR) were performed, followed by inverse probability of treatment weighting (IPTW) for age and sex balance. The left atrioventricular coupling index (LACI), left atrial (LA) strain, left ventricular (LV) global longitudinal strain (GLS), late gadolinium enhancement (LGE) and T1 values were assessed. Linear mixed models and restricted cubic spline (RCS) analysis were used to explore the effects of age and nonlinear relationships.
RESULTS: After IPTW adjustment, classic AFD patients showed more severe ECG abnormalities, including higher prevalence of delta waves, short PQ interval and pathological repolarization, increased Sokolow-Lyon indices, and lower T-wave amplitudes. On echocardiography and CMR, classic AFD patients exhibited greater impairment in LV diastolic dysfunction (LVDD), GLS, and LA function, along with larger LA volumes and lower T1 values, than those with late-onset AFD. Specifically, classic AFD males demonstrated significantly more impaired GLS and LA function, as well as larger LA volumes and LACI compared to late-onset AFD males, whereas classic females exhibited more severe LVDD without significant intergroup differences in GLS and LA function. Notably, LACI was significantly higher and progressed more rapidly with age in classic AFD patients. RCS analysis revealed that both LVMI and GLS demonstrated significant nonlinear associations with age in both classic and late-onset AFD patients, with a slower rate of progression observed after 41 years in classic patients and 46 years in late-onset patients.
CONCLUSIONS: Classic AFD was associated with more severe cardiac involvement than late-onset AFD, especially in AFD males, highlighting the importance of close cardiac monitoring in these patients.
METHODS: This retrospective study included 80 AFD patients (40 classic, 40 late-onset). Echocardiography, ECG and cardiac magnetic resonance (CMR) were performed, followed by inverse probability of treatment weighting (IPTW) for age and sex balance. The left atrioventricular coupling index (LACI), left atrial (LA) strain, left ventricular (LV) global longitudinal strain (GLS), late gadolinium enhancement (LGE) and T1 values were assessed. Linear mixed models and restricted cubic spline (RCS) analysis were used to explore the effects of age and nonlinear relationships.
RESULTS: After IPTW adjustment, classic AFD patients showed more severe ECG abnormalities, including higher prevalence of delta waves, short PQ interval and pathological repolarization, increased Sokolow-Lyon indices, and lower T-wave amplitudes. On echocardiography and CMR, classic AFD patients exhibited greater impairment in LV diastolic dysfunction (LVDD), GLS, and LA function, along with larger LA volumes and lower T1 values, than those with late-onset AFD. Specifically, classic AFD males demonstrated significantly more impaired GLS and LA function, as well as larger LA volumes and LACI compared to late-onset AFD males, whereas classic females exhibited more severe LVDD without significant intergroup differences in GLS and LA function. Notably, LACI was significantly higher and progressed more rapidly with age in classic AFD patients. RCS analysis revealed that both LVMI and GLS demonstrated significant nonlinear associations with age in both classic and late-onset AFD patients, with a slower rate of progression observed after 41 years in classic patients and 46 years in late-onset patients.
CONCLUSIONS: Classic AFD was associated with more severe cardiac involvement than late-onset AFD, especially in AFD males, highlighting the importance of close cardiac monitoring in these patients.