Electrocardiographic findings in Anderson-Fabry patients on disease specific therapy: can treatment prevent ECG changes?

Abstract Background Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder that have gained attention due to the availability of therapeutic options. Disease specific therapy (DST), either by enzyme replacement or oral pharmacological chaperone, mainstay for AFD treatment. Although its widespread use, few data are available on electrocardiographic variations associated with DST. Purpose To evaluate ECG findings and in according time duration DST, comparing patients under long-term naïve starting during follow-up. Methods One-hundred-seventy-nine patients, ≥18 years old, 2 readable ECGs, were recruited present multicentre study cohort. Two excluded pacemaker (PM) implantation. Only DST (n=107) considered final cohort divided into groups duration: Group A (n=42) included treated ≥12 months at first evaluation, whereas B (n=65) started Results had no significant difference terms age presentation (48 [39–60] vs 48 [36–56] years; p=0.856) maximal wall thickness (13 [11–15] 13 [11–18] mm; p=0.090) they differed male prevalence (61% 38%; p=0.029) classic phenotype (86% 29%; p<0.0001). At baseline, more than half both abnormalities 61%; p=1.000). The among atrial fibrillation (AF, 5% 6%; p=1.000), degree atrioventricular block (AVB, 7% 5%; p=0.677), right bundle branch (RBBB, complete 8%; p=1.000; incomplete RBBB 14% 12%; p=0.776), left anterior fascicular (LAFB, 10% 9%; p=1.000) repolarization (48% p=0.423) was not significantly different. Conversely, ventricular hypertrophy (LVH) prevalent (64% 37%; p=0.010). During follow-up period (57 [60–28] 70 (37–85) B; p=0.152), developed alterations (38% 23%; p=0.127). In particular, A, 4 (10%) presented AF, 1 (2%) AVB, 7 (17%) RBBB, LAFB, LVH 8 (19%) abnormalities. B, (3%) (11%) 11 abnormalities; none LAFB. Conclusions this cohort, chronic (Group A) who treatment B) alterations. changes frequent 23%), mainly composed suggesting a prompt start early stage. Funding Acknowledgement Type funding sources: None.