Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease

摘要: Objectives Assess the impact of end-stage renal disease (chronic kidney stage 5 (CKD5)) on cardiovascular outcomes in patients with Fabry enzyme replacement therapy. Background disease, an X-linked lysosomal storage causes hypertrophic cardiomyopathy and dysfunction. Methods Cardiac function 25 male were analysed at 0, 1, 2, 5, 7 10 years after initiation treatment. Patients grouped baseline into those CKD5 (n=10) without (n=15). ECG echocardiography performed 6 12 monthly, respectively, while was measured yearly. Results After treatment, cardiac non-CKD5 remained unchanged. In contrast, associated worse parameters progressive LV hypertrophy. mass index grew by 35.4±31.8 g/m 2.7 versus 5.7±7.9 g/m , p=0.044 non-CKD5, predominantly due to increased interventricular septal wall thickness (7.7±5.5 mm vs 1.3±1.7 mm, p=0.003). Cardiovascular events, including sudden death, arrhythmia pacing device insertion, occurred 100% (21 events) 26% (7 events), p Conclusions End-stage strongest indicator progression disease. Enzyme initiated prior stability showed ongoing deterioration. Additionally, E/Ea ≥15 may predict risk events.