Cardiovascular survival in a cohort of 33 male Fabry patients followed for a median of 7.3 years at a single centre was most strongly associated in a Cox proportional hazard regression model with age and elevated arterial stiffness at baseline. Seven patients died of cardiovascular causes. The presence of cardiomyopathy and renal involvement (either clinical nephropathy or CKD stage >1) were also negatively associated with survival when analysed as single factors. In this small study, presence of hypertension and hypercholesterolemia at baseline did not significantly predict cardiovascular death. The significant effect of elevated arterial stiffness, especially in a small cohort, argues for its clinical utility in individual patient risk assessment and for further intervention studies focusing on therapeutic reduction.
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