“…In another recent cohort of 261 patients, 12 of the 21 deaths were caused by cardiac complications (six were the result of sudden cardiac death [SCD]). An ICD was implanted in 19 (7%) patients, mainly for primary prevention (11 patients) [12]. Finally, a recent systematic review including 13 studies and 4185 patients, with a follow-up period of 1.2-10 years, revealed that of the 8.3% of deaths that occurred, 75% had cardiovascular causes and 62% were the result of SCD, a major cause of cardiovascular mortality in FD.…”