Mohamed El Sayed

32 Chapter 2 Table 2: Prevalence, event rates, and age at onset of death and cardiovascular events All (213) Men (83) Women (130) Classical (57) Non-classical (26) Classical (98) Non-classical (32) Death Number of patients, n (%) 24 (11%) 15 (26%) 3 (12%) 6 (6 %) 0 (0%) Deaths per 1000 person years (from the age of 15), with 95%-CI 3.4 (2.2-5.0) 9.7 (5.7-15.6) 2.5 (0.6-6.9) 1.9 (0.8-3.9) 0 (-) Age at death (years), median (range) 58 (26-77) 56 (26-66) 65 (64-68) 72 (57-77) (-) Cause of death: heart failure, n (%) 10 (42%) 4 (27%) 2 (67%) 4 (67%) 0 (0%) Cause of death: myocardial infarction, n (%) 2 (8%) 2 (13%) 0 (0%) 0 (0%) 0 (0%) Cause of death: ischemic or hemorrhagic cerebrovascular accident, n (%) 4 (17%) 2 (13%) 0 (0%) 2 (33%) 0 (0%) Cause death: sudden cardiac death during hemodialysis 2 (8%) 2 (13%) 0 (0%) 0 (0%) 0 (0%) Cause death: other, n (%) 6 (25%) 5 (33%) 1 (33%) 0 (0%) 0 (0%) Major adverse cardiovascular events¥ Number of patients 38 (18%) 17 (30%) 7 (27%) 14 (14%) 0 (0%) Event rate, with 95%-CI 5.4 (3.9-7.3) 11.0 (6.6-17.3) 5.9 (2.6-11.6) 4.4 (2.5-7.1) 0 (-) Age at first event 54 (33-75) 52 (33-66) 64 (34-67) 54 (34-75) (-) Cardiovascular death Number of patients 18 (9%) 10 (18%) 2 (8%) 6 (6 %) 0 (0%) Event rate, with 95%-CI 2.5 (1.6-3.9) 6.5 (3.3-11.5) 1.7 (0.3-5.6) 1.9 (0.8-3.9) 0 (-) Age at CVD 58 (47-77) 55 (47-66) 66 (65-68) 72 (57-77) (-) Heart failure hospitalization Number of patients 18 (9%) 8 (14%) 4 (15%) 6 (6%) 0 (0%) Event rate, with 95%-CI 2.5 (1.6-3.9) 5.2 (2.4-9.8) 3.4 (1.1-8.1) 1.9 (0.8-3.9) 0 (-) Age at first event 63 (43-77) 54 (43-66) 68 (64-69) 69 (52-77) (-)

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