50 Chapter 2 Supplemental table 2: Prevalence, event rates and age at onset of individual outcomes † All (213) Men (83) Women (130) Classical (57) Non-classical (26) Classical (98) Non-classical (32) 1. Sudden cardiac death Number of patients 4 (2%) 4 (7%) 0 (0%) 0 (0%) 0 (0%) Event rate, with 95%-CI 0.6 (0.2-1.4) 2.6 (0.8-6.2) 0 (-) 0 (-) 0 (-) Age at death 48 (47-56) 48 (47-56) (-) (-) (-) 2. Sudden cardiac arrest Number of patients 6 (3%) 1 (2%) 3 (12%) 2 (2%) 0 (0%) Event rate, with 95%-CI 0.8 (0.3-1.8) 0.6 (0.03-3.2) 2.5 (0.6-6.9) 0.6 (0.1-2.1) 0 (-) Age at first event 66 (46-73) 46 (-) 67 (64-67) 65 (57-73) (-) 3. Sustained ventricular tachycardia Number of patients 4 (2%) 0 (0%) 2 (8%) 2 (2%) 0 (0%) Event rate, with 95%-CI 0.6 (0.2-1.4) 0 (-) 1.7 (0.3-5.6) 0.6 (0.1-2.1) 0 (-) Age at first event 66 (51-73) (-) 66 (64-67) 62 (51-73) (-) 4. Ventricular fibrillation Number of patients 2 (1%) 1 (2%) 0 (0%) 1 (1%) 0 (0%) Event rate, with 95%-CI 0.3 (0.1-0.9) 0.6 (0.03-3.2) 0 (-) 0.3 (0.02-1.5) 0 (-) Age at first event 59 (46-73) 46 (-) (-) 73 (-) (-) 5. Second- degree AV block, Mobitz II Number of patients 1(1%) 0 (0%) 1 (4%) 0 (0%) 0 (0%) Event rate, with 95%-CI 0.1 (0.01-0.7) 0 (-) 0.8 (0.04-4.1) 0 (-) 0 (-) Age at first documentation 65 (-) (-) 65 (-) (-) (-) 6. Third- degree AV block Number of patients 9 (4%) 1 (2%) 4 (15%) 4 (4%) 0 (0%) Event rate, with 95%-CI 1.3 (0.6-2.3) 0.6 (0.03-3.2) 3.4 (1.1-8.1) 1.2 (0.4-3.0) 0 (-) Age at first documentation 60 (50-72) 60 (-) 58 (50-63) 59 (54-72) (-)
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