33 Cardiac outcomes in Fabry disease Table 2: Prevalence, event rates, and age at onset of death and cardiovascular events (continued) All (213) Men (83) Women (130) Classical (57) Non-classical (26) Classical (98) Non-classical (32) Sustained ventricular arrhythmias ‡ Number of patients 9 (4%) 4 (7%) 3 (12%) 2 (2%) 0 (0%) Event rate, with 95%-CI 1.3 (0.6-2.3) 2.6 (0.8-6.2) 2.5 (0.6-6.9) 0.6 (0.1-2.6) 0 (-) Age at first event 56 (46-73) 48 (46-56) 67 (64-67) 62 (51-73) (-) Myocardial infarction Number of patients 22 (10%) 8 (14%) 4 (15%) 10 (10%) 0 (0%) Event rate, with 95%-CI 3.1 (2.0-4.6) 5.2 (2.4-9.8) 3.4 (1.1-8.1) 3.1 (1.6-5.6) 0 (-) Age at first event 51 (33-75) 51 (33-58) 57 (34-67) 51 (34-75) (-) Conduction abnormalities § Number of patients 29 (14%) 7 (12%) 8 (50%) 12 (12%) 2 (6%) Event rate, with 95%-CI 4.1 (2.8-5.8) 4.5 (2.0-9.0) 6.7 (3.1-12.8) 3.7 (2.0-6.4) 1.8 (0.3-5.8) Age at first documentation 60 (48-74) 56 (48-60) 62 (50-65) 63 (49-74) 72 (71-72) Atrial fibrillation Number of patients 44 (21%) 20 (35%) 7 (27%) 16 (16%) 1 (3%) Event rate, with 95%-CI 6.2 (4.6-8.3) 12.9 (8.1-19.6) 5.9 (2.6-11.6) 5.0 (3.0-7.9) 0.9 (0.04-4.3) Age at first event 55 (18-69) 49 (18-61) 57 (34-69) 58 (47-67) 68 (-) Data in table 2 is presented as number (percentage) or median (range). All event rates are per 1000 patient years from the age of 15 onwards. ¥ Major adverse cardiac events: composite of cardiovascular death, heart failure hospitalization, sustained ventricular arrhythmias and myocardial infarction. ‡ Sustained ventricular arrhythmias: composite of sudden cardiac death, sudden cardiac arrest, sustained ventricular tachycardia including appropriate ICD shock, and ventricular fibrillation. § Conduction abnormalities: composite of second-degree AV block Mobitz II, third-degree AV block, sinusarrest, and pacemaker or implantable cardiac defibrillator device implantation for conduction abnormalities. 2
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