Prevalence, event rates and age at onset of death and cardiovascular events
| All (N=213) | Men (N=83) | Women (N=130) | |||
| Classical (n=57) | Non-classical (n=26) | Classical (n=98) | Non-classical (n=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 years), with 95% CI | 3.4 (2.2 to 5.0) | 9.7 (5.7 to 15.6) | 2.5 (0.6 to 6.9) | 1.9 (0.8 to 3.9) | 0 (–) |
| Age at death (years), median (range) | 58 (26–77) | 56 (26–66) | 65 (64–68) | 72 (57–77) | (–) |
| Cause of death: HF, n (%) | 10 (42) | 4 (27) | 2 (67) | 4 (67) | 0 (0) |
| Cause of death: MI, n (%) | 2 (8) | 2 (13) | 0 (0) | 0 (0) | 0 (0) |
| Cause of death: ischaemic or haemorrhagic cerebrovascular accident, n (%) | 4 (17) | 2 (13) | 0 (0) | 2 (33) | 0 (0) |
| Cause death: SCD during haemodialysis | 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, n (%) | 38 (18) | 17 (30) | 7 (27) | 14 (14) | 0 (0) |
| Event rate, with 95% CI | 5.4 (3.9 to 7.3) | 11.0 (6.6 to 17.3) | 5.9 (2.6 to 11.6) | 4.4 (2.5 to 7.1) | 0 (–) |
| Age at first event (years) | 54 (33–75) | 52 (33–66) | 64 (34–67) | 54 (34–75) | (–) |
| CVD | |||||
| Number of patients, n (%) | 18 (9) | 10 (18) | 2 (8) | 6 (6) | 0 (0) |
| Event rate, with 95% CI | 2.5 (1.6 to 3.9) | 6.5 (3.3 to 11.5) | 1.7 (0.3 to 5.6) | 1.9 (0.8 to 3.9) | 0 (–) |
| Age at CVD (years) | 58 (47–77) | 55 (47–66) | 66 (65–68) | 72 (57–77) | (–) |
| HF hospitalisation | |||||
| Number of patients, n (%) | 18 (9) | 8 (14) | 4 (15) | 6 (6) | 0 (0) |
| Event rate, with 95% CI | 2.5 (1.6 to .9) | 5.2 (2.4 to 9.8) | 3.4 (1.1 to 8.1) | 1.9 (0.8 to 3.9) | 0 (–) |
| Age at first event (years) | 63 (43–77) | 54 (43–66) | 68 (64–69) | 69 (52–77) | (–) |
| SVAs† | |||||
| Number of patients, n (%) | 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 (years) | 56 (46–73) | 48 (46–56) | 67 (64–67) | 62 (51–73) | (–) |
| MI | |||||
| Number of patients, n (%) | 22 (10) | 8 (14) | 4 (15) | 10 (10) | 0 (0) |
| Event rate, with 95% CI | 3.1 (2.0 to 4.6) | 5.2 (2.4 to 9.8) | 3.4 (1.1 to 8.1) | 3.1 (1.6 to 5.6) | 0 (–) |
| Age at first event | 51 (33–75) | 51 (33–58) | 57 (34–67) | 51 (34–75) | (–) |
| CAs‡ | |||||
| Number of patients, n (%) | 29 (14) | 7 (12) | 8 (50) | 12 (12) | 2 (6) |
| Event rate, with 95% CI | 4.1 (2.8 to 5.8) | 4.5 (2.0 to 9.0) | 6.7 (3.1 to 12.8) | 3.7 (2.0 to 6.4) | 1.8 (0.3 to 5.8) |
| Age at first documentation (years) | 60 (48–74) | 56 (48–60) | 62 (50–65) | 63 (49–74) | 72 (71–72) |
| Atrial fibrillation | |||||
| Number of patients, n (%) | 44 (21) | 20 (35) | 7 (27) | 16 (16) | 1 (3) |
| Event rate, with 95% CI | 6.2 (4.6 to 8.3) | 12.9 (8.1 to 19.6) | 5.9 (2.6 to 11.6) | 5.0 (3.0 to 7.9) | 0.9 (0.04 to 4.3) |
| Age at first event (years) | 55 (18–69) | 49 (18–61) | 57 (34–69) | 58 (47–67) | 68 (–) |
Data are presented as number (percentage) or median (range). All event rates are per 1000 patient-years from the age of 15 years onwards.
*Major adverse cardiac events: composite of CVD, HF hospitalisation, SVAs and MI.
†SVAs: composite of SCD, sudden cardiac arrest, sustained ventricular tachycardia, including appropriate ICD shock, and ventricular fibrillation.
‡CAs: composite of second-degree AV block Mobitz II, third-degree AV block, sinus arrest and pacemaker or ICD device implantation for CAs.
AV, atrioventricular; CA, conduction abnormality; CVD, cardiovascular death; HF, heart failure; ICD, implantable cardiac defibrillator; MI, myocardial infarction; SCD, sudden cardiac death.