Table 1.
Demographic and clinical characteristics | |
---|---|
Patients, n | 74 |
Families, n | 51 |
Males, n (%) | 38 (51) |
Age at last follow-up (years), median (IQR) | 9.0 (4.5–15.0) |
Population/Ethnicity, n (%)a | |
European/European American | 40 (55) |
Latino | 3 (4) |
East Asian (Japan) | 8 (11) |
South Asian (India) | 4 (5) |
African/African American | 2 (3) |
Others | 16 (22) |
Symptomatic, n (%) | |
Any cardiac event | 64 (86.5) |
MAEb | 50 (68) |
SUD/SCD | 20 (27) |
Age at first event (years), median (IQR) | 4.0 (1.5–8.0) |
Any treatment prescribed (n = 69) | |
No therapy | 13 (19) |
β-blockers, n (%) | 54 (78) |
Sodium channel blockers, n (%) | 21 (30) |
Other antiarrhythmic drugs (verapamil, nicorandil) | 4 (6) |
LCSD/RCSD,cn (%) | 4 (6) |
Implantable devices | 32 (46) |
Pacemaker, n (%) | 7 (10) |
ICD, n (%) | 25 (36) |
Events on therapy (n = 54 with known data) | 29 (54) |
Appropriate ICD discharges, n (%) | 16 (30) |
SCD, n (%) | 8 (15) |
Patients’ ethnicity was presented according to the population subgroups proposed in ExAC/gnomAD databases.22 For one patient, ethnicity was not reported.
ACA, SUD/SCD, VF detected/treated by ICD.
One patient underwent both procedures, the remaining three only LCSD.