Table 1.
International Calmodulinopathy Registry
| 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) |
a
Patients’ ethnicity was presented according to the population subgroups proposed in ExAC/gnomAD databases.22 For one patient, ethnicity was not reported.
b
ACA, SUD/SCD, VF detected/treated by ICD.
c
One patient underwent both procedures, the remaining three only LCSD.