Table 1
Open in new tab

International Calmodulinopathy Registry

Demographic and clinical characteristics
Patients, n74
Families, n51
Males, n (%)38 (51)
Age at last follow-up (years), median (IQR)9.0 (4.5–15.0)
Population/Ethnicity, n (%)a
 European/European American40 (55)
 Latino3 (4)
 East Asian (Japan)8 (11)
 South Asian (India)4 (5)
 African/African American2 (3)
 Others16 (22)
Symptomatic, n (%)
 Any cardiac event64 (86.5)
 MAEb50 (68)
 SUD/SCD20 (27)
Age at first event (years), median (IQR)4.0 (1.5–8.0)
Any treatment prescribed (n = 69)
 No therapy13 (19)
 β-blockers, n (%)54 (78)
 Sodium channel blockers, n (%)21 (30)
 Other antiarrhythmic drugs (verapamil, nicorandil)4 (6)
 LCSD/RCSD,c  n (%)4 (6)
 Implantable devices32 (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)
Demographic and clinical characteristics
Patients, n74
Families, n51
Males, n (%)38 (51)
Age at last follow-up (years), median (IQR)9.0 (4.5–15.0)
Population/Ethnicity, n (%)a
 European/European American40 (55)
 Latino3 (4)
 East Asian (Japan)8 (11)
 South Asian (India)4 (5)
 African/African American2 (3)
 Others16 (22)
Symptomatic, n (%)
 Any cardiac event64 (86.5)
 MAEb50 (68)
 SUD/SCD20 (27)
Age at first event (years), median (IQR)4.0 (1.5–8.0)
Any treatment prescribed (n = 69)
 No therapy13 (19)
 β-blockers, n (%)54 (78)
 Sodium channel blockers, n (%)21 (30)
 Other antiarrhythmic drugs (verapamil, nicorandil)4 (6)
 LCSD/RCSD,c  n (%)4 (6)
 Implantable devices32 (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.

Table 1
Open in new tab

International Calmodulinopathy Registry

Demographic and clinical characteristics
Patients, n74
Families, n51
Males, n (%)38 (51)
Age at last follow-up (years), median (IQR)9.0 (4.5–15.0)
Population/Ethnicity, n (%)a
 European/European American40 (55)
 Latino3 (4)
 East Asian (Japan)8 (11)
 South Asian (India)4 (5)
 African/African American2 (3)
 Others16 (22)
Symptomatic, n (%)
 Any cardiac event64 (86.5)
 MAEb50 (68)
 SUD/SCD20 (27)
Age at first event (years), median (IQR)4.0 (1.5–8.0)
Any treatment prescribed (n = 69)
 No therapy13 (19)
 β-blockers, n (%)54 (78)
 Sodium channel blockers, n (%)21 (30)
 Other antiarrhythmic drugs (verapamil, nicorandil)4 (6)
 LCSD/RCSD,c  n (%)4 (6)
 Implantable devices32 (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)
Demographic and clinical characteristics
Patients, n74
Families, n51
Males, n (%)38 (51)
Age at last follow-up (years), median (IQR)9.0 (4.5–15.0)
Population/Ethnicity, n (%)a
 European/European American40 (55)
 Latino3 (4)
 East Asian (Japan)8 (11)
 South Asian (India)4 (5)
 African/African American2 (3)
 Others16 (22)
Symptomatic, n (%)
 Any cardiac event64 (86.5)
 MAEb50 (68)
 SUD/SCD20 (27)
Age at first event (years), median (IQR)4.0 (1.5–8.0)
Any treatment prescribed (n = 69)
 No therapy13 (19)
 β-blockers, n (%)54 (78)
 Sodium channel blockers, n (%)21 (30)
 Other antiarrhythmic drugs (verapamil, nicorandil)4 (6)
 LCSD/RCSD,c  n (%)4 (6)
 Implantable devices32 (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.

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