Table 3.
TS | COTS | LQTS | Total | P-valuea | |
---|---|---|---|---|---|
Probands | 9 | 3 | 16 | 28 | |
Mutations | 4 | 2 | 14 | 20 | |
Age (y) | 2.15 ± 0.85 | 20.0 ± 5.0 | 19.1 ± 3.3 | 13.7 ± 2.5 | 0.001 |
% female | 44% | 100% | 75% | 68% | 0.2 |
QTc | 650 ± 29 | 493 ± 7 | 486 ± 10 | 539 ± 18 | <0.0001 |
AV block | 66% | 0% | 0% | 25% | 0.0024 |
SCD | 55% | 33% | 31% | 39% | 0.12 |
Fam Hx | 11% | 100% | 44% | 39% | 0.39 |
Fam Hx SCD | 0% | 33% | 19% | 14% | 0.28 |
TS vs LQTS cohorts; COTS, cardiac-only Timothy syndrome, SCD, personal history of aborted sudden cardiac death; Fam Hx, family history of disease in a 1st degree relative; Fam Hx SCD, family history of sudden cardiac death in a 1st degree relative.