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. Author manuscript; available in PMC: 2018 Dec 30.
Published in final edited form as: Int J Cardiol. 2016 Jun 23;220:290–298. doi: 10.1016/j.ijcard.2016.06.081

Table 3.

Comparison of Timothy syndrome, cardiac-only Timothy syndrome, and long QT syndrome probands in the literature.

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
a

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.