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. Author manuscript; available in PMC: 2012 Apr 23.
Published in final edited form as: Circulation. 2007 Apr 30;115(19):2481–2489. doi: 10.1161/CIRCULATIONAHA.106.665406

TABLE 4.

Phenotypic Characteristics by Biophysical Function of the KCNQ1 Mutations in 356 Subjects

Characteristics Dominant-Negative Effect (n=187) Haploinsufficiency (n=169)
Female, % 51 61
ECG at enrollment
 QTc,* ms 500±60 470±50
Therapy, %
β-Blockers 47 37
 Pacemaker 1.1 4.1
 Sympathectomy 0.5 0
 Defibrillator 4.8 7.7
First cardiac event*, % 53 27
 Syncope 45 22
 Aborted cardiac arrest 2.1 3.0
 Death 5.3 2.4

Percentages >10 are rounded to a whole number. Two subjects had missing data about the date of their first cardiac event.

*

P<0.01.