Heart rate dependence of the QT interval duration: differences among congenital long QT syndrome subtypes.
BACKGROUND
The heart rate dependence of QT interval duration is abnormal in patients with congenital long QT syndrome. Patients with LQT1 have a defective I(Ks) current, a major determinant of QT response to heart rate.


RESULTS
We studied the heart rate dependence of QT interval duration in different long QT syndrome genotypes and control subjects using computerized QT measurements obtained from Holter recordings. The dependence of QT duration on heart rate is steeper in long QT syndrome than in control subjects (0.347 +/- 0.263 vs 0.162 +/- 0.083 at heart rate 100 beats/min; P < 0.05). In addition, QT interval is significantly longer in LQT2 and LQT3 than in LQT1 patients at slow (533 +/- 23 ms vs 468 +/- 30 ms at heart rate 60 beats/min; P < 0.0001) but not at rapid heart rate. The heart rate dependence of QT interval is steeper in LQT2 and LQT3 than in LQT1 (0.623 +/- 0.245 vs 0.19 +/- 0.079 at heart rate 100 beats/min; P < 0.05). For a given heart rate, the QT intervals vary more in LQT2 and LQT3 than in LQT1 patients (25.98 +/- 11.18 ms vs 14.39 +/- 1.55 ms; P < 0.01).


CONCLUSIONS
Individual long QT syndrome genotypes differ with respect to QT interval dependence on heart rate. These differences may relate to the propensity of LQT2 and LQT3 patients to develop arrhythmias during bradycardia.
Nemec
Jan
J
Minnesota Heart Clinic, Edina, USA.
Buncová
Marie
M
Bůlková
Veronika
V
Hejlik
Joseph
J
Winter
Bruce
B
Shen
Win-Kuang
WK
Ackerman
Michael J
MJ
D000328:Adult; D015716:Electrocardiography, Ambulatory / Q000379:methods*; D005260:Female; D020022:Genetic Predisposition to Disease; D006329:Heart Conduction System / Q000503:physiopathology*; D006339:Heart Rate; D006801:Humans; D008133:Long QT Syndrome / Q000151:congenital* / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D008297:Male; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity