Mazzanti et al reported the 1-2-3-LQTS-Risk model for evaluating a patient with a congenital long QT syndrome with one of the 3 most common genotypes. A patient at risk for a life-threatening arrhythmic event may be a candidate for an implantable cardioverter-defibrillator. The authors are from multiple institutions in Italy and the University of Rochester.
Patient selection: congenital long QT syndrome with genotype 1, 2, or 3
Parameters:
(1) QTc interval in milliseconds
(2) genotype of congenital LQT
Parameter
Finding
Points
QTc interval
0.01365 * (QTc - 469.9075)
genotype
LQT1
0
LQT2
0.83454
LQT3
0.94523
prognostic index =
= SUM(points for both parameters)
5-year risk of experiencing a life-threatening arrhythmic event (LAE) =
= 1 - (0.9849143482^EXP(prognostic index))
Performance:
• The c-statistic is 0.79 in the derivation and 0.69 in the validation cohort.
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