Long QT syndrome in children: the value of the rate corrected QT interval in children who present with fainting

Objective-A strategy was evaluated for identifying a proportion of children with long QT syndrome (LQTS) using the rate corrected QT interval (QTc) to systematically evaluate children who faint. Methods-QTc measurements and rates of fainting for the present analysis are available from families with KVLQT1, HERG, or SCN5A genotypes. QTc distributions in affected and unaffected children were documented and detection and false positive rates were modelled. Results-The mean QTc (SD) in 117 affected children was 0.484 seconds (0.031), and 0.420 seconds (0.021) among 133 unaffected children. At a cut off of 0.49 seconds, QTc measurement will identify 42.5% of affected and 0.1% of unaffected persons with a history of fainting who are alive at the time of testing. Assumptions include a prevalence of 1:5000 for LQTS, 2% mortality with the first arrhythmia, and a rate of fainting of 50% in affected children and 7% in unaffected children. Given these variables, a QTc cut off of 0.49 seconds detects 42 of 200 affected, along with 70 unaffected children out of a population of I million. If QTc greater than or equal to0.49 seconds is found in either parent of children with a QTc of 0.44 through 0.48 seconds, another 21 affected and 25 unaffected children will be identified. Conclusion-Systematically performing QTc measurements as part of the evaluation of children who faint might optimally identify about one third of patients with LQTS with few false positives and thereby offer an opportunity to prevent some sudden deaths.