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An "abnormal" QTc in males is a QTc above 450 ms; and, in females, above 470 ms. [17] If there is not a very high or low heart rate, the upper limits of QT can roughly be estimated by taking QT = QTc at a heart rate of 60 beats per minute (bpm), and subtracting 0.02 s from QT for every 10 bpm increase in heart rate.
The value for a normal QT interval is similar in males and females from birth up to adolescence. During infancy, a normal QTc is defined as 400 ± 20 milliseconds. Before puberty, the 99th percentile of QTc values is 460 milliseconds. After puberty, this value increases to 470 milliseconds in males and 480 milliseconds in females. [citation needed]
Long QT syndrome is associated with a prolonged QTc, although in some genetically proven cases of LQTS this prolongation can be hidden, known as concealed LQTS. [23] The QTc is less than 450 ms in 95% of normal males, and less than 460 ms in 95% of normal females. LQTS is suggested if the QTc is longer than these cutoffs.
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The cardiac features of JLNS can be diagnosed by measuring the QT interval corrected for heart rate (QTc) on a 12-lead electrocardiogram (ECG). The QTc is less than 450 ms in 95% of normal males, and less than 460 ms in 95% of normal females. In those with Jervell and Lange-Nielsen syndrome the QTc is typically greater than 500 ms. [8]
QT interval variability (QTV) refers to the physiological phenomenon of beat-to-beat fluctuations in QT interval of electrocardiograms. Increased QTV appears to be a marker of arrhythmic and cardiovascular death; it may also play a role for noninvasive assessment of sympathetic nervous system activity.
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The rarity of short QT syndrome makes calculating prognosis accurately difficult. The risk of sudden cardiac death has been estimated at 0.8% per year, [2] leading to a cumulative risk of sudden cardiac death of 41% by the age of 40. [1] A previous history of cardiac arrest predicts a higher likelihood of further dangerous arrhythmias. [1]