Background: The diagnosis of the long-QT syndrome (LQTS) may be difficult to establish in patients with normal or borderline prolongation of the QT interval. Noninvasive markers are needed to identify patients with LQTS.
Methods and results: Fourteen patients with known LQTS, 9 unaffected family members, and 40 control subjects underwent modified Bruce protocol exercise testing. The RT interval (peak of R wave to peak of T wave) and rate-corrected RT interval (RTc) were measured during exercise and recovery. The RT interval at 1 minute into recovery was subtracted from the RT interval at a similar heart rate during exercise (deltaRT). The RTc shortened by 61 milliseconds (ms) in the LQTS patients compared with 23 to 26 ms in the other two groups (P=.003 by ANOVA). The RT interval shortened in a linear fashion in all patients but demonstrated persistent shortening during recovery in the LQTS patients. This was manifested as a hysteresis loop in the curve relating the RT interval to cycle length. The hysteresis loop was present in 13 of 14 LQTS patients and only 4 of 40 control subjects. DeltaRT >25 ms had a sensitivity of 73%, a specificity of 92%, a positive predictive value of 79%, and a negative predictive value of 90% for LQTS.
Conclusions: Hysteresis of the RT interval with exercise may be useful for the diagnosis of LQTS.