0BJECTIVES: This study compared the effects of beta-blockade on transmural and spatial dispersion of repolarization (TDR and SDR, respectively) between the LQT1 and LQT2 forms of congenital long QT syndrome (LQTS). BACKGROUND: The LQT1 form is more sensitive to sympathetic stimulation and more responsive to beta-blockers than either the LQT2 or LQT3 forms. METHODS: Eighty-seven-lead, body-surface electrocardiograms (ECGs) were recorded before and after epinephrine infusion (0.1 μg/kg body weight per rain) in the absence and presence of oral propranolol (0.5-2.0 mg/kg per day) in 11 LQT1 patients and 11 LQT2 patients. The Q-Tend interval, the Q-Tpeak interval and the interval between Tpeak and Tend (Tp-e), representing TDR, were measured and averaged from 87-lead ECGs and corrected by Bazett's method (corrected Q-Tend interval [cQTe], corrected Q-Tpeak interval [cQTp ] and corrected interval between Tpeak and Tend [cTp-e]). The dispersion of cQTe (cQTe-D) was obtained among 87 leads and was defined as the interval between the maximum and minimum values of cQTe. RESULTS: Propranolol in the absence of epinephrine significantly prolonged the mean cQTp value but not the mean cQTe value, thus decreasing the mean cTp-e value in both LQT1 and LQT2 patients; the differences with propranolol were significantly larger in LQT1 than in LQT2 (p < 0.05). The maximum cQTe, minimum cQTe and cQTe-D were not changed with propranolol. Propranolol completely suppressed the influence of epinephrine in prolonging the mean cQTe, maximum cQTe and minimum cQTe values, as well as increasing the mean cTp-e and cQTe-D values in both groups. CONCLUSIONS: Beta-blockade under normal sympathetic tone produces a greater decrease in TDR in the LQT1 form than in the LQT2 form, explaining the superior effectiveness of beta-blockers in LQT1 versus LQT2. Beta-blockers also suppress the influence of sympathetic stimulation in increasing TDR and SDR equally in LQT1 and LQT2 syndrome.
ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine