Torsade de pointes is the arrhythmia producing syncope and risk of sudden death in patients with the congenital long-QT syndrome (LQTS). The magnitude of TDR is not related to a history of TdP. LQT1 patients showed a capacity to increase TDR at elevated heart rates, but LQT2 patients did so at a much wider rate range. Asymptomatic and symptomatic patients showed similar TDRs.Ĭonclusions- TDR is greater in LQT2 than in LQT1 patients. LQT1 patients showed abrupt increases in TPE values at RR intervals from 600 to 900 ms, but LQT2 patients did so at RR intervals from 600 to 1400 ms (longest RR studied). The median TPE interval was greater in LQT2 (112±5 ms) than LQT1 (91☒ ms) or unaffected (86☓ ms) patients ( P<0.001 for all group comparisons), and the maximal TPE values differed as well. Methods and Results- We reviewed Holter recordings from 90 genotyped subjects, 31 with LQT1, 28 with LQT2, and 31 from unaffected family members, to record TPE intervals by use of an automated computerized program. We explored the relationship of TPE interval to heart rate and to the presence of symptoms in patients with LQT1 and LQT2. The interval from T-wave peak to T-wave end (TPE interval) is the clinical counterpart of TDR. Clinically, LQT1 patients experience symptoms at elevated heart rates, but LQT2 patients do so at lower rates. Experimentally, LQT2 models show increased TDR compared with LQT1, and β-adrenergic stimulation increases TDR in both models. Customer Service and Ordering Informationīackground- Transmural dispersion of repolarization (TDR) may be related to the genesis of torsade de pointes (TdP) in patients with the long-QT (LQT) syndrome.Stroke: Vascular and Interventional Neurology.Journal of the American Heart Association (JAHA).Circ: Cardiovascular Quality & Outcomes. ![]() Arteriosclerosis, Thrombosis, and Vascular Biology (ATVB).
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