AIMS: The aim of this pilot study was to evaluate provocative sotalol testing to unmask abnormal repolarization due to altered myocardial electrical properties as the key feature in acquired Long-QT-Syndrome. Reliable diagnosis and risk stratification for the individual patient are complicated by the multitude of mechanisms involved in acquired QT-prolongation. The combined influence of all components determines susceptibility to arrhythmias related to QT-prolongation. METHODS: Twenty consecutive patients who had experienced torsades de pointes in association with QT-prolonging drugs were tested with i.v. D,L-sotalol (2mg/kg) with 24-h intensive care monitoring to evaluate the repolarization process by determining QT- and QTc-prolongations. Results were compared to age and sex matched controls. RESULTS: At baseline, no differences between control and study population with regard to QT and QTc were detected. After sotalol infusion, QTc increased from 422+/-17 to 450+/-22ms in controls and from 434+/-20 to 541+/-37ms in the study population. Torsades de pointes occurred in three out of 20 patients (15%) in the study population but in none of the control patients following i.v. sotalol testing. CONCLUSIONS: Controlled exposure to sotalol successfully identifies patients with normal QTc intervals but altered myocardial repolarization. This may be useful for clarifying diagnosis and pathogenesis of acquired Long-QT-Syndrome.
AIMS: The aim of this pilot study was to evaluate provocative sotalol testing to unmask abnormal repolarization due to altered myocardial electrical properties as the key feature in acquired Long-QT-Syndrome. Reliable diagnosis and risk stratification for the individual patient are complicated by the multitude of mechanisms involved in acquired QT-prolongation. The combined influence of all components determines susceptibility to arrhythmias related to QT-prolongation. METHODS: Twenty consecutive patients who had experienced torsades de pointes in association with QT-prolonging drugs were tested with i.v. D,L-sotalol (2mg/kg) with 24-h intensive care monitoring to evaluate the repolarization process by determining QT- and QTc-prolongations. Results were compared to age and sex matched controls. RESULTS: At baseline, no differences between control and study population with regard to QT and QTc were detected. After sotalol infusion, QTc increased from 422+/-17 to 450+/-22ms in controls and from 434+/-20 to 541+/-37ms in the study population. Torsades de pointes occurred in three out of 20 patients (15%) in the study population but in none of the control patients following i.v. sotalol testing. CONCLUSIONS: Controlled exposure to sotalol successfully identifies patients with normal QTc intervals but altered myocardial repolarization. This may be useful for clarifying diagnosis and pathogenesis of acquired Long-QT-Syndrome.
Authors: L Gouas; V Nicaud; S Chaouch; M Berthet; A Forhan; J Tichet; L Tiret; B Balkau; P Guicheney Journal: Eur J Hum Genet Date: 2007-05-30 Impact factor: 4.246
Authors: David G Strauss; Jose Vicente; Lars Johannesen; Ksenia Blinova; Jay W Mason; Peter Weeke; Elijah R Behr; Dan M Roden; Ray Woosley; Gulum Kosova; Michael A Rosenberg; Christopher Newton-Cheh Journal: Circulation Date: 2017-02-17 Impact factor: 29.690