Background-Experimental studies suggest that the interval between peak and end of T wave (Tpe) in transmural ECGs reflects transmural dispersion of repolarization (TDR), which is amplified by ␤-adrenergic stimulation in the LQT1 model. In 82 patients with genetically identified long-QT syndrome (LQTS) and 33 control subjects, we examined T-wave morphology and various parameters for repolarization in 12-lead ECGs including corrected QT (QTc; QT/R-R 1/2 ) and corrected Tpe (Tpec; Tpe/R-R 1/2 ) before and during exercise stress tests. Methods and Results-Under baseline conditions, LQT1 (nϭ51) showed 3 cardinal T-wave patterns (broad-based, normal-appearing, late-onset) and LQT2 (nϭ31) 3 patterns (broad-based, bifid with a small or large notch). The QTc and Tpec were 510Ϯ68 ms and 143Ϯ53 ms in LQT1 and 520Ϯ61 ms and 195Ϯ69 ms in LQT2, respectively, which were both significantly larger than those in control subjects (402Ϯ36 ms and 99Ϯ36 ms). Both QTc and Tpec were significantly prolonged during exercise in LQT1 (599Ϯ54 ms and 215Ϯ46 ms) with morphological change into a broad-based T-wave pattern. In contrast, exercise produced a prominent notch on the descending limb of the T wave, with no significant changes in the QTc and Tpec (502Ϯ82 ms and 163Ϯ86 ms: nϭ19) in LQT2. Conclusions-Tpe interval increases during exercise in LQT1 but not in LQT2, which may partially account for the finding that fatal cardiac events in LQT1 are more often associated with exercise.

Background-The management of aortic intramural hematoma (IMH) involving the ascending aorta (type A) has not been well-established. The purpose of this study was to clarify the long-term clinical outcomes of patients with type A IMH who were treated with medical therapy and timely operation. Methods and Results-Clinical data including operative mortality, IMH-related events, and long-term survival were retrospectively reviewed in 66 patients with type A IMH, who were admitted to our institution from 1986 to 2006. Emergent surgical repair was performed in 16 (24%) patients because of severe complications, whereas 50 patients were treated with initial medical therapy. In medically treated patients, 15 (30%) patients who demonstrated progression to classic dissection or increase in hematoma size within 30 days underwent surgical repair except for 2 patients who refused surgery. The 30-day mortality rate was 6% with emergent surgery and 4% with supportive medial therapy. There were 7 late deaths and the actuarial survival rates of all patients were 96Ϯ3%, 94Ϯ3%, and 89Ϯ5% at 1, 5, and 10 years, respectively. In medically treated patients, maximum aortic diameter was the only predictor of early and late progression of ascending IMH (hazard ratio, 4.43; 95% CI, 2.04 -9.64; PϽ0.001). Aortic diameter Ն50 mm predicted progression of ascending IMH with the positive and negative value of 83% and 84%, respectively. Conclusions-Combination

In the catheter ablation for AF, we found no significant reduction in the 1-year incidence of recurrent atrial tachyarrhythmias by ATP-guided PVI compared with conventional PVI.

Our findings suggest that loss-of-function SCN5A mutations resulting in Brugada syndrome are distinguished by profound bradyarrhythmias.

Titration of the duration of the ablation energy delivery while monitoring the ET could prevent periesophageal nerve injury due to the AF ablation, without decreasing the success rate of maintaining sinus rhythm.