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J Am Coll Cardiol, 2001; 37:911-919
© 2001 by the American College of Cardiology Foundation
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Sympathetic stimulation produces a greater increase in both transmural and spatial dispersion of repolarization in LQT1 than LQT2 forms of congenital long QT syndrome

Yasuko Tanabe, MD*, Masashi Inagaki, MD{dagger}, Takashi Kurita, MD*, Noritoshi Nagaya, MD*, Atsushi Taguchi, MD*, Kazuhiro Suyama, MD, PhD*, Naohiko Aihara, MD*, Shiro Kamakura, MD, PhD*, Kenji Sunagawa, MD, PhD{dagger}, Kazufumi Nakamura, MD, PhD{ddagger}, Tohru Ohe, MD, PhD, FACC{ddagger}, Jeffrey A. Towbin, MD§, Silvia G. Priori, MD, PhD|| and Wataru Shimizu, MD, PhD*

* Division of Cardiology, Department of Internal Medicine, National Cardiovascular Center, Suita, Japan
{dagger} Department of Cardiovascular Dynamics, National Cardiovascular Center, Suita, Japan
{ddagger} Department of Cardiovascular Medicine, Okayama University Medical School, Okayama, Japan
§ Department of Pediatrics (Cardiology), Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas, USA
|| Molecular Cardiology, Salvatore Maugeri Foundation, Pavia, Italy



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Figure 1 Twenty-four-lead ECGs (G–K: 2–6) that are expected to reflect the potential from the left ventricular free wall under baseline condition (A) and during epinephrine infusion (B) in a patient (pt) with LQT1 syndrome. A representative ECG (H4) is shown on the upper trace in each panel. The ECGs showed broad-based T waves commonly observed in LQT1 patients. Both the QTc-e and QTc-p were prolonged (603, 482 ms1/2) and the Tcp-e was increased (121 ms1/2) under the baseline condition (A). Epinephrine produced a prominent prolongation in the QTc-e (712 ms1/2), but a mild prolongation in the QTc-p (520 ms1/2), resulting in a dramatic increase in the Tcp-e (192 ms1/2) (B).

 


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Figure 2 Twenty-four-lead ECGs (G–K: 2–6) under baseline condition (A) and during epinephrine infusion (B) in a patient (pt) with LQT2 syndrome. A representative ECG (I4) is shown on the upper trace in each panel. The ECGs showed low-amplitude T wave with a notched appearance commonly seen in LQT2 patients. The QTc-e and QTc-p were prolonged (518, 414 ms1/2) and the Tcp-e was increased (104 ms1/2) under the baseline condition (A). Epinephrine produced a moderate prolongation in the QTc-e (618 ms1/2) and the QTc-p (494 ms1/2), resulting in a mild increase in the Tcp-e (124 ms1/2) (B).

 


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Figure 3 Twenty-four-lead ECGs (G–K: 2–6) under baseline condition (A) and during epinephrine infusion (B) in a control patient (pt). A representative ECG (H4) is shown on the upper trace in each panel. The QTc-e and QTc-p were much shorter (396, 314 ms1/2) and the Tcp-e was smaller (82 ms1/2) than those in LQT1 and LQT2 patients under the baseline condition (A). Epinephrine produced no significant changes in the QTc-e (410 ms1/2), the QTc-p (325 ms1/2), and the Tcp-e (85 ms1/2).

 


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Figure 4 Comparison of the differences before and after epinephrine in the mean QTc-e (A), QTc-p (B), Tcp-e (C), and Tcp-e/QTc-e ratio (D), which were averaged among 87-leads, in LQT1, LQT2, and control groups. The changes in the mean QTc-e with epinephrine were largest in LQT1 patients, intermediate in LQT2 patients, and were not significant in control patients (A), whereas changes in the mean QTc-p were not different among the three groups (B). As a consequence, the changes in the Tcp-e (C) and Tcp-e/QTc-e ratio (D) were largest in LQT1 patients, intermediate in LQT2 patients, and were not significant in control patients.

 


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Figure 5 Comparison of the differences before and after epinephrine in the max QTc-e (A), min QTc-e (B), QTc-eD (C), max QTc-p (D), minQTc-p (E), and QTc-pD (F), which were obtained from 87-leads, in LQT1, LQT2, and control groups. Changes in the max QTc-e with epinephrine were largest in LQT1 patients, intermediate in LQT2 patients, and were not significant in control patients (A), whereas changes in the min QTc-e were not different between LQT1 and LQT2 patients, but were significantly larger than those in control patients (B). As a consequence, changes in the QTc-eD were larger in LQT1 patients than those in LQT2 and control patients (C). In contrast, no significant differences were seen in the changes in the max QTc-p (D), min QTc-p (E), and QTc-pD (F) among the three groups.

 




 
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