Literature DB >> 26632536

Pronounced Shortening of QT Interval With Mexiletine Infusion Test in Patients With Type 3 Congenital Long QT Syndrome.

Moritoshi Funasako1, Takeshi Aiba, Kohei Ishibashi, Ikutaro Nakajima, Koji Miyamoto, Yuko Inoue, Hideo Okamura, Takashi Noda, Shiro Kamakura, Toshihisa Anzai, Teruo Noguchi, Satoshi Yasuda, Yoshihiro Miyamoto, Kengo Fukushima Kusano, Hisao Ogawa, Wataru Shimizu.   

Abstract

BACKGROUND: Mexiletine is often used for medical therapy in LQT3 patients, however, the usefulness of mexiletine infusion test for LQT3 patients has not been reported. The aim of this study was to evaluate the usefulness of mexiletine infusion test for detecting LQT3 patients. METHODS AND 
RESULTS: We analyzed response in 12-lead electrocardiogram parameters measured in II or V5 to i.v. mexiletine infusion (2 mg/kg) during sinus rhythm among 31 genotype-positive LQT patients (29 ± 18 years, 12 male). Change in QTc interval after mexiletine was compared between LQT3 (n=15, 24 ± 21 years, 9 male) and other LQT patients (4 LQT1 and 12 LQT2; 34 ± 14 years, 3 male). Baseline RR, QT, and QTc interval were not different between the 2 groups (981 ± 182 vs. 1,023 ± 192 ms; 550 ± 94 vs. 524 ± 75 ms; 556 ± 66 vs. 520 ± 62 ms, respectively). While QTc interval was shortened with mexiletine in both groups (P<0.0001 vs. baseline), degree of QTc shortening (∆QTc) was significantly larger in LQT3 than in LQT1/LQT2 patients (99 ± 39 vs. 48 ± 32 ms; P=0.0004). The sensitivity, specificity and predictive accuracy of mexiletine infusion test for differentiating LQT3 from LQT1/LQT2 were 86.7%, 81.3% and 81.3%, respectively, and the optimal cut-off for ∆QTc was 69 ms on receiver operating characteristic analysis. No pro-arrhythmic event was observed.
CONCLUSIONS: Pronounced shortening of QT interval with mexiletine may facilitate genetic testing in patients with LQT3 syndrome.

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Year:  2015        PMID: 26632536     DOI: 10.1253/circj.CJ-15-0984

Source DB:  PubMed          Journal:  Circ J        ISSN: 1346-9843            Impact factor:   2.993


  12 in total

1.  Mexiletine rescues a mixed biophysical phenotype of the cardiac sodium channel arising from the SCN5A mutation, N406K, found in LQT3 patients.

Authors:  Rou-Mu Hu; David J Tester; Ryan Li; Tianyu Sun; Blaise Z Peterson; Michael J Ackerman; Jonathan C Makielski; Bi-Hua Tan
Journal:  Channels (Austin)       Date:  2018       Impact factor: 2.581

2.  Association of Genetic and Clinical Aspects of Congenital Long QT Syndrome With Life-Threatening Arrhythmias in Japanese Patients.

Authors:  Wataru Shimizu; Hisaki Makimoto; Kenichiro Yamagata; Tsukasa Kamakura; Mitsuru Wada; Koji Miyamoto; Yuko Inoue-Yamada; Hideo Okamura; Kohei Ishibashi; Takashi Noda; Satoshi Nagase; Aya Miyazaki; Heima Sakaguchi; Isao Shiraishi; Takeru Makiyama; Seiko Ohno; Hideki Itoh; Hiroshi Watanabe; Kenshi Hayashi; Masakazu Yamagishi; Hiroshi Morita; Masao Yoshinaga; Yoshiyasu Aizawa; Kengo Kusano; Yoshihiro Miyamoto; Shiro Kamakura; Satoshi Yasuda; Hisao Ogawa; Toshihiro Tanaka; Naotaka Sumitomo; Nobuhisa Hagiwara; Keiichi Fukuda; Satoshi Ogawa; Yoshifusa Aizawa; Naomasa Makita; Tohru Ohe; Minoru Horie; Takeshi Aiba
Journal:  JAMA Cardiol       Date:  2019-03-01       Impact factor: 14.676

Review 3.  Molecular Pathophysiology of Congenital Long QT Syndrome.

Authors:  M S Bohnen; G Peng; S H Robey; C Terrenoire; V Iyer; K J Sampson; R S Kass
Journal:  Physiol Rev       Date:  2017-01       Impact factor: 37.312

4.  European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) Expert Consensus Statement on the state of genetic testing for cardiac diseases.

Authors:  Arthur A M Wilde; Christopher Semsarian; Manlio F Márquez; Alireza Sepehri Shamloo; Michael J Ackerman; Euan A Ashley; Back Sternick Eduardo; Héctor Barajas-Martinez; Elijah R Behr; Connie R Bezzina; Jeroen Breckpot; Philippe Charron; Priya Chockalingam; Lia Crotti; Michael H Gollob; Steven Lubitz; Naomasa Makita; Seiko Ohno; Martín Ortiz-Genga; Luciana Sacilotto; Eric Schulze-Bahr; Wataru Shimizu; Nona Sotoodehnia; Rafik Tadros; James S Ware; David S Winlaw; Elizabeth S Kaufman; Takeshi Aiba; Andreas Bollmann; Jong-Il Choi; Aarti Dalal; Francisco Darrieux; John Giudicessi; Mariana Guerchicoff; Kui Hong; Andrew D Krahn; Ciorsti Mac Intyre; Judith A Mackall; Lluís Mont; Carlo Napolitano; Pablo Ochoa Juan; Petr Peichl; Alexandre C Pereira; Peter J Schwartz; Jon Skinner; Christoph Stellbrink; Jacob Tfelt-Hansen; Thomas Deneke
Journal:  J Arrhythm       Date:  2022-05-31

5.  European Heart Rhythm Association (EHRA)/Heart Rhythm Society (HRS)/Asia Pacific Heart Rhythm Society (APHRS)/Latin American Heart Rhythm Society (LAHRS) Expert Consensus Statement on the state of genetic testing for cardiac diseases.

Authors:  Arthur A M Wilde; Christopher Semsarian; Manlio F Márquez; Alireza Sepehri Shamloo; Michael J Ackerman; Euan A Ashley; Eduardo Back Sternick; Héctor Barajas-Martinez; Elijah R Behr; Connie R Bezzina; Jeroen Breckpot; Philippe Charron; Priya Chockalingam; Lia Crotti; Michael H Gollob; Steven Lubitz; Naomasa Makita; Seiko Ohno; Martín Ortiz-Genga; Luciana Sacilotto; Eric Schulze-Bahr; Wataru Shimizu; Nona Sotoodehnia; Rafik Tadros; James S Ware; David S Winlaw; Elizabeth S Kaufman; Takeshi Aiba; Andreas Bollmann; Jong Il Choi; Aarti Dalal; Francisco Darrieux; John Giudicessi; Mariana Guerchicoff; Kui Hong; Andrew D Krahn; Ciorsti MacIntyre; Judith A Mackall; Lluís Mont; Carlo Napolitano; Juan Pablo Ochoa; Petr Peichl; Alexandre C Pereira; Peter J Schwartz; Jon Skinner; Christoph Stellbrink; Jacob Tfelt-Hansen; Thomas Deneke
Journal:  Europace       Date:  2022-09-01       Impact factor: 5.486

6.  Gating Properties of Mutant Sodium Channels and Responses to Sodium Current Inhibitors Predict Mexiletine-Sensitive Mutations of Long QT Syndrome 3.

Authors:  Gang Li; Ryan L Woltz; Cheng-Yu Wang; Lu Ren; Pei-Xin He; Shan-Dong Yu; Xue-Qin Liu; Vladimir Yarov-Yarovoy; Dan Hu; Nipavan Chiamvimonvat; Lin Wu
Journal:  Front Pharmacol       Date:  2020-08-04       Impact factor: 5.810

7.  A distinct molecular mechanism by which phenytoin rescues a novel long QT 3 variant.

Authors:  Ivan Gando; Chiara Campana; Reina Bianca Tan; Frank Cecchin; Eric A Sobie; William A Coetzee
Journal:  J Mol Cell Cardiol       Date:  2020-04-24       Impact factor: 5.000

8.  Mexiletine infusion challenge test for neonatal long QT syndrome with 2:1 atrioventricular block.

Authors:  Hajime Okuwaki; Yoshiaki Kato; Lisheng Lin; Yoshihiro Nozaki; Miho Takahashi-Igari; Hitoshi Horigome
Journal:  J Arrhythm       Date:  2019-06-17

9.  Unusual Overlapping Cardiac Sarcoidosis and Long-QT Type 3 Induced Ventricular Fibrillation.

Authors:  Tatsuya Nishikawa; Takeshi Aiba; Nobuhiko Ueda; Kenzaburo Nakajima; Yuko Yamada-Inoue; Tsukasa Kamakura; Mitsuru Wada; Kenichiro Yamagata; Kohei Ishibashi; Emi Tateishi; Keisuke Kiso; Yoshihiko Ikeda; Hatsue Ishibashi-Ueda; Koji Miyamoto; Satoshi Nagase; Takashi Noda; Kengo Kusano
Journal:  Intern Med       Date:  2021-01-01       Impact factor: 1.271

Review 10.  The congenital long QT syndrome Type 3: An update.

Authors:  Andrés Ricardo Pérez-Riera; Raimundo Barbosa-Barros; Rodrigo Daminello Raimundo; Marianne Penachini da Costa de Rezende Barbosa; Isabel Cristina Esposito Sorpreso; Luiz Carlos de Abreu
Journal:  Indian Pacing Electrophysiol J       Date:  2017-10-31
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