Literature DB >> 29194862

A case report: Is mexiletine usage effective in the shortening of QTC interval and improving the T-wave alternans in Timothy syndrome?

Gulhan Tunca Sahin1, Yakup Ergul1.   

Abstract

Timothy syndrome (TS) is a multisystemic disease that occurs because of a mutation in CACN1C gene and is characterized by prolonged QT interval. Mexiletine is a Class 1B antiarrhythmic drug that causes the disappearance of T-wave alternans by shortening QTc and peak-to-end of the T wave. It may block the development of torsades de pointes in a prolonged QT. This study presented the case of a patient diagnosed with TS and had a cardiac arrest history, prolonged QT, and T-wave alternans. After mexiletine treatment, the QTc interval shortened and T-wave alternans disappeared. Such a case has rarely been seen in the literature, and hence considered rare. This case presentation was of particular importance because it highlighted the use of mexiletine besides an initial beta-blocker treatment in the cases with TS.
© 2017 Wiley Periodicals, Inc.

Entities:  

Keywords:  Timothy syndrome; cardiac arrhythmia; child; mexiletine

Mesh:

Substances:

Year:  2017        PMID: 29194862      PMCID: PMC6931748          DOI: 10.1111/anec.12522

Source DB:  PubMed          Journal:  Ann Noninvasive Electrocardiol        ISSN: 1082-720X            Impact factor:   1.468


  12 in total

1.  Sodium channel block with mexiletine is effective in reducing dispersion of repolarization and preventing torsade des pointes in LQT2 and LQT3 models of the long-QT syndrome.

Authors:  W Shimizu; C Antzelevitch
Journal:  Circulation       Date:  1997-09-16       Impact factor: 29.690

2.  Verapamil decreases ventricular tachyarrhythmias in a patient with Timothy syndrome (LQT8).

Authors:  Avrum Jacobs; Bradley P Knight; Karen T McDonald; Martin C Burke
Journal:  Heart Rhythm       Date:  2006-04-29       Impact factor: 6.343

Review 3.  Mexiletine: pharmacology and therapeutic use.

Authors:  A S Manolis; T F Deering; J Cameron; N A Estes
Journal:  Clin Cardiol       Date:  1990-05       Impact factor: 2.882

4.  Mexiletine Prevents Recurrent Torsades de Pointes in Acquired Long QT Syndrome Refractory to Conventional Measures.

Authors:  Marwan Badri; Aashay Patel; Chinmay Patel; Guizhi Liu; Matthew Goldstein; Victoria M Robinson; Xiaolin Xue; Lin Yang; Peter R Kowey; Gan-Xin Yan
Journal:  JACC Clin Electrophysiol       Date:  2015-06-22

Review 5.  Inhibition of late sodium current by mexiletine: a novel pharmotherapeutical approach in timothy syndrome.

Authors:  Yuanfeng Gao; Xiaolin Xue; Dayi Hu; Wenling Liu; Yue Yuan; Hongmei Sun; Lei Li; Katherine W Timothy; Li Zhang; Cuilan Li; Gan-Xin Yan
Journal:  Circ Arrhythm Electrophysiol       Date:  2013-04-11

6.  Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome: effects of beta-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes.

Authors:  W Shimizu; C Antzelevitch
Journal:  Circulation       Date:  1998-11-24       Impact factor: 29.690

7.  Ca(V)1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism.

Authors:  Igor Splawski; Katherine W Timothy; Leah M Sharpe; Niels Decher; Pradeep Kumar; Raffaella Bloise; Carlo Napolitano; Peter J Schwartz; Robert M Joseph; Karen Condouris; Helen Tager-Flusberg; Silvia G Priori; Michael C Sanguinetti; Mark T Keating
Journal:  Cell       Date:  2004-10-01       Impact factor: 41.582

8.  Cellular basis for the electrocardiographic and arrhythmic manifestations of Timothy syndrome: effects of ranolazine.

Authors:  Serge Sicouri; Katherine W Timothy; Andrew C Zygmunt; Aaron Glass; Robert J Goodrow; Luiz Belardinelli; Charles Antzelevitch
Journal:  Heart Rhythm       Date:  2007-01-07       Impact factor: 6.343

9.  A rare association of long QT syndrome and syndactyly: Timothy syndrome (LQT 8).

Authors:  U Krause; V Gravenhorst; T Kriebel; W Ruschewski; T Paul
Journal:  Clin Res Cardiol       Date:  2011-09-14       Impact factor: 5.460

10.  A rare association with suffered cardiac arrest, long QT interval, and syndactyly: Timothy syndrome (LQT-8).

Authors:  Yakup Ergül; İsa Özyılmaz; Sertaç Haydın; Alper Güzeltaş; Volkan Tuzcu
Journal:  Anatol J Cardiol       Date:  2015-08       Impact factor: 1.596

View more
  1 in total

1.  Dysfunctional Cav1.2 channel in Timothy syndrome, from cell to bedside.

Authors:  Dan Han; Xiaolin Xue; Yang Yan; Guoliang Li
Journal:  Exp Biol Med (Maywood)       Date:  2019-07-19
  1 in total

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