Congenital long QT syndrome with functionally impaired atrioventricular conduction

Successful treatment by mexiletine and propranolol

Chih Ta Yao, Jieh-Neng Wang, Yu Chien Tsai, Chia Shiang Lin, Jing-Ming Wu

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Congenital long QT syndrome (LQTS) with atrioventricular block is a rare and malignant arrhythmia, which usually responds poorly to traditional β-blocker therapy. We describe a male neonate with LQTS with ventricular tachycardia and 2:1 atrioventricular block. This patient's sister had similar presentation and died suddenly at the age of 8 months despite β-blocker and pacemaker therapy. Our patient responded to a combination of sodium channel blocker (mexiletine) and β-blocker (propranolol) therapy. He was asymptomatic during a 2-year follow-up period. This case suggests that propranolol combined with mexiletine might be useful in the treatment of patients with LQTS with atrioventricular block.

Original languageEnglish
Pages (from-to)291-293
Number of pages3
JournalJournal of the Formosan Medical Association
Volume101
Issue number4
Publication statusPublished - 2002

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Mexiletine
Long QT Syndrome
Propranolol
Atrioventricular Block
Sodium Channel Blockers
Therapeutics
Ventricular Tachycardia
Cardiac Arrhythmias
Siblings
Newborn Infant

All Science Journal Classification (ASJC) codes

  • Medicine(all)

Cite this

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title = "Congenital long QT syndrome with functionally impaired atrioventricular conduction: Successful treatment by mexiletine and propranolol",
abstract = "Congenital long QT syndrome (LQTS) with atrioventricular block is a rare and malignant arrhythmia, which usually responds poorly to traditional β-blocker therapy. We describe a male neonate with LQTS with ventricular tachycardia and 2:1 atrioventricular block. This patient's sister had similar presentation and died suddenly at the age of 8 months despite β-blocker and pacemaker therapy. Our patient responded to a combination of sodium channel blocker (mexiletine) and β-blocker (propranolol) therapy. He was asymptomatic during a 2-year follow-up period. This case suggests that propranolol combined with mexiletine might be useful in the treatment of patients with LQTS with atrioventricular block.",
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Congenital long QT syndrome with functionally impaired atrioventricular conduction : Successful treatment by mexiletine and propranolol. / Yao, Chih Ta; Wang, Jieh-Neng; Tsai, Yu Chien; Lin, Chia Shiang; Wu, Jing-Ming.

In: Journal of the Formosan Medical Association, Vol. 101, No. 4, 2002, p. 291-293.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Congenital long QT syndrome with functionally impaired atrioventricular conduction

T2 - Successful treatment by mexiletine and propranolol

AU - Yao, Chih Ta

AU - Wang, Jieh-Neng

AU - Tsai, Yu Chien

AU - Lin, Chia Shiang

AU - Wu, Jing-Ming

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AB - Congenital long QT syndrome (LQTS) with atrioventricular block is a rare and malignant arrhythmia, which usually responds poorly to traditional β-blocker therapy. We describe a male neonate with LQTS with ventricular tachycardia and 2:1 atrioventricular block. This patient's sister had similar presentation and died suddenly at the age of 8 months despite β-blocker and pacemaker therapy. Our patient responded to a combination of sodium channel blocker (mexiletine) and β-blocker (propranolol) therapy. He was asymptomatic during a 2-year follow-up period. This case suggests that propranolol combined with mexiletine might be useful in the treatment of patients with LQTS with atrioventricular block.

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