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J Formos Med Assoc. 2002 Apr;101(4):291-3.

Congenital long QT syndrome with functionally impaired atrioventricular conduction: successful treatment by mexiletine and propranolol.

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Department of Pediatrics, National Cheng Kung University Hospital, 138 Sheng Li Road, Tainan, Taiwan.


Congenital long QT syndrome (LQTS) with atrioventricular block is a rare and malignant arrhythmia, which usually responds poorly to traditional beta-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 beta-blocker and pacemaker therapy. Our patient responded to a combination of sodium channel blocker (mexiletine) and beta-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.

[Indexed for MEDLINE]

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