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 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.

• Authors: Chih-Ta Yao, Jieh-Neng Wang, Yu-Chien Tsai, Chia-Shiang Lin, Jing-Ming Wu
• Journal: Journal of the Formosan Medical Association = Taiwan yi zhi (J Formos Med Assoc) Vol. 101 Issue 4 Pg. 291-3 (Apr 2002) ISSN: 0929-6646 [Print] Singapore
• PMID: 12101867 (Publication Type: Case Reports, Journal Article)

Chemical References

• Mexiletine
• Propranolol

Topics

• Drug Therapy, Combination
• Heart Block (drug therapy)
• Humans
• Infant, Newborn
• Long QT Syndrome (congenital, drug therapy)
• Male
• Mexiletine (administration & dosage)
• Propranolol (administration & dosage)