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 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.affiliation: Department of Pediatrics, National Cheng Kung University Hospital, 138 Sheng Li Road, Tainan, Taiwan.
authors.forename: Chih-Ta
authors.initials: CT
authors.lastname: Yao
authors.forename: Jieh-Neng
authors.initials: JN
authors.lastname: Wang
authors.forename: Yu-Chien
authors.initials: YC
authors.lastname: Tsai
authors.forename: Chia-Shiang
authors.initials: CS
authors.lastname: Lin
authors.forename: Jing-Ming
authors.initials: JM
authors.lastname: Wu
mesh_terms: D004359:Drug Therapy, Combination; D006327:Heart Block / Q000188:drug therapy*; D006801:Humans; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000151:congenital* / Q000188:drug therapy*; D008297:Male; D008801:Mexiletine / Q000008:administration & dosage*; D011433:Propranolol / Q000008:administration & dosage*
chemical_list: D008801:Mexiletine; D011433:Propranolol