title: Fetal long QT syndrome manifested as atrioventricular block and ventricular tachycardia: a case report and a review of the literature.
abstract: Fetal onset of congenital long QT syndrome (LQTS) is a rare manifestation, and prenatal diagnosis is difficult. This report describes a boy who presented with both atrioventricular (AV) block and ventricular tachycardia during the antenatal period. The early postnatal electrocardiogram showed prolongation of the QT interval and AV block, subsequently leading to a polymorphic ventricular tachycardia torsade de pointes. This unique feature of congenital LQTS has a poor outcome, but the boy was successfully treated with beta-blockers and implantation of an automated cardioverter-defibrillator. The intrauterine manifestation of fetal AV block and ventricular tachycardia should raise a high suspicion of congenital LQTS, and the strong association with a malignant clinical course should warrant special evaluation. The literature on the prenatal diagnosis, fetal therapy, and neonatal outcome of this condition also are reviewed.
authors.affiliation: Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Prannok Road, Bangkoknoi, Bangkok, 10700, Thailand, asanitra@hotmail.com.
authors.forename: Sanitra
authors.initials: S
authors.lastname: Anuwutnavin
authors.forename: Prapat
authors.initials: P
authors.lastname: Wanitpongpan
authors.forename: Paweena
authors.initials: P
authors.lastname: Chungsomprasong
authors.forename: Jarupim
authors.initials: J
authors.lastname: Soongswang
authors.forename: Nattinee
authors.initials: N
authors.lastname: Srisantiroj
authors.forename: Tuangsit
authors.initials: T
authors.lastname: Wataganara
mesh_terms: D000293:Adolescent; D054537:Atrioventricular Block / Q000175:diagnosis / Q000196:embryology / Q000209:etiology*; D003937:Diagnosis, Differential; D004562:Electrocardiography; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008133:Long QT Syndrome / Q000150:complications / Q000175:diagnosis / Q000196:embryology*; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis; D017180:Tachycardia, Ventricular / Q000150:complications / Q000175:diagnosis / Q000196:embryology*