Fetal long QT syndrome manifested as atrioventricular block and ventricular tachycardia: A case report and a review of the literature

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 prolon...

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Bibliographic Details
Main Authors: Sanitra Anuwutnavin, Prapat Wanitpongpan, Paweena Chungsomprasong, Jarupim Soongswang, Nattinee Srisantiroj, Tuangsit Wataganara
Other Authors: Mahidol University
Format: Article
Published: 2018
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Online Access:https://repository.li.mahidol.ac.th/handle/123456789/32054
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Institution: Mahidol University
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Summary: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. © 2012 Springer Science+Business Media, LLC.