Pediatric Cardiology

, Volume 34, Issue 8, pp 1955–1962 | Cite as

Fetal Long QT Syndrome Manifested as Atrioventricular Block and Ventricular Tachycardia: A Case Report and a Review of the Literature

  • Sanitra Anuwutnavin
  • Prapat Wanitpongpan
  • Paweena Chungsomprasong
  • Jarupim Soongswang
  • Nattinee Srisantiroj
  • Tuangsit Wataganara
Case Report


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.


Atrioventricular block Fetal long QT syndrome Fetal therapy Prenatal diagnosis Ventricular tachycardia 


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© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Sanitra Anuwutnavin
    • 1
  • Prapat Wanitpongpan
    • 1
  • Paweena Chungsomprasong
    • 2
  • Jarupim Soongswang
    • 2
  • Nattinee Srisantiroj
    • 1
  • Tuangsit Wataganara
    • 1
  1. 1.Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Faculty of Medicine Siriraj HospitalMahidol UniversityBangkokThailand
  2. 2.Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine Siriraj HospitalMahidol UniversityBangkokThailand

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