Journal of Interventional Cardiac Electrophysiology

, Volume 26, Issue 1, pp 41–45

Long QT syndrome due to a novel mutation in SCN5A: treatment with ICD placement at 1 month and left cardiac sympathetic denervation at 3 months of age

  • Eric S. Silver
  • Leonardo Liberman
  • Wendy K. Chung
  • Henry M. Spotnitz
  • Jonathan M. Chen
  • Michael J. Ackerman
  • Christopher Moir
  • Allan J. Hordof
  • Robert H. Pass
Case Report

DOI: 10.1007/s10840-009-9428-1

Cite this article as:
Silver, E.S., Liberman, L., Chung, W.K. et al. J Interv Card Electrophysiol (2009) 26: 41. doi:10.1007/s10840-009-9428-1

Abstract

We describe the case of a newborn with congenital long QT syndrome, with 2:1 AV block and frequent episodes of Torsades de Pointes (TdP) requiring placement of a dual chamber ICD at 33 days and 3.63 kg, the youngest and smallest patient, thus far reported. Long QT syndrome was diagnosed due to bradycardia in the newborn nursery, with frequent episodes of TdP. The patient was initially treated with magnesium and esmolol then given lidocaine which resulted in dramatic transient normalization of the QTc with 1:1 AV nodal conduction. An attempt to transition to oral sodium channel and beta blockade was unsuccessful. An ICD was placed and dual chamber pacing was initiated which facilitated the transition to an oral medical regimen and ultimate discharge from the hospital. Soon after placement of the ICD, genetic testing revealed a novel F1473C mutation in the SCN5A gene. Episodes of TdP continued and left stellate gangliectomy was performed at 3 months of age. At 30 months follow-up, the patient has occasional, self-limited episodes of TdP and has received rare, successful, and appropriate ICD shocks.

Keywords

PediatricLong QT SyndromeSCN5AImplantable cardioverter defibrillatorSympathectomy

Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Eric S. Silver
    • 1
  • Leonardo Liberman
    • 1
  • Wendy K. Chung
    • 1
  • Henry M. Spotnitz
    • 2
  • Jonathan M. Chen
    • 2
  • Michael J. Ackerman
    • 3
  • Christopher Moir
    • 4
  • Allan J. Hordof
    • 1
  • Robert H. Pass
    • 5
  1. 1.Department of PediatricsNY Presbyterian Hospital—Columbia UniversityNew YorkUSA
  2. 2.Department of SurgeryNY Presbyterian Hospital—Columbia UniversityNew YorkUSA
  3. 3.Departments of Medicine, Pediatrics, and Molecular Pharmacology & Experimental Therapeutics/Divisions of Cardiovascular Diseases and Pediatric CardiologyMayo ClinicRochesterUSA
  4. 4.Department of SurgeryMayo ClinicRochesterUSA
  5. 5.Pediatric Arrhythmia Service, Division of Pediatric Cardiology, Department of Pediatrics, Montefiore Medical CenterAlbert Einstein College of MedicineBronxUSA