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
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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.
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- 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
Journal of Interventional Cardiac Electrophysiology
Volume 26, Issue 1 , pp 41-45
- Cover Date
- Print ISSN
- Online ISSN
- Springer US
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- Long QT Syndrome
- Implantable cardioverter defibrillator
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- Author Affiliations
- 1. Department of Pediatrics, NY Presbyterian Hospital—Columbia University, New York, NY, USA
- 2. Department of Surgery, NY Presbyterian Hospital—Columbia University, New York, NY, USA
- 3. Departments of Medicine, Pediatrics, and Molecular Pharmacology & Experimental Therapeutics/Divisions of Cardiovascular Diseases and Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
- 4. Department of Surgery, Mayo Clinic, Rochester, MN, USA
- 5. Pediatric Arrhythmia Service, Division of Pediatric Cardiology, Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine, 3415 Bainbridge Avenue, Rosenthal 1, Bronx, NY, 10467, USA