Document Detail

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.
MedLine Citation:
PMID:  19669871     Owner:  NLM     Status:  MEDLINE    
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.
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
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2009-08-11
Journal Detail:
Title:  Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing     Volume:  26     ISSN:  1572-8595     ISO Abbreviation:  J Interv Card Electrophysiol     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-09-18     Completed Date:  2009-12-01     Revised Date:  2014-09-17    
Medline Journal Info:
Nlm Unique ID:  9708966     Medline TA:  J Interv Card Electrophysiol     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  41-5     Citation Subset:  IM    
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MeSH Terms
Combined Modality Therapy
Defibrillators, Implantable*
Infant, Newborn
Long QT Syndrome / congenital,  genetics*,  therapy*
Muscle Proteins / genetics*
NAV1.5 Voltage-Gated Sodium Channel
Polymorphism, Single Nucleotide / genetics
Sodium Channels / genetics*
Treatment Outcome
Grant Support
Reg. No./Substance:
0/Muscle Proteins; 0/NAV1.5 Voltage-Gated Sodium Channel; 0/SCN5A protein, human; 0/Sodium Channels

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