Jervell-Lange-Nielson syndrome Congential QT prolongation

• Facts:
  Autosomal recessive
  Molecular defect in ion channels
  
  
• History / PE:
  Congenital deafness
  Syncopal episodes without post-ictal state
  
  
• Diagnosis:
  QT interval prolongation
• Treatment:
  Beta blockers
• Notes:
  QT prolongation predisposes to torsades de pointes