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ECG Disease Patterns
 
ECG Disease Patterns
 

Congenital Long QT Syndrome

 
 
 
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Overview

Several hereditary conditions are associated with a prolonged QT interval, which increases the risk of life-threatening arrhythmia.
A prolonged QT interval represent delayed ventricular repolarisation, and increases the risk of a re-entry circuit from froming (i.e. Torsade de Pointes).
  • Congenital Long QT Syndromes

  • Romano-ward syndrome (LQT1-6) - isolated prolonged QT precipitated by various triggers
  • Jervell and Lange-Nielsen syndrome (JLNS)
  • Anderson-Tawil syndrome (LQT7) - prolonged QT, periodic paralysis, facial abnormalities
  • Timothy syndrome (LQT8) - prolonged QT, AV block, congenital heart disease, developmental delay, dysmorphic features
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LQT1LQT2LQT3
ECG MorphologyBroad T wavesBifid T wavesLate T waves
TriggerExertion, fright, swimmingAuditorySleep / rest
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