Congenital Long QT Syndrome
February 15th, 2021
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
| LQT1 | LQT2 | LQT3 | |
| ECG Morphology | Broad T waves | Bifid T waves | Late T waves |
| Trigger | Exertion, fright, swimming | Auditory | Sleep / rest |
Next Page
----------------------------------------------------------------------------------------------------------------------------------------------------------------------------