Congenital Long QT Syndrome: Illness Script
Who?
- Kids or young adults, usually < 30
- high prevalence - 1/2000 live births
Symptoms:
- Syncope
- Seizure like episodes (may be misdiagnosed)
- Sudden cardiac arrest/death
- Symptoms mostly due to polymorphic VT (Torsades) or vFib
**Get baseline EKG in new syncope/seizures and consider long term monitor**
Triggers:
- Loud noises or emotional stress
- Exercise
- Electrolyte imbalances (especially low magnesium !)
- QT prolonging meds
Why?
- Various channel mutations w/prolongation of repolarization
• Three definite sub-types + others
• May be autosomal dominant OR recessive w/ variable penetrance (ie, may or may not have a family history)
Treatment?
- Beta-blockers
- Treat electrolyte abnormalities
- Avoid QTc prolonging meds
- Possible AICD (if refractory post-arrest or very long QTc)
- Refer to specialist
#Congenital #LongQT #Syndrome #Diagnosis #Cardiology
