Advanced arrhythmias and channelopathies - long QT syndrome
Description
- Cardiac repolarisation disorder -> prolonged QT -> early afterdepolarisations -> torsades de pointes -> VF/sudden death
- Congenital (channelopathy) or, far more commonly, acquired/drug-induced
- Presents as syncope, seizure (often misdiagnosed as epilepsy for years), or sudden cardiac death
- Romano-Ward - autosomal dominant, cardiac only (the great majority)
- Jervell and Lange-Nielsen - autosomal recessive, congenital sensorineural deafness, homozygous KCNQ1/KCNE1, very long QT, high event rate
- Andersen-Tawil (LQT7) - periodic paralysis, dysmorphism, prominent U waves
- Timothy (LQT8) - CACNA1C, syndactyly, autism, multisystem
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