Triadin Knockout syndrome
New cause of recessive genetic LQTS
- Presents before 5 years of age
- sudden cardiac arrest common
- Beta blockers no use
- ECG show symmetric inversion in anterior leads +/- Qt prolongation
- These may be the genotype negative cases of CPVT
- Triadin links calsequestrin, ryanodine receptor and junctophilin to L type calcium channel in myocardium
