Triadin knockout syndrome

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

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