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

ABC-VT score

A new score to help predict adverse events in patients with frequent premature ventricular contractions (PVCs).

In the study predictors of adverse remodeling were:

  • non-sustained VT) (odds ratio [OR], 5.3)
  • longer PVC coupling interval of at least 500 ms (OR, 4.7)
  • PVC burden (OR, 4.4 for >20%; OR, 3.5 for 10% to 20%)
  • Superiorly directed PVC axis (OR, 2.7)

These parameters were scored and scores from 0 to 4 were classified as low risk; from 5 to 8, intermediate risk; and from 9 to 12, high risk.


  1. Doesn’t include patients with low coupling interval prone for R on T phenomena
  2. PVC dispersion not included in score
  3. LV remodelling assessment by EF and correlating with endpoint may not be fool proof


Voskoboinik, Aleksandr et al.Predictors of Adverse Outcome in Patients with Frequent Premature Ventricular Complexes: the ABC-VT Risk Score. Heart Rhythm

“Equally Bicuspid” aortic valve

By far BAV is the most common congenital heart disease

Angiographically two types of BAV can be identified-Equally bicuspid and unequal

Interestingly Coarctation is associated with an “equally bicuspid ” aortic valve

It may be denoting a different pathogenetic process altogether

Ref :Catheterization and Cardiovascular Diagnosis 10:17-25 (1984)