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.

Limitations

  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

Ref-

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)