Arrhythmias & electrophysiologyCase-control study
A Clinical Diagnostic Test for Calcium Release Deficiency Syndrome
- Case-control study
- international
- multicentre
- 10 centres in 7 countries
- genetic mouse models
- Analyses performed April 2023 to December 2023
Authors' conclusion
There is a unique repolarization response on an electrocardiogram after provocation with brief tachycardia and a subsequent pause in CRDS cases and mouse models, which is absent from the controls.
Study context
- Population
- Patients with calcium release deficiency syndrome (CRDS) and control patients, tracings recorded June 2005–December 2023
- Participants
- 10 / 45 / 10 / 3
- Comparison
- ECG repolarization response after brief tachycardia and pause: CRDS cases vs controls
- CRDSn = 10
- Suspected supraventricular tachycardian = 45reference
- UCAn = 10reference
- CPVTn = 3reference
- Outcome
- Change in QT interval and change in T-wave amplitude (postpause sinus beat vs last beat before tachycardia)
Main finding
As reported in the abstract
Change in T-wave amplitude on postpause sinus beat (after brief VT ≥150 beats/min)
Smallest change in T-wave amplitude, CRDS case
0.250 mV
Largest change in T-wave amplitude, control
0.160 mV
Discrimination
100%
Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Change in QT interval · median change longer in CRDS vs controls | P = 0.002 |
Change in QT interval
- Overlap between cases and controls present
Genetic mouse models
- Mouse models recapitulated human findings; response suggested secondary to pathologically large systolic calcium release from the sarcoplasmic reticulum
Original publication
A Clinical Diagnostic Test for Calcium Release Deficiency Syndrome. JAMA. Published 16 July 2024. doi:10.1001/jama.2024.8599
Summarised from the published abstract; every number is checked against it