Heart failure & cardiomyopathiesCohort study
Cardiac MRI-Derived Right Ventricular Strain for Risk Stratification in Pediatric and Adolescent Arrhythmogenic Cardiomyopathy
- Multicentre cohort study
- multicenter
- consecutive patients
Authors' conclusion
Pediatric and adolescent ACM manifests a malignant phenotype with high burden of heart failure and arrhythmia. RVGLS provides independent and incremental prognostic value beyond conventional CMR markers and could improve risk stratification in this high-risk population.
Study context
- Population
- Consecutive pediatric and adolescent patients (≤21 years) with arrhythmogenic cardiomyopathy who underwent baseline cardiac magnetic resonance (CMR)plus 95 controls
- Participants
- 102 participants
- Exposure
- Reduced vs preserved CMR-derived right ventricular global longitudinal strain (RVGLS)
- Outcome
- Severe cardiac outcomes:
- Composite of malignant ventricular arrhythmias
- Heart transplantation
- HF-related death
- Follow-up
- median 58-month follow-up
Main finding
Severe cardiac outcomes · median 58 months
Hazard ratio
1.14
P = 0.004
As reported in the abstract
- Patients with severe cardiac outcomes
- 51 (50%)
- RVGLS ≥−12.2% identified higher risk (Kaplan-Meier)
- log-rank P < 0.001
Observational data: associations, not effects.
Further findings
| Outcome | subdistribution HR | P value |
|---|---|---|
| Malignant ventricular arrhythmia outcomes · median 58 months · adjusted for clinical and conventional CMR parameters | 1.15 | P < 0.001 |
- Malignant ventricular arrhythmia outcomes — Patients with MVA outcomes
- 28 (27.5%)
- Malignant ventricular arrhythmia outcomes — RVGLS ≥−12.2% identified higher MVA risk (Kaplan-Meier)
- log-rank P < 0.001
Original publication
Cardiac MRI-Derived Right Ventricular Strain for Risk Stratification in Pediatric and Adolescent Arrhythmogenic Cardiomyopathy. JACC Cardiovasc Imaging. Published 18 July 2026. doi:10.1016/j.jcmg.2026.05.018
Summarised from the published abstract; every number is checked against it