Heart failure & cardiomyopathiesCohort study
Disease progression in arrhythmogenic cardiomyopathy: a cardiac magnetic resonance longitudinal study
- Retrospective cohort study
- longitudinal CMR
Authors' conclusion
MF and right ventricular systolic dysfunction emerge as independent predictors of arrhythmic risk in ACM. Serial CMR assessments demonstrate that structural progression is common, supporting the concept that ACM represents a dynamic rather than a static disease process.
Study context
- Population
- Individuals with definite arrhythmogenic cardiomyopathy or carriers of P/LP arrhythmogenic cardiomyopathy (ACM)-related variants, with at least one CMR97 with ≥2 CMR
- Participants
- 202 individuals
- Exposure
- Higher-exercise vs Lower-exercise
- Outcome
- Composite of:
- Major ventricular arrhythmias
- Appropriate implantable cardioverter-defibrillator (ICD) interventions
- Sudden cardiac arrest
- SCD
- Follow-up
- Mean 5.2 years
Main finding
As reported in the abstract
Primary composite endpoint:
- Major ventricular arrhythmias (MVA)
- Appropriate ICD interventions
- Sudden cardiac arrest
- SCD
Each group vs lower-exerciseHazard ratio (95% CI)P value
Baseline myocardial fibrosis
4.9(1.8–12.9)
P < 0.001reduced right ventricular ejection fraction
2.6(1.2–5.4)
P = 0.002- Patients reaching the primary endpoint
- 32 (16%)
Observational data: associations, not effects.
Further findings
Structural progression (≥2 CMR, n = 97)
- De novo fibrosis
- 32%
- Progression of myocardial fibrosis
- 41%
- Longer intervals between CMR scans: greater increases in biventricular end-diastolic volumes and progressive declines in ventricular function
LV remodelling by exercise
- Significant LV dilation in the higher-exercise group, with increase in indexed end-diastolic volumes
Arrhythmic events in PKP2 carriers
- PKP2 carriers engaging in moderate-to-intense exercise had a higher incidence of arrhythmic events
Original publication
Disease progression in arrhythmogenic cardiomyopathy: a cardiac magnetic resonance longitudinal study. Eur J Prev Cardiol. Published 13 March 2026. doi:10.1093/eurjpc/zwag130
Summarised from the published abstract; every number is checked against it