Cardio-oncology & otherCohort study
Prevalence, patterns and outcomes of cardiac involvement in Erdheim–Chester disease
- Retrospective cohort study
- single-centre
- French tertiary center
Authors' conclusion
Cardiac involvement is present in nearly half of ECD patients and is associated with BRAFV600E mutation and complications (pericarditis, cardiac tamponade, and conduction disorders) but not with lower survival.
Study context
- Population
- Patients with Erdheim-Chester disease (ECD) who underwent cardiac magnetic resonance (CMR), French tertiary center, 2003–2019
- Participants
- 200 patients
- Exposure
- Cardiac involvement in ECD and its association with mutation status, clinical events and survival
- Outcome
- All-cause mortality
- Follow-up
- Median 5.5 years (3.3-9 years)
Main finding
All-cause mortality by cardiac involvement
Adjusted hazard ratio
1.4
95% CI 0.8–2.5
P = 0.2
Cardiac involvement
adjusted HR 1.4 (95% CI 0.8–2.5); P = 0.2
As reported in the abstract
Observational data: associations, not effects.
Secondary outcomes
Association of cardiac involvement with BRAFV600E mutation and ECD-related clinical events
| Comparison | OR (95% CI) | P value | |
|---|---|---|---|
| Cardiac involvement · BRAFV600E mutation | 7.4 (3.5–16.8) | P < 0.001 | |
| Cardiac involvement · ECD-related clinical events | 5 (1.5–21.2) | P = 0.004 | |
| 0.050.21520Odds ratio (log scale) |
Cardiac involvement (prevalence)
- Cardiac involvement
- 96 patients (48%)
CMR findings
- Right atrioventricular sulcus infiltration
- 37%
- Pericardial effusion
- 24%
Pericarditis, cardiac tamponade, conduction disorders, coronary artery disease (CAD)
patients
- Pericarditis (7 acute, 1 constrictive)
- 8 (4%)
- Cardiac tamponade
- 10 (5%)
- ECD-related high-degree conduction disorders
- 5 (2.5%)
- CAD
- 45 (23%)
Original publication
Prevalence, patterns and outcomes of cardiac involvement in Erdheim–Chester disease. Eur Heart J. Published 21 December 2022. doi:10.1093/eurheartj/ehac741
Summarised from the published abstract; every number is checked against it