Prevalence, patterns and outcomes of cardiac involvement in Erdheim–Chester disease

Azoulay, LD; Bravetti, M; Cohen-Aubart, F; Emile, JF; Seilhean, D; Plu, I; Charlotte, F; Waintraub, X; Carrat, F; Amoura, Z; Cluzel, P; Haroche, J

doi:10.1093/eurheartj/ehac741

Cardio-oncology & other

Prevalence, patterns and outcomes of cardiac involvement in Erdheim–Chester disease

Eur Heart JPublished 21 Dec 2022Retrospective cohort study

Main finding · All-cause mortality by cardiac involvementResultAdjusted hazard ratio 1.4 95% CI 0.8–2.5

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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)

Methodological detailssingle-centre, French tertiary center

Single-centre
French tertiary center

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

	← Lower odds ←→ Higher odds →	OR (95% CI)	P value
Cardiac involvementBRAFV600E mutation	0.050.21520	7.4 (3.5–16.8)	P < 0.001
Cardiac involvementECD-related clinical events	0.050.21520	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

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