ArticleLa Radiologia medica2023
Cardiovascular involvement in Erdheim-Chester diseases is associated with myocardial fibrosis and atrial dysfunction.
Article in La Radiologia medica, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 6 papers.
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Who cites it
6 citing papers in PubMed, 9 citations in OpenAlex.
- Erdheim-Chester disease presenting as complete heart block: A case report.HeartRhythm case reports · 2026Article
- Novel Diagnosis of Isolated Cardiac Erdheim-Chester Disease by Interatrial Biopsy Using Frozen Section Examination.Interventional cardiology (London, England) · 2025Article
- Paradoxical coronary embolism in Erdheim-Chester disease: invasive assessment and multidisciplinary management.Oxford medical case reports · 2024Article
- Erdheim‑Chester disease of multisystem involvement with delayed diagnosis: A case report and literature review.Experimental and therapeutic medicine · 2024Article
- Myocarditis in Erdheim-Chester disease.Journal of medicine and life · 2024Article
- Double Subclavian Steal Syndrome As Initial Manifestation of Erdheim-Chester Disease: A Case Report.Cureus · 2023Article
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Authors and funding
10 authors at 2 institutions in 1 country.
Funding
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Abstract
purposeErdheim-Chester disease (ECD) is a rare multisystem histiocytosis, whose cardiovascular involvement has not been systematically characterized so far. We aimed to systematically (qualitatively and quantitatively) describe the features of cardiovascular involvement in a large cohort of ECD patients and to evaluate its impact on myocardial fibrosis extension and cardiac function. MATERIAL AND
methodsAmong 54 patients with biopsy-proven ECD, 29 patients (59 ± 12 years, 79% males) underwent 1.5-T CMR using a standardized protocol for qualitative and quantitative assessment of disease localization, evaluation of atrial and ventricular function, and assessment of non-dense and dense myocardial fibrosis.
resultsThe right atrioventricular (AV) groove was the most commonly affected cardiac site (76%) followed by the right atrial walls (63%), thoracic aorta (59%), and superior vena cava (38%). Right AV groove involvement, encasing the right ventricular artery, was associated with non-dense myocardial fibrosis in the infero-septal (20/26 patients) and the inferior (14/26 patients) mid-basal left ventricular (LV) wall. In two patients with right AV groove localization, LGE revealed myocardial infarction in the same myocardial segments. Three out of five patients with left AV groove involvement had non-dense LGE on the lateral LV mid-basal wall. Bulky right atrial pseudomass was associated with atrial dysfunction and superior and inferior vena cava stenosis.
conclusionsIn ECD patients, AV groove localization is associated with LV wall fibrosis in the downstream coronary territories, suggesting hemodynamic alterations due to coronary encasement. Conversely, atrial pseudomass ECD localizations impact on atrial contractility causing atrial dysfunction and are associated with atrio-caval junction stenosis.
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