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Cardiovascular manifestations of Erdheim-Chester disease: A narrative review with two cases
Alan Wong1, Aditya Sharma1, Randy Ramcharitar1
1Department of Vascular Medicine, Division of Cardiovascular Medicine, University of Virginia School of Medicine, Charlottesville, VA, USA.
Insights
Erdheim-Chester disease (ECD), a rare histiocytic neoplasm, frequently impacts the cardiovascular system. Further research is needed on cardiovascular ECD outcomes and treatment responses.
Area of Science:
- Oncology
- Cardiology
- Rare Diseases
Background:
- Erdheim-Chester disease (ECD) is a rare histiocytic neoplasm affecting multiple organ systems.
- Cardiovascular involvement is common in ECD, occurring in up to 70% of patients.
- Manifestations range from asymptomatic findings to severe cardiac events like tamponade and heart failure.
Purpose of the Study:
- To review the cardiovascular manifestations of Erdheim-Chester disease.
- To highlight the diagnostic imaging findings of cardiac involvement in ECD.
- To underscore the need for more data on cardiovascular ECD outcomes and treatment efficacy.
Main Methods:
- Literature review of published cardiovascular ECD cases.
- Analysis of imaging findings (CT/MRI) in patients with ECD.
- Synthesis of clinical presentations and known outcomes.
Main Results:
- Cardiovascular involvement is prevalent in ECD, presenting with diverse symptoms and imaging findings.
- Common findings include periarterial thickening of large arteries (aorta), stenosis, and cardiac structures like pseudotumors and effusions.
- Reported cardiovascular complications include pericarditis, cardiac tamponade, myocardial infarction, and heart failure.
Conclusions:
- Cardiovascular involvement is a significant feature of Erdheim-Chester disease, requiring thorough evaluation.
- Further research is crucial to understand long-term outcomes and optimal treatment strategies for cardiovascular manifestations of ECD.
Abstract:
Erdheim-Chester disease (ECD) is a rare 'L' (Langerhans) group histiocytic neoplasm that affects a multitude of organ systems, causing osteosclerotic bone lesions, periaortic encasement ('coated' aorta), retroperitoneal fibrosis involving kidneys and ureters ('hairy kidney'), and infiltration of the central nervous system. Cardiovascular involvement can occur in up to 70% of patients and is usually found during computed tomography/magnetic resonance imaging evaluation. When present, cardiovascular symptoms can have wide variability in presentation from asymptomatic to pericarditis, fatal cardiac tamponade, myocardial infarction, conduction abnormalities, heart failure, renal artery stenosis, and claudication. Cardiac involvement found on imaging includes right atrial pseudotumor, right atrioventricular groove infiltration, and pericardial effusions. ECD can involve the large- and medium-sized arteries, often seen as periarterial thickening (commonly coating the aorta) with stenosis/occlusion. Although more cardiovascular ECD cases have begun to be published in the literature, more data are needed on the outcomes of these patients, as well as how cardiovascular manifestations respond to treatment of ECD.
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