Cardiovascular manifestations of Erdheim-Chester disease: A narrative review with two cases

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.