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Curative Pericardiectomy in Interpheron-Resistant Severe Pericardial Erdheim-Chester Disease

Javier Gómez-Herrero1, Carolina Hernández Luis1, Elena Tapia Moral2

  • 1Instituto de Ciencias del Corazón, Cardiology Department, Hospital Clínico Universitario, Valladolid, Valladolid, Spain.

JACC. Case Reports
|November 29, 2022
PubMed

Insights

Erdheim-Chester disease, a rare histiocytosis, can affect the heart. This case highlights cardiac tamponade as an unusual initial presentation requiring surgical intervention.

Area of Science:

  • Cardiology
  • Oncology
  • Histiocytosis

Background:

  • Erdheim-Chester disease is a rare clonal non-Langerhans cell histiocytosis.
  • It characteristically involves bones, large vessels, and retroperitoneum.
  • Cardiac involvement is a significant predictor of mortality.

Observation:

  • This report details an unusual case of Erdheim-Chester disease.
  • The disease presented initially with cardiac tamponade and pericardial constriction.
  • The patient required pericardiectomy for management.

Findings:

  • Cardiac tamponade and pericardial constriction can be the primary manifestation of Erdheim-Chester disease.
  • Pericardiectomy can provide definitive control for cardiac complications.
  • This presentation is atypical for Erdheim-Chester disease.

Implications:

  • Highlights the importance of considering Erdheim-Chester disease in cardiac emergencies.
  • Suggests a broader spectrum of cardiac involvement in Erdheim-Chester disease.
  • Emphasizes the role of surgical intervention in managing cardiac manifestations.

Keywords:
CT, computed tomographyECD, Erdheim-Chester diseaseMR sequencesMRI, magnetic resonance imagingPE, pericardial effusionPET, positron emission tomographyTTE, transthoracic echocardiographyaortacardiac magnetic resonancecardiovascular diseaseconstrictiveechocardiographyimagingnuclear medicinepegIFN-α, pegylated interferon-alphapericardial effusion

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