Cardiovascular Manifestations of Erdheim-Chester's Disease: A Case Series

Costa, Isabela Bispo Santos da Silva; Abdo, André; Bittar, Cristina Salvadori; Fonseca, Silvia Moulin Ribeiro; Moraes, Aline Sabrina Holanda Teixeira; Filho, Roberto Kalil; Pereira, Juliana; Hajjar, Ludhmila Abrahão

doi:10.5935/abc.20180218

Cited by 7 publications

(4 citation statements)

References 13 publications

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“…In addition, other radiological examinations could be selected according to clinical symptoms or signs [1] . Cardiovascular involvement is common in ECD patients and it is associated with a worse prognosis [6] . A contrast-enhanced cardiac MRI is optimal to evaluate the extent of myocardial and pericardial infiltration, tissue characterization of the lesion, and ventricular dysfunction.…”

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester disease with multisystem involvement evaluated by multimodal imaging: A case report

Liu

Gao

et al. 2022

Radiology Case Reports

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Erdheim-Chester disease is a rare, idiopathic, multisystemic non-Langerhans cell histiocytosis. Little is known about the imaging features. Herein, we report a very uncommon case of Erdheim-Chester disease in a 54-year-old woman with multisystem involvement including cardiovascular system, skeleton, retroperitoneum (renal and adrenal infiltration), orbit and pituitary. Multimodal imaging modalities, including computed tomography, magnetic resonance imaging, echocardiography, and bone scintigraphy were used to comprehensively evaluate different organs involvement. Finally, myocardial biopsy results indicated Erdheim-Chester disease. Electrocardiography showed sick sinus syndrome and slowest heart rate of 20 beats/min. The patient underwent permanent pacemaker implantation and had initial treatment with interferon. There were no remarkable changes in right atrial lesion during 9-month follow-up period. Erdheim-Chester disease was a rare entity with a dismal prognosis, especially when there were cardiac and neurological involvement. The present case report aimed to described and analyzed radiological findings of multiple organs involvement of Erdheim-Chester disease with multimodal imaging retrospectively, and being familiar with the imaging features of Erdheim-Chester disease might help prompt and correct diagnosis of this disease in the future.

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Section: Discussionmentioning

confidence: 99%

Erdheim-Chester disease with multisystem involvement evaluated by multimodal imaging: A case report

Liu

Gao

et al. 2022

Radiology Case Reports

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“…In this multi-systemic disease, various multi-modal imaging approaches, including CT, brain MRI, cardiac MRI, PET–CT, and bone scan, can be helpful in the diagnosis. 13 For this rare and complex disease, it is essential that clinicians know the main features of the disease so that they can perform an early and comprehensive diagnostic investigation. 14 …”

Section: Discussionmentioning

confidence: 99%

Erdheim–Chester disease presenting as an intracardiac mass and pericardial effusion confirmed by biopsy: a case report

Yoon

Lee

Shim

et al. 2021

European Heart Journal - Case Reports

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Background Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis that can affect the bones, heart, lungs, brain, and other organs. Cardiovascular involvement is common in ECD and is associated with a poor prognosis. Here, we report a case of ECD presenting as a intracardiac mass and pericardial effusion confirmed by biopsy with sternotomy. Case summary A 54-year-old man was admitted because of dyspnoea. He was previously diagnosed with bilateral hydronephrosis and retroperitoneal fibrosis. Echocardiography revealed a large amount of pericardial effusion and echogenic mass on the right atrial (RA) side and atrioventricular (AV) groove. Cardiac magnetic resonance imaging and positron emission tomography-computed tomography (CT) revealed infiltrative mass-like lesions in the RA and AV groove. Pericardial window formation and pericardial biopsy were performed, and the pathologic results showed only pericardial fibrosis with no specific findings. Bone scan revealed increased uptake in the long bones. Considering the high probability of ECD based on the patient’s manifestations and the imaging findings, we performed a cardiac biopsy with median sternotomy despite initial insufficient pathologic results in the pericardial biopsy. The surgical findings included multiple irregular and firm masses on the cardiac wall and large vessels; after obtaining a large amount of suspicious mass, ECD accompanied with CD68 (+) and BRAF V600E mutation was confirmed. Discussion ECD can be associated with various forms of cardiovascular involvement. Considering the multi-systemic manifestations and difficulty in identifying this rare disease, a comprehensive and meticulous diagnostic work-up is crucial.

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“…Myocardial involvement occurs sequentially to the pericardial involvement and manifests as myocardial hypertrophy, easily diagnosed by the echocardiogram. Thickening can be found in the ventricles, atria, coronary sulci and interatrial septum [11].…”

Section: Discussionmentioning

confidence: 99%

Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review

2020

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Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis characterized by systemic xanthogranulomatous infiltration. We described the case of a female adult presenting with pericardial effusion. Pericardial infiltration is the most frequent cardiac manifestation of ECD and is the one discussed in this article. We found that the majority of patients with pericardial infiltration needed a cardiovascular procedure.

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Cardiovascular Manifestations of Erdheim-Chester's Disease: A Case Series

Cited by 7 publications

References 13 publications

Erdheim-Chester disease with multisystem involvement evaluated by multimodal imaging: A case report

Erdheim-Chester disease with multisystem involvement evaluated by multimodal imaging: A case report

Erdheim–Chester disease presenting as an intracardiac mass and pericardial effusion confirmed by biopsy: a case report

Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review

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