Multimodality imaging showing complete cardiovascular involvement by Erdheim–Chester disease

Alharthi, Mohsen; Calleja, Anna; Panse, Prasad M.; Appleton, Christopher P.; Jaroszewski, Dawn E.; Tazelaar, Henry D.; Mookadam, Farouk

doi:10.1093/ejechocard/jeq052

Cited by 34 publications

(43 citation statements)

References 9 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…34,35 Diffuse infiltration of the myocardium or interatrial septum has been described, occasionally leading to heart failure. 41,42 Involvement of intrahepatic veins causing portal hypertension as well as the mesenteric vessels leading to ischemia have been seen but not published.…”

Section: Consensus Guidelines For Erdheim-chester Disease 485mentioning

confidence: 99%

Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Diamond

Dagna

Hyman

et al. 2014

Blood

516

695

View full text Add to dashboard Cite

Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis. Recent findings suggest that ECD is a clonal disorder, marked by recurrent BRAFV600E mutations in >50% of patients, in which chronic uncontrolled inflammation is an important mediator of disease pathogenesis. Although ∼500 to 550 cases have been described in the literature to date, increased physician awareness has driven a dramatic increase in ECD diagnoses over the last decade. ECD frequently involves multiple organ systems and has historically lacked effective therapies. Given the protean clinical manifestations and the lack of a consensus-derived approach for the management of ECD, we provide here the first multidisciplinary consensus guidelines for the clinical management of ECD. These recommendations were outlined at the First International Medical Symposium for ECD, comprised of a comprehensive group of international academicians with expertise in the pathophysiology and therapy of ECD. Detailed recommendations on the initial clinical, laboratory, and radiographic assessment of ECD patients are presented in addition to treatment recommendations based on critical appraisal of the literature and clinical experience. These formalized consensus descriptions will hopefully facilitate ongoing and future research efforts in this disorder. (Blood. 2014;124(4):483-492)

show abstract

Section: Consensus Guidelines For Erdheim-chester Disease 485mentioning

confidence: 99%

Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Diamond

Dagna

Hyman

et al. 2014

Blood

516

695

View full text Add to dashboard Cite

show abstract

“…Infiltration of the cardiovascular system can manifest itself as pericardial effusion, pericardial constriction, tamponade, congestive heart failure, right atrial pseudotumoral infiltration, valvular disorders, myocardial infarction due to coronary artery involvement, periaortic fibrosis, and renovascular hypertension due to renal artery stenosis. 4 Although the cardiovascular complications have a severe prognosis in ECD patients, 5 the symptoms can be subclinical and be overlooked unless a systematic cardiac evaluation is conducted. 5,6 In ECD, the mediastinal inf iltration often starts around the aorta and aortic branches.…”

Section: Fig 2 Multidetector Computed Tomographic Images A) Reformamentioning

confidence: 99%

Multidetector Computed Tomographic Imaging of Erdheim-Chester Disease

Yüceler

Kantarcı

Karabulut

et al. 2014

Texas Heart Institute Journal

View full text Add to dashboard Cite

E rdheim-Chester disease (ECD) is a rarely reported form of multisystemic, non-Langerhans-cell histiocytosis. The clinical manifestations range from no symptoms to life-threatening conditions. Involvement of the bones, lungs, retro-orbital space, perirenal space, nervous system, cardiovascular system, and skin has particularly been observed. The cardiovascular involvement of ECD is probably more frequent than was originally thought; technological advances in multidetector computed tomography (MDCT) have enabled better views of patients' coronary arteries at low radiation doses. We report the case of a young woman in whom we used 256-slice, dual-source MDCT with high pitch value to evaluate the coronary arteries and reveal the effects of ECD. Case ReportIn April 2013, a previously healthy 29-year-old woman was referred to our hospital with a 3-month history of progressive angina. She had no history of other medical illnesses or familial diseases. Results of physical examination, laboratory tests, electrocardiography (ECG), and echocardiography were normal. To analyze her coronary arteries, we used a 256-slice Somatom ® Definition Flash MDCT system (Siemens Medical Solutions; Forchheim, Germany) with a high pitch value of 3.2. The patient's effective radiation dose was 1.1 mSv for the cardiac imaging. The MDCT angiogram revealed large mediastinal soft tissue with muscle attenuation surrounding the ascending aorta, extending into the proximal segment of the left main coronary artery, and causing stenosis by compressing the proximal segment of the left anterior descending coronary artery (Figs. 1 and 2). This periaortic effect resembles "coated aorta," associated with ECD. A specimen from a computed tomographic-guided biopsy of the mediastinal mass displayed fibrosis and many foamy histiocytes (CD68 + and CD1a -). The imaging and histologic findings supported the diagnosis of ECD. Skeletal radiographs showed no bone involvement, and thoracoabdominal computed tomograms and magnetic resonance images of the brain and orbit revealed no effect on those organs. The patient was referred for hemato-oncologic treatment. She was subsequently lost to follow-up. DiscussionTo our knowledge, this is the first report of the use of low-dose, dual-source, 256-slice multidetector computed tomography to characterize Erdheim-Chester disease

show abstract

“…Thus the management of these is based on anecdotal case reports. Various approaches have been tried, these include repeated pericardiocentesis, failing which there is pericardial window and, finally, surgical pericardiectomy 1 18 19. Our patient, in spite of having multiple pericardiocentesis and pericardial window, continued to experience clinical deterioration.…”

Section: Discussionmentioning

confidence: 82%

Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

et al. 2015

View full text Add to dashboard Cite

Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disorder characterised by the proliferation of lipid laden histiocytes along with infiltration of various organs of the body. Although commonly presenting with bone pains secondary to bony infiltration, cardiac involvement in the form of periaortic fibrosis and pericardial involvement may be seen in a subgroup of patients. We report a case of ECD presenting as recurrent pericardial effusion along with pericardial tamponade.

show abstract

Multimodality imaging showing complete cardiovascular involvement by Erdheim–Chester disease

Cited by 34 publications

References 9 publications

Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease

Multidetector Computed Tomographic Imaging of Erdheim-Chester Disease

Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

Contact Info

Product

Resources

About