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A fatal case of Behçet's disease associated with multiple cardiovascular lesions
1Department of Cardiology, Rambam Medical Centre, Technion Faculty of Medicine, Haifa, Israel.
Insights
Behçet
Area of Science:
- Cardiovascular Medicine
- Rheumatology
- Vascular Surgery
Background:
- Behçet's disease is a chronic inflammatory disorder characterized by vasculitis.
- Cardiac involvement in Behçet's disease is rare but associated with a poor prognosis.
- This case highlights a rare presentation of cardiac and vascular complications in a young patient with Behçet's disease.
Observation:
- A 26-year-old male with a history of bilateral renal artery stenosis surgery presented with fever, chest pain, dyspnea, and hemoptysis.
- Imaging revealed a large right atrial thrombus, pulmonary aneurysms, and an ascending aortic pseudoaneurysm.
- Lung biopsy showed hemorrhagic infarcts and occluded pulmonary arteries.
Findings:
- The right atrial mass was surgically excised and identified as an organizing thrombus.
- Pulmonary artery aneurysms and an ascending aorta pseudoaneurysm were diagnosed.
- The patient experienced a fatal rupture of the ascending aortic pseudoaneurysm.
Implications:
- This case underscores the importance of considering Behçet's disease in young patients with unusual arterial and venous thromboses or aneurysms.
- Early recognition and management of vascular complications are crucial for improving outcomes in Behçet's disease.
- Multisystemic involvement necessitates a comprehensive diagnostic and therapeutic approach.
Abstract:
Behçet's disease is recognised as a chronic multisystem disorder with vasculitis as its underlying pathological process. Cardiac involvement is rare and often associated with poor prognosis. A large right atrial thrombus, pulmonary aneurysms and aortic pseudoaneurysm that developed 17 years after surgery for bilateral renal artery stenosis is presented in a 26-year-old Behçet's disease patient. He was admitted to the hospital with fever of unknown origin associated with chest pain, dyspnea, cough, haemoptysis and pulmonary opacity in chest X-ray. Initial pulmonary CT demonstrated small subpleural infiltrates bilaterally, one of which was round and suspected as being metastatic. Examination of open lung biopsy demonstrated haemorrhagic infarct surrounded by some occluded pulmonary arteries. Subsequent CT showed pulmonary aneurysms compatible with Behçet's disease. Echocardiography demonstrated a large pedunculated mass in the right atrium. Injection of urographin showed a right atrial mass and a large right pulmonary artery aneurysm. The atrial mass was completely excised during open heart surgery and was identified as being an organising thrombus. Eight weeks later while taking prednisone, he was readmitted because of an infected mid sternal wound. CT showed slight separation of the stemum, retrosternal fluid, pulmonary arteries aneurysm and ascending aorta aneurysm. The next day, the patient died from massive bleeding from his ruptured ascending aortic pseudoaneurysm. Bizarre presentation of arterial and venous thromboses or arterial aneurysm formation, particularly in young patients, should suggest Behçet's disease.