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Giant right ventricular thrombus as the revealing form of Behçet's disease
Anass A Maaroufi1, Sara S Abouradi2, Salah S Hayar2
1Cardiology Department, Ibn Rochd University Hospital, 20250, Casablanca, Morocco. m.anass1702@gmail.com.
Insights
A rare cardiac manifestation of Behçet disease (BD) presented as a giant intracavitary thrombus in a teenager. Early diagnosis via echocardiography and prompt treatment with anticoagulants and immunosuppressants are crucial for managing this life-threatening condition.
Area of Science:
- Cardiology
- Rheumatology
- Pathology
Background:
- Behçet disease (BD) is a rare multisystemic inflammatory disorder.
- Cardiac involvement in BD is uncommon but can be severe.
- This case highlights a giant intracavitary thrombus as the initial presentation of BD.
Purpose of the Study:
- To report a rare case of cardiac involvement in Behçet disease.
- To emphasize the diagnostic utility of echocardiography in intracardiac thrombus.
- To discuss the management of cardiac thrombus in the context of BD.
Main Methods:
- A 15-year-old male presented with dyspnea and hemoptysis.
- Echocardiography revealed a large right ventricular mass; CT angiography showed pulmonary embolism.
- Surgical removal and pathological examination confirmed a fibrin thrombus; BD was diagnosed based on clinical history and a positive pathergy test.
Main Results:
- A giant intracavitary thrombus was identified as the cause of symptoms.
- The patient's Behçet disease was confirmed through clinical criteria and pathergy testing.
- Treatment with anticoagulants, immunosuppressants, and corticosteroids proved effective in preventing recurrence.
Conclusions:
- Cardiac involvement in BD, though rare, can be life-threatening and may be initially undiagnosed.
- Intracardiac thrombus is an uncommon complication of BD, with limited reported cases.
- Echocardiography is the primary diagnostic modality for intracardiac thrombus in suspected BD patients.
Background:
Behçet's disease BD is a rare multisystemic disease, with rare cardiac involvement. This case illustrates a rare cardiac involvement as a giant intracavitary thrombus which was the revealing form of Behçet disease.
Case Presentation:
An 15-year-old male admitted to the emergency department for progressive dyspnoea, hemoptysis for which an echocardiogram displayed a large echogenic mass in the right ventricle and angio CT revealed associated bilateral pulmonary embolism. The patient was then proposed for surgery for removal and pathological study the later confirmed its fibrin thrombotic nature. Behçet disease was suspected based on past history of recurrent oral aphthosis and confirmed with a positive pathergy test. Further management by anticoagulants, immunosuppressants and corticosteroids seemed effective to avoid relapse.
Conclusions:
Cardiac involvement during BD can be life-threatening as it is not always diagnosed in timely manner. However, intracardiac thrombus is uncommon with only few case reports. Echocardiography is the key tool for the diagnosis of intracardiac thrombus.
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