Document Type

Conference Proceeding

Publication Date

5-1-2026

Abstract

Introduction Behçet’s disease (BD) is a chronic multisystem vasculitis characterized by recurrent mucocutaneous, ocular, vascular, and neurologic manifestations. Cardiac involvement is rare but can be severe, with intracardiac thrombosis representing an uncommon and potentially life-threatening complication. Management is challenging, as first-line medical therapy may be limited by bleeding risk or hemodynamic compromise. Case Presentation A 26-year-old woman with a 9-year history of Behçet’s disease presented with two weeks of progressive dyspnea, palpitations, fever, fatigue, and cough. She denied chest pain, or syncope, or lower extremity swelling. One year prior, she had been hospitalized for pulmonary embolism and was found to have a right interlobar pulmonary artery aneurysm, for which she received immunosuppressive therapy. On presentation, vital signs were stable. Cardiovascular and pulmonary examinations were unremarkable, with no murmurs, jugular venous distension, or peripheral edema. Laboratory studies revealed elevated inflammatory markers, leukocytosis, mild anemia, and normal renal function. Cardiac biomarkers were within normal limits, and blood cultures showed no growth. Transthoracic echocardiography demonstrated a large, mobile right atrial mass measuring 2.5 × 4 cm, attached to the free atrial wall and prolapsing through the tricuspid valve during diastole, causing significant tricuspid inflow obstruction with a mean gradient of 9.2 mmHg. Additional findings included moderate tricuspid regurgitation, right atrial dilation, and a dilated inferior vena cava. Cardiac CT confirmed a right atrial filling defect measuring 5.1 × 2.7 cm. Differential diagnoses included infective endocarditis, atrial tumor, and intracardiac thrombus. Given the patient’s history of pulmonary artery aneurysm, anticoagulation and thrombolytic therapy were withheld due to high bleeding risk. Broad-spectrum antibiotics were initiated empirically while awaiting culture results. Due to progressive obstructive physiology and diagnostic uncertainty, urgent surgical excision of the mass was pursued. Intraoperatively, the mass was adherent to the atrial wall without invasion and was completely excised. Histopathological examination revealed an organized thrombus with fibrin deposition and inflammatory infiltrates, consistent with Behçet’s disease–related thrombosis. Discussion Intracardiac thrombosis in Behçet’s disease most commonly affects the right heart. Medical management with aggressive immunosuppressive therapy, often combined with anticoagulation, is considered first-line treatment in hemodynamically stable patients without bleeding risk. Several reports describe resolution of intracardiac thrombi with immunosuppressive therapy alone. Management becomes challenging when standard medical therapy is contraindicated. In patients with pulmonary artery aneurysms, anticoagulation carries a significant risk of fatal hemoptysis and is generally avoided. Additionally, large or mobile thrombi causing valvular obstruction may result in hemodynamic compromise. Although surgical intervention is typically reserved for select cases due to concerns about recurrence and postoperative complications, it may be necessary in patients with severe obstruction or diagnostic uncertainty. In this case, the combination of tricuspid inflow obstruction and contraindication to anticoagulation necessitated surgical management. Conclusion This case highlights right atrial thrombosis as a rare but critical manifestation of Behçet’s disease. Internists should maintain a high index of suspicion for intracardiac thrombus in patients with Behçet’s disease presenting with cardiopulmonary or constitutional symptoms. Management should be individualized, with multidisciplinary collaboration to balance the risks of thrombosis, bleeding, and surgical intervention.

Comments

American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2026 Resident and Medical Student Day, May 1, 2026, Troy, MI

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