Behcet’s Disease: An Uncommon Cause of Severe Tricuspid Stenosis

Behçet’s disease is a vasculitic condition of unknown etiology that is characterized by oral and genital ulcers as well as various skin and ocular lesions. Cardiovascular manifestations of Behçet’s disease are rare, with very few cases having been reported previously in literature. We report a case of severe tricuspid stenosis and pulmonary artery aneurysm in a 29-year-old man with Behçet’s disease, who demonstrated characteristic vascular findings on computed tomography angiography and diagnostic valvular findings on transthoracic echocardiogram and cardiac magnetic resonance imaging. The patient’s Behçet’s disease was treated initially with cyclophosphamide, azathioprine, and prednisone, which subsequently led to complete resolution of the pulmonary artery aneurysm. As for the tricuspid stenosis, though symptoms were managed with diuretic therapy, the severity of valvular dysfunction required consideration and an attempt at tricuspid valve replacement surgery, which unfortunately was met with complications and led to an unfavorable outcome of refractory cardiogenic shock and death. Given the rarity of cardiovascular involvement in patients with Behçet’s disease, along with the lack of clear treatment guidelines, management of findings of tricuspid stenosis and pulmonary artery aneurysm in these patients can be challenging.