Hughes Stovin Syndrome, a Rare Form of Behcet's Disease Presenting as Recurrent Intracardiac Thrombus

Abstract

Hughes Stovin syndrome (HSS) is a particularly rare disease characterized by multiple pulmonary artery and/or bronchial artery aneurysms with concomitant peripheral venous thrombosis and is believed to be a cardiovascular variant of Behcet's disease. Intracardiac thrombus occurring as a thrombotic manifestation of HSS is an unusual presentation and represents a challenge in diagnosis and treatment. Here we report a 25-year-old male presenting with recurrent right-sided intracardiac thrombi, in whom pulmonary artery aneurysm was later detected in the clinical course corroborating the diagnosis of HSS and leading to appropriate initiation of immunosuppressive agents. The patient required multiple cardiac surgeries during the clinical course for cardiovascular complications associated with recurrent cardiac thrombus. Unfortunately, the patient was readmitted a year later for massive hemoptysis secondary to pulmonary arterial aneurysm rupture requiring left lower lobectomy. Our case highlights also the significant morbidity, complications, and treatment challenges associated with this potentially life-threatening syndrome, which is intensified in the presence of cardiac involvement.

Keywords: cardiac thrombus; hughes stovin syndrome; pulmonary artery aneurysm.