A Case of Vascular Behcet's Leading to Cardiogenic Shock Requiring VA-ECMO

Abstract

<h3>Introduction</h3> Vascular Behcet's (VB) is a rare inflammatory disorder with venous and less commonly arterial involvement. Coronary arteries are rarely involved. High clinical suspicion is required to make the diagnosis when the mucocutaneous manifestations of Behcet's are absent. <h3>Case Report</h3> A 45-year-old Portuguese male with prior unprovoked venous thromboembolism at the age of 30 presented with ST-elevation myocardial infarction and cardiogenic shock. Coronary angiography showed a large left main (LM) aneurysm with thrombus <i>(Figure 1A)</i>. He underwent LM balloon angioplasty and coronary artery bypass grafting. Postoperatively, he was placed on peripheral venoarterial-extracorporeal membrane oxygenation for cardiogenic shock with biventricular failure, as a bridge to decision. An intraoperative right ventricular biopsy showed only fibrinous pericarditis. He required septostomy for left ventricular decompression. Despite therapeutic heparinization, he was diagnosed with bilateral pulmonary emboli and occlusion of the common hepatic artery proper with suspected porta hepatis arterial aneurysm <i>(Figure 1B-C)</i>. He was found to carry the HLA-B51 allele and thus VB was favoured as a unifying diagnosis. Intravenous solumedrol was administered, but the patient developed anuric renal failure and limb ischemia. He ultimately succumbed to his illness due to septic shock with multiorgan failure. An autopsy confirmed the coronary aneurysm (<i>Figure 1D</i>) but could not confirm etiology. The hepatic aneurysm was not confirmed on autopsy due to technical challenges. <h3>Summary</h3> VB can lead to cardiac complications such as coronary aneurysms or thrombosis which may be the initial presentation in a smaller proportion of patients. Though immunosuppression is the mainstay of treatment, there is no established protocol and mortality/morbidity remain high. VB should be included in the differential diagnosis of a young patient presenting with coronary aneurysms to prompt appropriate therapy, though prognosis remains reserved.