Int J Cardiovasc Imaging. 2026 May 26. doi: 10.1007/s10554-026-03733-6. Online ahead of print.
ABSTRACT
Behçet's disease (BD) is a chronic, relapsing multisystem inflammatory disorder classified as a variable-vessel vasculitis. Venous thrombosis is common, and Budd-Chiari syndrome (BCS) represents one of the most severe manifestations, whereas coronary artery involvement is rare and associated with poor prognosis. Concomitant presentation of BCS and coronary pseudoaneurysm has been rarely reported. A 23-year-old man with a history of BCS caused by thrombotic occlusion of the retrohepatic inferior vena cava (IVC), previously treated with balloon dilatation and anticoagulation, presented with acute chest pain and dyspnea. Transthoracic echocardiography revealed severe anterior wall motion abnormalities with a left ventricular ejection fraction of 25% and findings suggestive of a left anterior descending artery (LAD) pseudoaneurysm with suspected contained rupture, which were further supported by contrast-enhanced computed tomography. Coronary angiography demonstrated diffuse critical stenosis of the proximal LAD with pseudoaneurysm formation and severely impaired distal flow. Percutaneous coronary intervention with sequential stent implantation, including a covered stent, was performed to seal the pseudoaneurysm and restore coronary perfusion. Subsequent targeted history-taking revealed recurrent oral aphthous ulcers, scrotal ulceration, and lower-limb skin lesions, fulfilling the International Criteria for Behçet's Disease. Immunosuppressive therapy was initiated following revascularization, resulting in significant clinical and functional improvement during follow-up. In young patients presenting with myocardial infarction in the absence of conventional cardiovascular risk factors, particularly those with coexisting Budd-Chiari syndrome (BCS) or unexplained venous thrombosis, Behçet's disease (BD) should be strongly considered. Multimodality cardiovascular imaging is essential not only for establishing the diagnosis but also for guiding interventional strategies and subsequent immunosuppressive therapy in vascular Behçet's disease, and for prognostic assessment.
PMID:42189355 | DOI:10.1007/s10554-026-03733-6