Recurrent left main stem stenosis in a young female with Behçet's aortitis: a case report

Behçet's disease (BD) is a rare and complex vasculitis disorder renowned for its diverse clinical presentations. Cardiovascular involvement is reported to be present in 7-46% of the patients, with coronary arteries being involved in only 0.5%. The management of cardiovascular complications can be challenging due to the rarity of such cases and the absence of standardized guidelines regarding diagnosis and treatment.

We report the case of a 27-year-old patient with BD with known aortitis and pulmonary arteritis, who presented with recurrent acute coronary syndromes related to critical left main coronary artery stenosis. She was initially managed with percutaneous coronary interventions twice. Following recurrent stent failure, she eventually underwent urgent coronary artery bypass surgery, together with aortic valve replacement and aortic root repair. She made an uneventful recovery and remains well 6 months following her operation.

This case illustrates the significant challenges that can be encountered when managing coronary complications in patients with BD. Both percutaneous and surgical options have been reported in the literature with variable outcomes. Multi-disciplinary team involvement is of utmost importance in order to offer a balanced therapeutic strategy to these patients. Further research is required to shed light to the unknowns surrounding this rare cohort.