A twisted tale of Takayasu and left main stenosis

Takayasu's arteritis (TA) is a rare large-vessel vasculitis that can involve the coronary arteries, most often the ostial left main, and may present with acute coronary syndrome (ACS). Its diagnosis is challenging due to nonspecific clinical features and the rarity of coronary involvement. We report a 32-year-old female with a history of sarcoidosis and prior provoked pulmonary embolism who presented in severe distress with chest pain, hypoxemia, and shock. Initial investigations demonstrated diffuse ST depressions with aVR elevation, elevated cardiac biomarkers, and reduced ejection fraction with moderate aortic regurgitation. Coronary angiography revealed critical (90 %) ostial left main coronary artery (LMCA) stenosis. A real time heart team discussion was undertaken, and intravascular ultrasound (IVUS)-guided percutaneous coronary intervention (PCI) with mechanical circulatory support was performed successfully. Multimodality imaging demonstrated diffuse aortic wall thickening and left subclavian artery stenosis, consistent with Takayasu's arteritis. Rheumatology consultation confirmed the diagnosis, and the patient was initiated on high-dose corticosteroids and disease-modifying therapy. Over follow-up, she was transitioned to tocilizumab with normalization of inflammatory markers, resolution of aortic wall thickening, and stable moderate aortic regurgitation. This case highlights the importance of considering vasculitis as an etiology of ACS in young patients and demonstrates the feasibility of IVUS-guided PCI for LMCA involvement in Takayasu's arteritis. • A young female presented with cardiogenic shock and was found to have critical ostial left main coronary artery stenosis without traditional risk factors. • Transesophageal echocardiography revealed aortic wall thickening suggestive of underlying aortitis. • IVUS-guided PCI was performed with mechanical support as a life-saving intervention in the setting of suspected vasculitis. • Progression of vascular involvement led to a diagnosis of Takayasu arteritis based on clinical, imaging, and laboratory criteria. • The case underscores the need for vigilance in identifying non-atherosclerotic causes of ACS and highlights the role of serial imaging and multidisciplinary care in managing large-vessel vasculitis.