Abstract
Malignant tumors of the aortic valve are extremely rare and challenging to diagnose. Here, we present a case of a 59-year-old woman with an intimal sarcoma of the aortic valve that led to acute aortic regurgitation (AR) and recurrent acute coronary syndrome (ACS). The patient initially presented with an out-of-hospital cardiac arrest due to ACS and underwent emergent percutaneous coronary intervention. Despite revascularization and medical therapy, she later developed severe AR and multivessel coronary artery disease, requiring coronary artery bypass grafting (CABG) and aortic valve replacement (AVR). Histopathologic examination confirmed aortic intimal sarcoma, which had remained undiagnosed preoperatively. Although rare, malignant tumors should be considered a potential cause of ACS, particularly in patients with minimal risk factors.