Abstract 4365288: Silent Storm: Aortitis and Coronary Compression Unveiled in a Healthy Woman

Abstract

Background: Takayasu arteritis is a rare, granulomatous large-vessel vasculitis primarily affecting the aorta and its branches. Its non-specific presentation often delays diagnosis and can increase the risk of cardiovascular complications. Case Summary: A healthy 42-year-old woman with a BRCA mutation presented for breast cancer screening via a magnetic resonance imaging (MRI). This revealed cardiomegaly, for which she was referred to our hospital for workup. Her exam revealed a widened pulse pressure (70 mmHg) and a diastolic murmur at the right upper sternal border. Subsequently, a computed tomography (CT) and transthoracic echocardiogram showed perivascular fat-stranding concerning for aortitis (Figure 1A) and aortic regurgitation (AR) with left ventricular (LV) dilation and dysfunction, respectively. These findings were confirmed with a cardiac MRI (Figure 2) and transesophageal echo. Subsequent coronary CT angiogram ruled out aortic dissection but revealed 70% ostial left main (LM) coronary artery stenosis (Figure 1B), which was confirmed on coronary angiogram (Figure 3A). Given these findings, she was started on empiric steroids for presumed Takayasu arteritis after multidisciplinary discussions. Due to her severe AR associated with LV dysfunction and severe left main disease, she met Class I indications for surgical aortic valve replacement (SAVR) and coronary artery bypass grafting (CABG). A multidisciplinary discussion was held between cardiology, cardiothoracic surgery, and rheumatology regarding surgical timing given concerns for ongoing inflammation and associated surgical risks. However, her course was complicated by refractory chest pain requiring nitroglycerin infusion. After extensive discussions, via shared decision-making we proceeded with a temporizing, high-risk coronary stenting as a bridge to surgery following successful tapering of immunosuppressive therapy. She underwent a successful PCI (Figure 3B) and was initiated on dual-antiplatelet therapy. Ultimately, she was discharged on a prednisone taper and methotrexate, with plans for interval SAVR and CABG once on the lowest dose steroid. Discussion: Timely diagnosis and treatment is crucial to improving outcomes in Takayasu arteritis. Complications such as coronary artery stenosis and AR often require surgery but can be complicated by inflammation leading to friable tissue and an increased operative risk. Multidisciplinary management is key for optimal management and timing of such.