Rheumatoid arthritis and multisite cardiac aneurysms involving the LVOT, valves, and intervalvular Fibrosa: A case-based systematic review

Cardiac involvement in rheumatoid arthritis (RA) is an uncommon but clinically relevant manifestation that may extend beyond pericardial and valvular disease to involve peri-annular and subvalvular fibrous structures. Aneurysmal and pseudoaneurysmal complications affecting the left ventricular outflow tract (LVOT), aortic root, and adjacent regions are exceptionally rare and largely described in isolated case reports. We performed a systematic review of the reported cases to characterize the clinical, imaging, pathological, and therapeutic features of RA-associated aneurysmal or pseudoaneurysmal cardiac involvement. The review identified a small number of reported cases in which RA-related inflammation involved peri-annular structures, often mimicking infective endocarditis and frequently associated with significant valvular dysfunction. Across cases, multilevel structural involvement, including the aortic root, LVOT, membranous interventricular septum, and cardiac valves, was common. High-grade atrioventricular conduction disturbances were observed in a substantial proportion of patients, reflecting the close anatomical relationship between inflammatory lesions and the cardiac conduction system. Multimodality imaging, combining transthoracic and transesophageal echocardiography with cardiac computed tomography, cardiac magnetic resonance, or FDG-PET/CT, was consistently required to define anatomical extent and guide management. Histopathological examination, when available, proved essential to exclude infection and demonstrated granulomatous inflammation or rheumatoid nodules in autoimmune-mediated cases. Most patients required surgical intervention due to advanced structural disease, while isolated inflammatory involvement without aneurysmal degeneration responded to immunosuppressive therapy. Outcomes were generally favorable. This review underscores that RA-related cardiac inflammation may predispose to aneurysmal degeneration of peri-annular fibrous structures, with complex presentations in which a complete diagnostic pathway, including multimodality imaging and pathological confirmation is critical.