Immunoglobulin G4-Related Aortitis and Severe Aortic Valve Stenosis Treated With Transcatheter Aortic Valve Replacement and Immunosuppression

Abstract

IgG4-related disease is an immune-mediated fibro-inflammatory disorder with multisystemic involvement. Aortitis and peri-aortitis are the most common cardiovascular manifestations of the disease. We present the case of a 65-year-old man with symptomatic severe aortic stenosis and concomitant IgG4 aortitis. The diagnosis was confirmed by IgG4 serum levels, positive positron emission computed tomography (PET-CT) scans, and pathology from mediastinal dissection. Surgical aortic valve replacement (SAVR) was unfeasible owing to extensive mediastinal fibrosis, and transcatheter aortic valve replacement (TAVR) was successfully performed. As ascending aorta access for SAVR in IgG4 aortitis with long-run fibrosis entails a high risk of mortality, TAVR could be considered in certain suitable patients.