Journal of Genetic Syndromes & Gene Therapy
Open Access

Abstract

Igg4-Related Aortitis of the Ascending Aorta in a Patient Undergoing Emergent Coronary Artery Bypass Graft: A Challenging Disease

Benassi Filippo, Molardi Alberto, Nicolini Francesco, Cocconcelli Flavio, Corradi Domenico, Cobelli Rocco, Bozzetti Francesca and Gherli Tiziano

Immunoglobulin G4 (IgG4)-related sclerosing disease in the cardiovascular system is a recently proposed disease entity, which is still unfamiliar to most clinicians. We present a case of IgG4-related periaortitis of the ascending aorta undergoing emergent surgical revascularization. During the operation we found an extremely thick and stiff aorta that led us to an incomplete surgical revascularization due to the impossibility of cross-clamp the vessel. We performed several biopsies of the aorta that confirmed the diagnosis of IgG-4 related aortitis. Before discharging the patients we perform a CT scan in order to better evaluate the real extension of the aortitis. The patient was discharged in good clinical condition and the revascularization was completed three month later performing a percutaneous transluminal angioplasty on right and circumflex coronary artery. IgG4-related periaortitis needs to be considered in the differential diagnosis in a patient with thickened aortic wall.