IgG4-related disease of the aortic valve: a report of two cases and review of the literature

IgG4-related disease (IgG4-RD) is a relatively recently described disorder that can affect multiple organ systems, including the cardiovascular system. While most reported cases of cardiovascular involvement are of the aorta, usually the abdominal aorta, rare isolated reports of involvement of the heart proper have been described. Herein, we describe two cases of IgG4-RD involving the aortic valve. Each case was found to be associated with increased levels of IgG4 in the tissue and one case resulted in the subsequent discovery of IgG4-related pancreatitis.