Mitral and aortic valvular insufficiency required staged valve replacement in a patient with chronic relapsing polychondritis. The homograft valves functioned normally but the patient died of an unrecognized mediastinitis and overwhelming sepsis while on immunosuppression for his underlying disease 16 days following aortic valve replacement and six months after mitral replacement. Pathologically the valvular insufficiency was primarily due to annular dilatation. The media of the aorta, pulmonary artery, and other major arteries demonstrated marked disruption of the elastic fibers. At postmortem examination the homograft valves appeared normal and were firmly united to the supportive stents, even while on immunosuppression.
Pappas G, Johnson M. Mitral and Aortic Valvular Insufficiency in Chronic Relapsing Polychondritis. Arch Surg. 1972;104(5):712-714. doi:10.1001/archsurg.1972.04180050086022