Surgical treatment of the cardiac manifestations of relapsing polychondritis: overview of 33 patients identified through literature review and the Mayo Clinic records.

OBJECTIVES: To analyze the cardiac findings that necessitate surgery in patients with relapsing polychondritis (RP) and to compare our results to cases in the literature.
MATERIAL AND METHODS: A systematic overview of the literature was completed with the addition of cases of RP from the Mayo patient population that necessitated cardiac surgery.
RESULTS: Thirty-three patients were identified (25 from the literature and 8 from the Mayo patient population). Nine patients (27%) were female, 22 (67%) were male, and sex was not stated for 2 patients (6%). The patient age ranged from 17 to 69 years (mean +/- SD, 42.5 +/- 15.7 years). At operation, 30 patients (91%) had aortic regurgitation, 21 (64%) had aortic root disease, and 13 (39%) had mitral regurgitation. The most common surgical procedure performed was aortic valve replacement in 12 patients (36%). The most common complications were death in 12 patients (36%) and prosthetic valve dehiscence in 4 patients (12%).
CONCLUSIONS: Cardiac involvement is more prominent in the male population and requires more invasive procedures. Aortic valve replacement with composite graft replacement of the ascending aorta along with coronary artery ostial reimplantation should be considered in these patients. Postsurgical valvular complications include prosthetic dehiscence, paravalvular leakage, mediastinitis, and heart failure, and these complications are associated with postoperative corticosteroid therapy. Initiation of second-line immunosuppressive therapy should be substituted for corticosteroids after cardiac surgery.