Aortic valve incompetence and replacement in rheumatoid arthritis.

Five cases of aortic incompetence and nodular seropositive rheumatoid arthritis are presented. Four cases underwent aortic valve replacement. Two of these had granulomatous involvement of the aortic cusps similar to subcutaneous rheumatoid nodules, and another showed a nonspecific fibrosis. One case had definite coincidental rheumatic aortic and mitral heart disease. Two patients had undergone pericardectomy previously for constrictive pericarditis. Good results were obtained in all four operated cases and cardiac surgery enabled continuation of rehabilitation for the rheumatoid arthritis, including major orthopaedic procedures. A review of 22 cases from the literature with rheumatoid granulomata within the aortic valve shows that they are associated with mitral valve granulomata in 63-6%. Congestive cardiac failure was found in 75%. Macroscopical evidence of aortic incompetence was seen in 36-8% and of aortic stenosis in 15-8%. Associated pericarditis occurred in 59-1%, which was severe or complicated in 13.6%. The associated arthritis was severe in 77-8% with subcutaneous nodules (71-5%), rheumatoid factor (83-6%), and episcleritis (66-6%). From these cases and a review of the literature the following points are emphasized. (1) Both the granulomatous and nonspecific aortic valvulitis of rheumatoid arthritis may result in significant haemodynamic abnormality. (2) The valve lesions found are often clinically and macroscopically indistinguishable from rheumatic valve lesions. (3) Granulomata, when present, are usually found in the valve cusp or ring and only occasionally in the aortic wall. (4) Associated joint disease, although usually severe, may be mild. (5) The valve lesion may be accompanied by a severe pericardial involvement--either tamponade or constriction. (6) Aortic valve replacement for aortic incompetence in rheumatoid arthritis is both feasible and worthwile, despite severe joint disease.