Background In ankylosing spondylitis (AS) the occurrence of involvement of ascending aorta and aortic (and sometimes mitral) valve is known. The microscopic changes include inflammatory destruction of aortic and valvular tissue and replacement by granulation and fibrous tissue. Aortic regurgitation results from thickening and displacement of valve cusps and dilatation of aortic root. It may be clinically silent or may cause severe problems to the patient. Immunosuppressive therapy may prevent or delay the need for aortic valve replacement but many times valve replacement is the only life-saving therapy.

Results Recently we had an opportunity to treat 3 patients with AS and aortic incompetence. All three were suffering from AS with peripheral arthritis. In one patient aortic failure was complicated with bacterial endocarditis although previous symptoms of aortic failure were only subclinical. In the second patient aortic insufficiency proceeded very rapidly to severe and life-threatening cardiac failure. In both of them aortic valve replacement was successful. Interestingly, in the second patient massive immunosuppressive therapy prior to cardiosurgery led to a significant improvement of cardiac failure. The third patient has not been suffering from any cardiac troubles but the clinical and x-ray examination (aortography) revealed a significant aortic regurgitation.

Conclusion It is a matter of interest that the second patient had some features of Marfan’s syndrome and the third had some features of Ehlers-Danlos syndrome. It raised the question which of these two components ? inflammation or primary defect of collagen or both of them participates in the aortic involvement.