Ankylosing Spondylitis and Aortic Regurgitation

Clinical and cardiovascular necropsy findings are described in eight patients with combined ankylosing spondylitis and aortic regurgitation. All were men (aged 34-55 years), each had peripheral arthritis in addition to spondylitis, all had severe congestive failure, and six had conduction disturbances. In three patients aortic regurgitation was present before distinctive radiologic changes of ankylosing spondylitis were apparent and only two patients had advanced arthritic changes of ankylosing spondylitis. Thus, cardiac dysfunction may be present before signs of spondylitis are apparent, and aortic regurgitation may be severe when signs of spondylitis are minimal. A characteristic cardiovascular morphologic abnormality was present in each patient. The aortic valve cusps and the aorta behind and immediately above the sinuses of Valsalva were thickened, the latter by dense adventitial scar tissue and by intimal fibrous proliferation. In each patient the scar tissue in the root of aorta extended below the base of aortic valve to produce a subaortic fibrous ridge. The subaortic bump involves the base of anterior mitral leaflet and may cause mitral regurgitation. Extension of the fibrous scar into ventricular septum may cause heart block. The distinctive cardiovascular morphologic findings in patients with ankylosing spondylitis clearly separate this condition from syphilis and other entities associated with aortic regurgitation.