Background: Takayasu���s arteritis (TA) is primary vasculitis. Cardiac involvements in TA are due to the consequences of the vascular lesions as well as the primary pathology of the heart. The disease activity of TA is known to influence the prognosis of TA. We evaluated the cardiovascular manifestations of TA, and assessed their relation to the disease activity of TA. Methods: From September, 1994 to March, 2009, 204 patients were diagnosed with TA according to the diagnostic criteria of the 1990 American College of Rheumatology. Their clinical features and the laboratory, angiographic and echocardiographic findings were retrospectively reviewed. Active TA was defined as satisfying one of the following criteria: i) an elevated ESR or CRP level, ii) vessel wall thickening or enhancement on MR or CT angiography, and iii) carotodynia. Results: According to our definition, 139(69.2%) patients were classified as active group. The cardiovascular signs and symptoms were not generally different between the active and inactive groups. The active TA patients had more frequent involvement of the ascending aorta, aortic arch and its main branches than did the inactive group. The active group showed a higher incidence of pulmonary hypertension and significant aortic valve regurgitation. A higher level of NT-proBNP was observed in the active group. Conclusions: These findings suggest that disease activity plays an important role for the cardiovascular manifestations of TA. The TA patients with higher activity have more cardiovascular morbidity as compared to the TA patients with low disease activity.