Cardiovascular manifestations of Takayasu arteritis and their relationship to the disease activity: Analysis of 204 Korean patients at a single center Ga Yeon Lee a , Shin Yi Jang b , Sung Min Ko a , Eun Kyoung Kim a , Sung Ho Lee a , Hyejin Han a,b , Seung Hyuk Choi a , Young-Wook Kim c , Yeon Hyeon Choe b,d , Duk-Kyung Kim a,b, ⁎ a Division of Cardiology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea b Cardiovascular Imaging Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea c Division of Vascular Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea d Department of Radiology and Center for Imaging Science, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea abstract article info Article history: Received 16 August 2010 Received in revised form 24 December 2010 Accepted 29 January 2011 Available online 26 February 2011 Keywords: Takayasu arteritis Takayasu disease Disease activity Cardiovascular manifestations Takayasu's arteritis (TA) is primary vasculitis. Cardiac involvements in TA is 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 hypothesized that the cardiovascular involvement of TA is related to the disease activity. We evaluated the cardiovascular manifestations of TA, and we assessed their relation to the disease activity of TA. Two hundred four patients were diagnosed with TA from September, 1994 to March, 2009 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. The group with active disease activity was defined as satisfying one of the following criteria: i) an elevated ESR or CRP level, ii) thickened arterial wall with mural enhancement on CT or MR angiography, and iii) carotidynia at the time of the initial diagnosis. One hundred thirty nine patients (69.2%) were classified as the 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 and the aortic arch and its main branches than did the inactive group. The active group showed a higher incidence of significant aortic valve regurgitation and pulmonary hypertension, and a higher level of NT-proBNP. 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 compared to the TA patients with low disease activity. © 2011 Elsevier Ireland Ltd. All rights reserved. 1. Introduction Takayasu arteritis (TA) is systemic inflammatory disease involving the aorta and its main branches. The initial prominent finding of TA is granulomatous inflammation in the adventitia and medial wall of the involved vessels [1]. It progresses to fibrosis and causes stenosis or occlusion in the lesions. In the advanced stage, destruction of the elastic fibers in the medial wall can cause dilatation or aneurismal changes [2]. The progression of vascular lesions is known to be related to the disease activity of TA. In patients with active progressive disease, the established vascular abnormalities further advance to stenosis or dilatation. The outcome of intervention or bypass surgery for the involved vessels is influenced by the TA activity [3,4]. Patients with TA, as compared with the patients with atherosclerosis, have been reported to have decreased rates of sustained vessel patency after the procedures [5]. The cardiac manifestations in TA are the consequences of the various features related to TA; hypertension, pathologic involvements of the pulmonary/coronary artery, aortic regurgitation as well as direct involvement of the myocardium [6,7]. Cardiac manifestations may represent another type of cardiovascular morbidity in TA patients. One study investigating the prognostic factors associated with TA found two major predictors of outcome: the presence of a progressive disease course, which reflects the disease activity, and the incidence of complications (Takayasu's retinopathy, hypertension, aortic regurgitation and aneurysm) [8], However, the cardiovascular manifestations and their relationship with disease activity have not been evaluated in a systemic manner. Since our earlier publication about the characterization of 45 TA patients [9], we have continued careful documentation of each subsequent case. In this current series that includes 204 TA patients, which is one of the largest in the world, we intended to characterize the cardiovascular manifestations in Korean patients and evaluate whether the disease activity of TA International Journal of Cardiology 159 (2012) 14–20 ⁎ Corresponding author at: Division of Cardiology, Department of Medicine, Cardiac and Vascular Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, #50 Irwon-dong, Gangnam-gu, Seoul, 135-710, Republic of Korea. Tel: +82 2 3410 3419; fax: +82 2 3410 3849. E-mail address: dkkim@skku.edu (D.-K. Kim). 0167-5273/$ – see front matter © 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2011.01.094 Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard