804 C. Meierhofer et al. 31. Hope MD, Hope TA, Crook SES, Ordovas KG, Urbania TH, Alley MT et al. 4D flow CMR in assessment of valve-related ascending aortic disease. JACC Cardiovasc Imaging 2011;4:781 –7. 32. Barker AJ, Markl M, Burk J, Lorenz R, Bock J, Bauer S et al. Bicuspid aortic valve is associated with altered wall shear stress in the ascending aorta. Circ Cardiovasc Imaging 2012;5:457 –66. 33. Schaefer BM, Lewin MB, Stout KK, Byers PH, Otto CM. Usefulness of bicuspid aortic valve phenotype to predict elastic properties of the ascending aorta. Am J Cardiol 2007;99:686 – 90. 34. Fernandes SM, Sanders SP, Khairy P, Jenkins KJ, Gauvreau K, Lang P et al. Morphology of bicuspid aortic valve in children and adolescents. J Am Coll Cardiol 2004; 44:1648 –51. 35. McKusick VA, Logue RB, Bahnson HT. Association of aortic valvular disease and cystic medial necrosis of the ascending aorta: report of four instances. Circulation 1957;16:188 –94. 36. McKusick VA. Association of congenital bicuspid aortic valve and Erdheim’s cystic medial necrosis. Lancet 1972;1:1026 –7. 37. Stefanadis C, Vlachopoulos C, Karayannacos P, Boudoulas H, Stratos C, Filippides T et al. Effect of vasa vasorum flow on structure and function of the aorta in experimental animals. Circulation 1995;91:2669 –78. 38. Stefanadis CI, Karayannacos PE, Boudoulas HK, Stratos CG, Vlachopoulos CV, Dontas IA et al. Medial necrosis and acute alterations in aortic distensibility following removal of the vasa vasorum of canine ascending aorta. Cardiovasc Res 1993;27:951 –6. IMAGE FOCUS doi:10.1093/ehjci/jet035 Online publish-ahead-of-print 14 March 2013 ............................................................................................................................................................................. Rheumatic disease mimicking an infiltrative mass of the mitral valve Ferande Peters1, Bijoy K. Khandheria2*, Michelle L. Wong3, and Mohammed R. Essop1 * Corresponding author. Tel: +1 414 649 3909; fax: +1 414 649 3551. Email: [email protected] A 32-year-old man previously was diagnosed with sarcoidosis on the basis of bilateral lung infiltrates, hypercalcaemia, multiple renal calculi and an elevated serum angiotensin-converting enzyme level, all of which improved on corticosteroids. Nine months later he developed symptomatic, sputum-positive pulmonary tuberculosis and tuberculous lymphadenitis, which were successfully treated. At his initial presentation, there was evidence of severe mitral regurgitation and moderate mitral stenosis complicated by severe pulmonary hypertension (Panels A and B).The medial halves of both mitral leaflets were immobilized by what appeared to be an infiltrative process with an associated pedunculated mass (Panels C – F, see Supplementary data online, Videos S1 and S2). The subvalvular apparatus and basal posterior wall were abnormal and presumed to represent a continuum of the infiltrative process. No clinical or laboratory features of infective endocarditis were present. Two years later, the morphology of the valve was unchanged except for the absence of the pedunculated mass, while the degree of pulmonary hypertension and mitral stenosis was worse. Cardiac catheterization confirmed the severe pulmonary hypertension was due almost exclusively to the severe mixed mitral valve disease. This haemodynamic abnormality was eliminated following successful mitral valve replacement. Histology of the resected valve revealed features of chronic inflammation compatible with chronic rheumatic disease with no features suggestive of tuberculosis or sarcoid (Panel G). We postulate that the pedunculated mass might have represented nonbacterial endocarditis and that excessive scarring and chronic inflammation from untreated rheumatic disease resulted in this unusual morphological appearance of the mitral valve. Supplementary data are available at European Heart Journal – Cardiovascular Imaging online. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2013. For permissions please email: [email protected] Downloaded from by guest on December 4, 2014 1 Department of Cardiology, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Diepkloof 319-Iq, Johannesburg, Soweto 1862, South Africa; 2Aurora Cardiovascular Services, Aurora Sinai/Aurora St Luke’s Medical Centers, University of Wisconsin School of Medicine and Public Health, 2801 W. Kinnickinnic River Parkway, #840, Milwaukee, WI 53215, USA; and 3Department of Pulmonology, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Diepkloof 319-Iq, Johannesburg, Soweto 1862, South Africa
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