Unusual Heart Involvement of Wegener's Granulomatosis and Literature Review

Esther Cambronero-Cortinas, Miguel Jose Corbi-Pascual, Gonzalo Gallego-Sanchez, Nuria Baxeiras-Gonzalez, Tomas Cros Ruiz de la Galarreta, Raquel Fuentes-Manso, Antonia Tercero-Martinez, Daniel Prieto-Mateos, Gonzalo Aldamiz-Castillo Echevarria, Jose Luis Rodriguez Garcia


A female patient, 60 years of age, was presented to our hospital with chest pain and monomorphic ventricular tachycardia (VT). She was transferred to the Coronary Care Unit and amiodarone perfusion restored basal rhythm in atrial fibrillation. She has not sign of heart failure. A transtoracic echocardiogram (TTE) was performed and an one mitral mass was found at atrioventricular junction with displacement of the posterior mitral leaflet A transesophageal echocardiogram (TEE) demonstrated a mass at atrioventricular junction level with severe mitral regurgitation. Cardiac Magnetic Resonance (CMR) confirmed the mass and anterolateral papillary muscle was thickening and hypertrophied with hyperenhancement consistent with fibrosis.  Moreover, T2-weighted imaging demonstrated hyperintense mass with respect to the surrounding myocardium in relation of inflammatory mass. She had saddle nose by destruction of the septum, bilateral hearing loss, sinusitis and scleritis and renal involvement as well. This patient was diagnosed of Wegener's Granulomatosis (WG) and she was treated with methylprednisolone during 3 days, continued with prednisolone and cyclophosphamide. An 8 days later echocardiogram did not find the mass. However, the patient developed symptomatology of heart failure and in the context of severe mitral regurgitation, mitral valve replacement was decided in multi-disciplinary Cardiology-Cardiothoracic meeting.


- Fauci AS, Wolff SM. Goodfield NE, et al. Cardiac involvement in Wegener's granulomatosis. Br Heart Journal. 1995;73:110.

- Shakil O, Matyal R, Khabbaz K, et al. Intracardiac Wegener’s Granulomatosis. The Ann Thorac Surgery. 2012;94:105. doi: 10.1016/j.athoracsur.2012.05.095

- Florian A, Slavich M, Blockmans D, et al. Cardiac Involvement in Granulomatosis With Polyangiitis (Wegener Granulomatosis). Circulation. 2011;24:3342-344.

- Sunita J, Ferns, Nguyenvu V. et al. Left ventricular mass in Wegener´s Granulomatosis: a brief report. Cardiology in the young. 2010;20:710-713. doi: 10.1161/CIRCULATIONAHA.111.030809

- Ruisi M, Ruisi P, Finkielstein D. Cardiac manifestations of Wegener’s granulomatosis: Case report and review of the literature. Journal of Cardiology Cases. 2010;2:99—102. doi:10.1016/j.jccase.2010.04.002

- Singh R, Rosen S. Tumor of the heart in a young woman; a rare manifestation of Wegener granulomatosis. Human Pathology. 2012;43: 289–292. DOI: http://dx.doi.org/10.1016/j.humpath.2011.04.020

- Fauci AS, Haynes BF, Katz P, et al. Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98:76—85.

- Jon Torgny R. Wilcke, Palle K. Nielsen, Tage N. Jacobsen. Reversible complete heart block due to Wegener’s granulomatosis. International Journal of Cardiology. 2003;89:297–298. DOI: http://dx.doi.org/10.1016/S0167-5273(02)00464-3

- Brihaye B, Aouba A, Pagnoux C, et al. Rituximab reversed cardiac involvement of Wegener's granulomatosis: magnetic resonance imaging assessment. La presse Médicale. 2008;37(3 Pt 1):412-415. doi:10.1016/j.lpm.2007.08.019

- Shewan LG, Coats AJS, Henein M. Requirements for ethical publishing in biomedical journals. International Cardiovascular Forum Journal. 2015;2:2.

DOI: 10.17987/icfj.v2i1.4

DOI: https://doi.org/10.17987/icfj.v8i0.299

Copyright (c) 2016 Esther Cambronero-Cortinas

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