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European Journal of Echocardiography Advance Access published online on July 11, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen200
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Shone's syndrome diagnosed with echocardiography and confirmed at pathology

Bogdan A. Popescu1,2,*, Ruxandra Jurcut1,2, Marinela Serban1, Liliana Parascan1 and Carmen Ginghina1,2

1 ‘Prof. Dr. C. C. Iliescu’ Institute of Cardiovascular Diseases, Bucharest, Romania
2 ‘Carol Davila’ University of Medicine and Pharmacy, Bucharest, Romania

Received 19 April 2008; accepted after revision 22 June 2008.

* Corresponding author. Tel: +40 21 3175227; Fax: +40 21 3175227.E-mail address: bogdan.a.popescu{at}gmail.com


  Abstract

The Shone's complex, defined by four cardiovascular defects such as a supravalvular mitral membrane, valvular mitral stenosis by a parachute mitral valve, subaortic stenosis, and aortic coarctation, is a rare entity, which occurs most frequently in its incomplete form. We report the case of a 19-year-old female patient who presented at the emergency room for progressively worsening dyspnoea, orthopnoea, fever, and productive cough, due to bronchopneumonia. Echocardiography revealed the co-existence of aortic coarctation with bicuspid aortic valves, mitral supravalvular ring, and dysplastic mitral valves producing severe mitral stenosis and severe pulmonary hypertension. Although wide spectrum antibiotics were administered from the first day of hospitalization, the patient developed severe sepsis and died. The components of the Shone's complex diagnosed by echocardiography were confirmed at pathology.

Keywords: Shone's complex; Supramitral ring; Mitral stenosis; Aortic coarctation; Pulmonary hypertension


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