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The “Polar Light Sign” is a useful tool to detect discrete membranous supravalvular mitral stenosis

  • Christine Hertwig (a1), Nikolaus A. Haas (a1), Sheeraz Habash (a1), Andreas Hanslik (a1), Deniz Kececioglu (a1), Eugen Sandica (a1) and Kai-Thorsten Laser (a1)...

Abstract

Mitral valve stenosis caused by a discrete supravalvular membrane is a rare congenital malformation haemodynamically leading to significant mitral valve stenosis. When the supravalvular mitral stenosis consists of a discrete supravalvular membrane adherent to the mitral valve, it is usually not clearly detectable by routine echocardiography. We report about the typical echocardiographic finding in three young patients with this rare form of a discrete membranous supravalvular stenosis caused by a membrane adherent to the mitral valve. These cases present a typical echocardiographic feature in colour Doppler generated by the pathognomonic supramitral flow acceleration. Whereas typical supravalvular mitral stenosis caused by cor triatriatum or a clearly visible supravalvular ring is easily detectable by echocardiography, a discrete supravalvular membrane adjacent to the mitral valve leaflets resembling valvular mitral stenosis is difficult to differentiate by routine echocardiography. In our opinion, this colour phenomenon does resemble the visual impression of polar lights in the northern hemisphere; owing to its typical appearance, it may therefore be named as “Polar Light Sign”. This phenomenon may help to detect this anatomical entity by echocardiography in time and therefore improve the prognosis for repair.

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Corresponding author

Correspondence to: Dr C. Hertwig, MD, Department for Pediatric Cardiology, Center for Congenital Heart Defects, Herz- und Diabeteszentrum NRW, Georgstrasse 11, 37545 Bad Oeynhausen, Germany. Tel: +49 5731 97 3643; Fax: +49 5731 97 2131; E-mail: chertwig@hdz-nrw.de

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