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

Published online by Cambridge University Press:  28 January 2014

Christine Hertwig ,
Nikolaus A. Haas ,
Sheeraz Habash ,
Andreas Hanslik ,
Deniz Kececioglu ,
Eugen Sandica  and
Kai-Thorsten Laser
Christine Hertwig*
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
Nikolaus A. Haas
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
Sheeraz Habash
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
Andreas Hanslik
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
Deniz Kececioglu
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
Eugen Sandica
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
Kai-Thorsten Laser
Affiliation:
Department for Pediatric Cardiology, Center for Congenital Heart Defects, Heart and Diabetes Center NRW, Ruhr University of Bochum, Bad Oeynhausen, Germany
*
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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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.

Keywords

Supravalvular mitral stenosisechocardiographic findingscolour Doppler
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 2 , February 2015 , pp. 328 - 332
Copyright
© Cambridge University Press 2014 

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