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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: [email protected]
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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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References

1. Deshpande, J, Vaideeswar, P, Amonkar, G, Vasandani, S. Rheumatic heart disease in the past decade: an autopsy analysis. Indian Heart J 2002; 54: 676–680.Google ScholarPubMed
2. Carpentier, A, Branchini, B, Cour, J, et al. Congenital malformations in the mitral valve in children. J Thorac Cardiovasc Surg 1976; 72: 854–866.CrossRefGoogle ScholarPubMed
3. Fischer, T. Two cases of congenital disease of the left side of the heart. Br Med J 1902; 1: 639–641.CrossRefGoogle Scholar
4. Mychaskiw, G II, Sachdev, V, Braden, D, Heath, B. Supramitral ring: an unusual cause of congenital mitral stenosis. J Cardiovasc Surg 2002; 43: 199–202.Google ScholarPubMed
5. Glaser, J, Yakirevich, V, Vidne, B. Am Heart J 1984; 108: 169–171.CrossRefGoogle Scholar
6. Lynch, M, Ryan, N, Williams, J, et al. Preoperative diagnosis and surgical correction of supravalvular mitral stenosis and ventricular septum defect. Circulation 1962; 25: 854–861.CrossRefGoogle Scholar
7. Vaideswaar, P, Baldi, M, Warghade, S. An analysis of 24 autopsied cases with supramitral rings. Cardiol Young 2009; 19: 70–75.CrossRefGoogle Scholar
8. Sullivan, I, Robinson, P, de Leval, M, Graham, T Jr. Membranous supravalvular mitral stenosis: a treatable form of congenital heart disease. J Am Coll Cardiol 1986; 8: 159–164.CrossRefGoogle ScholarPubMed
9. Watraida, S, Shiraishi, S, Katsuyama, K, et al. Supravalvular stenotic mitral ring with ventricular septum defect. J Card Surg 1997; 12: 46–48.CrossRefGoogle Scholar
10. Banerjee, A, Kohl, T, Silverman, N. Echocardiographic evaluation of congenital mitral valve anomalies in children. Am J Cardiol 1995; 76: 1284–1291.CrossRefGoogle ScholarPubMed
11. Hoashi, T, Bove, E, Devaney, E, Hirsch, J, Ohye, R. Mitral valve repair for congenital mitral valve stenosis in the pediatric population. Ann Thorac Surg 2010; 90: 36–41.CrossRefGoogle ScholarPubMed
12. Shone, J, Sellers, R, Anderson, R, Adams, P Jr, Lillehei, C, Edwards, J. The developmental complex of “Parachute Mitral Valve”, supravalvular ring of left atrium, subaortic stenosis and coarctation of aorta. Am J Cardiol 1963; 11: 714–725.CrossRefGoogle ScholarPubMed
13. Brickner, M, Hillis, L, Lange, R. Congenital heart disease in adults. N Engl Med 2000; 342: 256–263.CrossRefGoogle ScholarPubMed
14. Pettersen, M, Berger, S. Mitral Stenosis, Supravalvular Ring. Medscape Reference, updated Jan 14, 2010. Available at http://emedicine.medscape.com/article/897081 Google Scholar
15. Alvares, S, Sa Melo, A, Antunes, M. Divided left atrium associated with supravalvular ring. Cardiol Young 1999; 9: 423–426.CrossRefGoogle Scholar
16. Collison, S, Kaushal, S, Dagar, K, et al. Supramitral ring: good prognosis in a subset of patients with congenital mitral stenosis. Ann Thorac Surg, 81: 997–1001.CrossRefGoogle Scholar
17. La Corte, M, Harada, K, Williams, R. Echocardiographic features of congenital left ventricular inflow obstruction. Circulation 1976; 54: 562–566.CrossRefGoogle ScholarPubMed
18. Ma, X, Huang, G, Liang, X, Liu, X, Jia, B. Atypical Shone’s Complex diagnosed by echocardiography. Ped Cardiol 2011; 32: 442–448.CrossRefGoogle ScholarPubMed
19. Lai, W, Mertens, L, Cohen, M, Geva, T. Echocardiography in Pediatric and Congenital Heart Disease. Blackwell Publishing Ltd., 2009: 221–223.CrossRefGoogle ScholarPubMed
20. Eidem, B, Cetta, F, O’Leary, P. Echocardiography in Pediatric and Adult Congenital Heart Disease. Lippincott Williams & Wilkins; 2010: 134–136.Google Scholar