The "Polar Light Sign" is a useful tool to detect discrete membranous supravalvular mitral stenosis.
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.
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Christine
C
Hertwig
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Nikolaus A
NA
Haas
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Sheeraz
S
Habash
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Andreas
A
Hanslik
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Deniz
D
Kececioglu
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Eugen
E
Sandica
Department for Pediatric Cardiology,Center for Congenital Heart Defects,Heart and Diabetes Center NRW,Ruhr University of Bochum,Bad Oeynhausen,Germany.
Kai-Thorsten
KT
Laser
D002675:Child, Preschool; D018618:Echocardiography, Doppler, Color; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008946:Mitral Valve Stenosis / Q000000981:diagnostic imaging*
colour Doppler