Supravalvular Stenotic Mitral Ring with Ventricular Septal Defect

Watraida, Shoji; Shiraishi, Shuichi; Katsuyama, Kazuhiko; Nakajima, Yasuhiko; Onoe, Masahiko; Sugita, Tadaaki; Yamamoto, Rie; Imura, Masataka

doi:10.1111/j.1540-8191.1997.tb00088.x

Cited by 5 publications

(4 citation statements)

References 4 publications

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“…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. [15][16][17]4,9 These various forms are important to differentiate from the anatomical variation we describe here in our patients, that is, the discrete form of supravalvular membranous stenosis adherent to the mitral valve. This entity is very rare in childhood, and only a few cases have been described in the literature.…”

Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 99%

“…This malformation may occur approximately in 17–31% of congenital anomalies of the mitral valve, and is reported to be 71% in patients with a parachute mitral valve 9 10 Hoashi et al found a supravalvular ring in four out of 20 patients with congenital mitral valve stenosis, whereas Vaideeswar et al even reported 37.5% – 24 out of 64 congenital left ventricular inflow tract obstructions from 1633 autopsied examples 11 …”

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confidence: 99%

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

et al. 2014

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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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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

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

et al. 2014

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“…Other common associated lesions in patients with supravalvular mitral ring are ventricular septal defect and tetralogy of Fallot [9]. …”

Section: Discussionmentioning

confidence: 99%

Mitral supravalvular ring: a case report

Serra¹,

Testa²,

Ardissino³

2005

Cardiovasc Ultrasound

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Supravalvular mitral stenosis is a rare condition characterized by an abnormal ridge, with one or two orifices, covering and obstructing the mitral valve. Preoperative diagnosis is difficult with transtoracic echo (TTE), angiography and magnetic resonance imaging (MRI).In this case, a 36-year-old male, was admitted to our Heart department: He experienced progressive dyspnea on effort and at rest.Diagnosis was made by transesophageal echocardiography which showed, on apical 4-chamber section, an anulare structure attached since a membrane to the atrial wall anterior mitral valve leaflet and just proximal to the posterior mitral leaflet.Pre-operative identification of the supravalvular mitral ring is the target for obtaining good surgical results. Cineangiography and MRI both failed in reaching this objective, whereas, transesophageal echocardiography is the best method to identify this congenital heart disease.Using TEE the identification is not only possible but also easier.

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2003

The Natural and Modifed History of Congenital Heart Disease

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Supravalvular Stenotic Mitral Ring with Ventricular Septal Defect

Abstract: In our case, no mitral stenosis was evident on postoperative echocardiogram performed 3 years after surgery.

Cited by 5 publications

References 4 publications

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

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

Mitral supravalvular ring: a case report

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