Supravalvular Congenital Mitral Stenosis

Moraes, Fernando; Lapa, Cleuza; Ventura, Cristina; Santana, Rejane Conceição; Tenório, Euclides; Gomes, Cláudio A; Moraes, Carlos R.

doi:10.1590/s0066-782x2002001000008

Cited by 9 publications

(9 citation statements)

References 9 publications

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“…However, there are still many limitations in visualization of the membrane, that is usually very thin; in this case, we performed an MRI examination, but as described in other papers [14,15], there was a failure in the detection of the supravalvular mitral ring.…”

Section: Discussionmentioning

confidence: 92%

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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Section: Discussionmentioning

confidence: 92%

Mitral supravalvular ring: a case report

Serra¹,

Testa²,

Ardissino³

2005

Cardiovasc Ultrasound

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“…The incidence is reported to range from one-eighth to two-fifths among congenital mitral valvar anomalies that were not necessarily stenotic. 2,[7][8][9][10] All were children. In our analysis, the ring had an incidence of 1.5% among all congenital cardiac defects at autopsy, and accounted for just over one-third of all congenital lesions producing obstruction of the left ventricular inflow tract.…”

Section: Discussionmentioning

confidence: 99%

“…Most of the previous series do not particularly comment on the completeness or nature of the ring. 2,[7][8][9][10] In a recent report of 15 children, 11 all had been circumferential, though incomplete rings have been described. 5 Circumferential ridges were seen in only 7 of our 24 hearts (29.1%), and these were entirely located on the valvar leaflets adjacent to the annulus.…”

Section: Discussionmentioning

confidence: 99%

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An analysis of 24 autopsied cases with supramitral rings

Vaideeswar

Baldi

Warghade

2008

CTY

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The supramitral ring is a rare congenital malformation formed by presence of a ridge of connective tissue, usually attached at or above the mitral annulus. The incidence and clinical presentation is highly variable due to difficulty in diagnosis. A review of autopsied congenital heart diseases at our institute over a 17-year-period revealed 24 cases of supramitral ring. These were classified with respect to the morphology of the ridge and the presence of associated cardiac lesions. The ring was found in 1.5% of the autopsied specimens of congenitally malformed hearts, and in 37.5% of those with obstructed left-sided inflow tracts. The majority of the specimens came from children (79.2%). A clinical diagnosis had been made in only two. In one-third of the cases, the ring was associated with incomplete Shone's complex. Varied anomalies were seen in others, chiefly ventricular septal defects. An interesting association was the presence of rheumatic mitral valvar disease, found in 3 cases. There was no difference in the completeness or width of the supramitral ridge in the hearts from those with or without Shone's complex. Circumferential rings were fleshy and stenosing, while incomplete rings had variable locations and stenosis. The presence of a supramitral ring may be underestimated due to association with other cardiac anomalies, both congenital and acquired. Since the ridge need not always produce stenosis, the correct designation would be simply a supramitral ring.

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“…Mitral ring is a subtype of congenital mitral valve stenosis (CMS) and is part of a spectrum of obstructive lesions affecting the left heart [1] . Rarely isolated [2] , mitral ring is usually associated with various congenital heart defects [3] , such as interventricular communication, coarctation of the aorta, subaortic stenosis, parachute mitral valve, left superior vena cava draining in the tectum of the left atrium, and cor triatriatum [4] . The variability of the pathology and the defects interfere with the best strategy choice.…”

Section: Introductionmentioning

confidence: 99%

Congenital Supravalvar Mitral Ring – A Case Report

Soares¹,

Ferber²,

Ferber³

et al. 2020

Braz J Cardiovasc Surg

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Congenital mitral valve stenosis is a rare and severe disease, usually associated with other heart defects. The appropriate intervention depends on the site and mechanism of valvular obstruction and the aim is to avoid or delay valve replacement since it is associated with significant morbidity and mortality. Early single-stage complete repair is associated with better prognosis.We report the case of a 20-month-old child with a supravalvar mitral ring combined with a ventricular septal defect; pulmonary arterial systolic pressure before the surgery was 79 mmHg. The patient underwent a successful surgical repair with good clinical resolution.

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Supravalvular Congenital Mitral Stenosis

Cited by 9 publications

References 9 publications

Mitral supravalvular ring: a case report

Mitral supravalvular ring: a case report

An analysis of 24 autopsied cases with supramitral rings

Congenital Supravalvar Mitral Ring – A Case Report

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