A condition characterized by a combination of OSTIUM SECUNDUM ATRIAL SEPTAL DEFECT and an acquired MITRAL VALVE STENOSIS.

Value and limitations of Doppler echocardiographic determination of mitral valve area in Lutembacher syndrome. (1/4)

OBJECTIVES: Our objective was to compare the Doppler pressure half-time, Doppler continuity equation and two-dimensional echocardiographic planimetric methods of estimating mitral valve area in Lutembacher syndrome. BACKGROUND: Fluid dynamics theory predicts that mitral pressure half-time varies inversely with mitral valve area and directly with net chamber compliance and the peak early diastolic transmitral gradient in pure mitral stenosis. The effects of an atrial shunt on these interrelations have not been investigated. METHODS: Correlation and agreement between mitral valve area estimates obtained by the three methods and that obtained by cardiac catheterization was ascertained in 11 patients with Lutembacher syndrome. RESULTS: Valve areas determined by planimetry and the continuity equation method correlated and agreed well with catheterization measurements (r = 0.83 and 0.81, respectively). The pressure half-time method consistently overestimated mitral valve area; the extent of overestimation was greater in patients with larger atrial shunts. The hemodynamic pressure half-time was independent of the mitral valve area, chamber compliance and the peak transmitral gradient. It was dependent on the magnitude of the atrial shunt, although the correlation obtained was only fair (r = 0.61). CONCLUSIONS: These findings suggest that the Doppler pressure half-time method is an inaccurate measure of mitral valve area whenever an atrial shunt coexists with mitral stenosis. Planimetry and the Doppler continuity equation methods yield accurate estimates of mitral valve area in Lutembacher syndrome.  (+info)

Trans-catheter therapy of Lutembacher syndrome: a case report. (2/4)

Lutembacher syndrome refers to the rare combination of a congenital atrial septal defect and acquired mitral stenosis. Traditionally, Lutembacher syndrome has been corrected by surgical treatment. We describe two patients treated percutaneouly with a combined Inoue balloon valvuloplasty and septal defect closure using the Amplatzer septal occlusion device.  (+info)

Percutaneous treatment of Lutembacher syndrome in a case with difficult mitral valve crossing. (3/4)

Most cases of combination congenital cardiac anomalies are treated with open-heart surgeries because the coexisting anomalies change the cardiac anatomy in an adverse way, making catheter manipulations complex. Lutembacher syndrome is a combination of acquired mitral stenosis and congenital ostium secundum atrial septal defect. The large defect in the septum makes an Inoue balloon catheter unstable, which provides excessive space for free floatation of the catheter, making its passage into the left ventricle difficult by Inoue technique. We present a case of elective definitive percutaneous treatment of Lutembacher syndrome, discussing the technical difficulties faced in mitral valve crossing and reviewing the possible strategies to improve chances of success.  (+info)

Partial type of common atrioventricular canal defect associated with mitral stenosis. (4/4)

We report a 63-year-old woman, with a partial type of common atrioventricular canal and mitral stenosis, who was hospitalized because of dyspnea on exertion. Two-dimensional echocardiogram showed an ostium primum atrial septal defect with two well-formed AV valves located at the same level. However, both anterior and posterior mitral leaflets were markedly thickened with a thickened subvalvular apparatus, and the commisures were fused. Echocardiographic measurements demonstrated a mitral valve area of 1.48 cm2 with mild mitral regurgitation. Cardiac catheterization demonstrated mild pulmonary artery hypertension with a large left to right shunt (72%) at the atrial level. The combination of the partial type of common atrioventricular canal and mitral stenosis is rare; only one similar case has been reported previously in the literature.  (+info)

Lutembacher syndrome is a rare heart condition that is characterized by the presence of both an atrial septal defect (a hole in the wall between the two upper chambers of the heart) and mitral stenosis (narrowing of the mitral valve, which controls blood flow between the left atrium and left ventricle).

In most cases, the atrial septal defect is congenital (present from birth), while the mitral stenosis is acquired, often as a result of rheumatic heart disease. The combination of these two conditions can lead to various symptoms such as shortness of breath, fatigue, and fluid retention.

The syndrome is named after André Lutembacher, a Belgian cardiologist who first described the condition in 1916.

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