The original description of Lutembacher syndrome was as a combination of atrial septal defect with mitral stenosis [Lutembacher R. De la sténose mitrale avec communication interauriculaire. Archives des maladies du coeur et des vaisseaux, Paris, 1916, 9: 237-260]. Though Lutembacher thought that both atrial septal defect and mitral stenosis were congenital, it is likely that in that sixty one year old lady, the atrial septal defect was congenital and mitral stenosis of rheumatic etiology. Later on several authors called a combination of atrial septal defect with any mitral valve lesion (stenosis, regurgitation or a combination) as Lutembacher syndrome. Some expanded it to include any left to right shunt at the atrial level. Recently even atrial level shunts created by septal puncture for balloon mitral valvotomy has been included in the spectrum of Lutembacher syndrome. Personally I would prefer to restrict the terminology to a combination of congenital atrial septal defect and rheumatic mitral stenosis as would have been the etiology of the original Lutembacher’s case. Presence of the two lesions have significant hemodynamic effects on the other lesion. The large unrestrictive atrial septal defect lowers the left atrial pressure and gradient across the mitral valve so that findings of mitral stenosis may be obscured and so will be the features of pulmonary venous congestion. In the presence of a large atrial septal defect, even a small gradient across the mitral valve is to be taken as significant. The obstruction to the left ventricular inflow on the other hand enhances the left to right shunt across interatrial septum so that torrential left to right shunts are likely in Lutembacher syndrome. This manifests as prominent right ventricular outflow murmur with a thrill. Cardiomegaly is also common. George Joseph and associates from Christian Medical College Hospital, Vellore, India has described complete percutaneous management of Lutembacher syndrome [Definitive percutaneous treatment of Lutembacher's syndrome. Catheter Cardiovasc Interv. 1999;48:199-204]. They closed the atrial septal defect with an Amplatzer septal occluder and dilated the stenotic mitral valve with Joseph balloon mitral valvotomy catheter (JOMIVA balloon).
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