Patent Interauricular Septum Associated with Mitral Stenosis: Lutembacher's Syndrome

Abstract

Two cases studied in the Department of Radiology at Duke Hospital present such interesting and unusual roentgen features that they are reported here in some detail. Both are instances of a defective interauricular septum, associated with mitral stenosis, and the correlation between the resultant anatomical changes and the x-ray findings is noteworthy. Patency of the interauricular septum is relatively common, but the co-existence of stenosis of the mitral valve is rare. The latter condition, though it presents a varied clinical picture, produces a roentgenogram which is characteristic; yet, unless the roentgenologist is aware of the syndrome, unfortunate misinterpretations are apt to ensue. A careful fluoroscopic study of the chest in which a “roentgenographic dissection” of the heart is accomplished will always help in clarifying the picture. Literature There are 26 cases in the literature of co-existent mitral stenosis and patency of the interauricular septum. The first report was presented in 1865 by Martineau (1). Subsequently, several French investigators added to the list. Abbott (2) reported her cases in 1915, and one year later Lutembacher (3) produced a comprehensive survey of the condition. Since his contribution, the combination of lesions has been called Lutembacher's disease. Dressler and Rosler (4) in 1930 added a new case and discussed the roentgen findings. McGinn and White (5), in 1933, reviewed all the reported cases and recorded a case of their own. Since that time, Dr. Paul White (6) has studied another case which has not yet been published. Roesler (7) and Kirshbaum and Perlman (8) have recently written about the condition (1934 and 1939). Embryology The primitive atrium is at first a single chamber, but the topical modification into two portions is distinct very early. At the time that the single chamber is constricted by pressure from the developing bulbar limb of the primitive heart, the various septa are forming on the inner walls (9). At first, there is a sickle-shaped fold formed along the anterior and posterior walls of the atrium; this is the septum primum. This septum grows downward from the posterior upper wall and finally reaches the opening from the atrium into the ventricular cavity. Its line of origin, however, gradually thins out as the edges thicken and there finally appears an opening at or near its origin, the foramen ovale. As this septum separates from its line of attachment on the posterior wall, another leaflet, the septum secundum, appears to the right of it. The two atria are now open by a wide communication. The final growth of the septa is as follows. Septum I, which has grown along the anterior wall and formed the foramen ovale at its original attachment posteriorly, now extends backward to the posterior and superior walls so that its free edge, directed toward the original foramen ovale, extends forward and downward over the upper wall; hence its free edge now faces backward and downward.