Congenital Mitral Valve Disease Associated with Coarctation of the Aorta

Fifty-three specimens in which the primary diagnosis in life was coaretation of the aorta were examined; only nine had a mitral valve of normal size and configuration. Ten specimens had a normally formed valve mechanism which was small in comparison to both tricuspid valve and left ventricle. Three other specimens had a normally formed but small valve which exhibited a short free margin of the anterior leaflet. The remaining 31 specimens demonstrated various anomalies which included some forms of parachute mitral valve. The spectrum of malformations may be divided into three basic types: some showed fused or closely apposed chordae tendineae, due to nondevelopment of the intervening space; in others the space between the papillary muscles and the ventricular wall was underdeveloped, which prevented the papillary muscles from moving independently of the ventricular wall. The third type was found in a case of parachute mitral valve, where the chordae tendineae were attached solely to the anterolateral papillary muscle, and the posteromedial papillary muscle was inserted directly into the valve, near the annulus. Although the spectrum of congenital anomalies reported here does not include all forms of mitral stenosis, it does indicate that mitral valve disease probably occurs more frequently in coarctation of the aorta than previously was recognized.