Supra-aortic stenosis associated with Marfan's syndrome.

The association of arachnodactyly with other skeletal abnormalities and with ocular and cardiac malformations is now well recognized, and numbers of cases illustrating the syndrome have been reported in recent years. The main cardiac malformations have been degeneration of the aortic media with dissecting aneurysm or aneurysmal dilatation, usually of the ascending aorta, but coarctation, changes in the pulmonary artery, atrial septal defect, and valvular lesions have also been described. The mitral valve is said to be affected more frequently but the aortic valve also may show thickening of the cusps. Aortic regurgitation is common, due to dilatation of the ring rather than to the valvular lesions. The following case is of interest in that Marfan's syndrome was accompanied by aortic stenosis of a type apparently not previously reported.