The impetus to investigation of cardiovascular complications in the Marfan syndrome was the descriptions of aortic dilatation and dissecting aneurysm1,2 in 1943. A number of necropsy series3-8 subsequently published showed increased frequency of cardiovascular lesions. These reports focused their attention on the aortic pathology (dissecting aneurysm, fusiform aneurysm, and dilated aortic ring), with little emphasis placed on other vascular abnormalities. Thickening, wrinkling, nodularity, shortened chordae tendineae, and other deformities of the mitral valve were alluded to as incidental findings.
Recent reports9-15 have stressed the increased frequency with which significant mitral insufficiency is being recognized in the Marfan syndrome. However, association of this valvular defect with a calcified mitral annulus has been infrequent.6,15
We report a patient with the Marfan syndrome who had mitral insufficiency and massive calcification of the annulus fibrosus.
This 21-year-old white woman was referred to the Naval Hospital, Great Lakes, 111,
Grossman M, Knott AP, Jacoby WJ. Calcified Annulus Fibrosus With Mitral Insufficiency in the Marfan Syndrome. Arch Intern Med. 1968;121(6):561–563. doi:10.1001/archinte.1968.03640060075014
Customize your JAMA Network experience by selecting one or more topics from the list below.