HEART - Marfan's Syndrome

The heart is markedly enlarged and shows a fibrinous pericarditis which in this case is secondary to open heart surgery. There is thickening and ballooning of the tricuspid valve. The same changes are present to a greater degree in the mitral valve and just above the mitral valve, there is some endocardial thickening consistent with a "jet" lesion. The aortic valve has been replaced by a prosthesis and a Dacron graft has been placed inside the aorta.
Marfan's syndrome was diagnosed at the age of four years in this woman who died at age 33. Some time before her death it became apparent that she had an annulo-ectasia of the aortic root with aortic regurgitation. She also had signs of mitral valve prolapse. Following replacement of the aortic valve and insertion of a graft in the aorta, she suffered a cardiac arrest, developed acute tubular necrosis and died. At autopsy the heart weighed 1400 grams.