AORTIC SURGERY IN MARFAN'S SYNDROME. HEMODYNAMIC AND HISTOLOGIC RESPONSE

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Abstract
A patient with Marfan''s syndrome, aneurysmal dilatation of the ascending aorta, and severe aortic regurgitation had an artificial (Hufnagel) valve inserted in the descending thoracic aorta. Cardiac output and the reciprocal of the downslope of dye dilution curves were similar before and after operation. The patient died 3 months after operation from a tear in the aorta at the site of the prosthetic valve. At autopsy an aneurysm of the abdominal aorta was demonstrated in addition to the aneurysm of the ascending aorta. Degeneration of the elastica was present in the aortic media of both aneurysms. The severity of the elastic degeneration correlated well with the degree of aortic widening. The fatal aortic tear occurred superior to the prosthesis where smooth muscle necrosis of the media secondary to the operative procedure was combined with pre-existing change. It is concluded that the aorta of Marfan''s syndrome tolerated incision poorly in regions of elastic medial degeneration. Since medial elastic degeneration occurs in dilated vessels of a wide variety of cardiac disorders, the histologic observations reported herein may have general significance.

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