Marfan's syndrome with aneurysm of ascending aorta and aortic regurgitation. Surgical treatment and new histochemical observations

A 16 year old girl with Marfan's syndrome, severe aortic regurgitation and ascending aorta aneurysm was treated successfully with replacement of the aortic valve and ascending aorta. The patient is the youngest known patient with Marfan's syndrome to have undergone this dual procedure. The reported cases with Marfan's syndrome and aortic regurgitation treated surgically were reviewed. The main causes for the post-operative deaths in these patients were hemorrhage, aortic dissection and persistent or recurrent aortic regurgitation. Aortic valve replacement and repair of the aortotomy with the use of Teflon felt enforcement at the suture lines appear to be effective ways to overcome some of the associated difficulties with the correction of these lesions. Large accumulation of dermatan sulfate or heparitin sulfate, in addition to chondroitin-sulfate, was found in the media of the resected aortic wall.