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  • Title: [Surgical treatment of cardiovascular manifestations of Marfan's syndrome].
    Author: Lange R, Ebert E.
    Journal: Herz; 1999 Dec; 24(8):634-41. PubMed ID: 10652677.
    Abstract:
    Patients with Marfan's syndrome suffer mainly from the cardiovascular manifestations of the disease, in particular the acute dissection or rupture of the dilated aorta. Due to improved diagnostic and early surgical intervention the life expectancy of these patients could be considerably improved. However, rupture is still the most frequent and dissection the second frequent cause of death. Life threatening complications of aortic dissection are pericardial effusion, aortic insufficiency and malperfusion syndrome, due to obstruction of aortic branches. Dissection of the ascending aorta is treated by implantation of a valved conduit with reimplantation of the coronary arteries. Some surgeons favor the complete replacement of the ascending aorta with preservation of the aortic valve, although long-term results show some development of aortic insufficiency after this procedure. Based on the experience of the last years, most surgeons prefer the prophylactic replacement of the aorta in Marfan patients, i.e. before complications have occurred. A special treatment algorithm helps to define the indication for the operative treatment in different manifestations of the disease. The low mortality of the elective replacement of the ascending aorta in contrast to replacement in emergency cases speaks in favor of the early operative treatment. For the long-term prognosis of the patient a closed and continuous cardiologic surveillance is mandatory. The patient should be close to a center with the necessary diagnostic tools and with sufficient experience with the medical and surgical treatment, in order to further improve the life expectancy in the future.
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