Acute dissection of the thoracic aorta that occurs during pregnancy in Marfan's syndrome is often fatal. To date, 3 cases of successful surgical treatment have been reported worldwide. The case described herein serves as a basis for a discussion of the pathologic features, diagnosis, timing, and extent of the surgical procedures that may be employed.
Get full access to this article
View all access options for this article.
References
1.
Murdoch JL, Walker BA, Halpern BA, et al: Life expectancy and causes of death in the Marfan syndrome. N Engl J Med286:804-808, 1972.
2.
Svensson LG, Crawford ES, Coselli JS, et al: Impact of cardiovascular operation on survival in the Marfan patient. Circulation80 (suppl I):I-223-I-242, 1989.
3.
Ferguson JE, Ueland K., Stinson EB, et al: Marfan's syndrome: Acute aortic dissection during labor, resulting in fetal distress and cesarean section, followed by successful surgical repair . Am J Obstet Gynecol147:759-762, 1983.
4.
Cola LM, Lavin JP: Pregnancy complicated by Marfan's syndrome with aortic arch dissection, subsequent aortic arch replacement and triple coronary artery bypass grafts. J Reprod Med30:685-688, 1985.
5.
Smith VS, Eckenbrecht PD, Hankins GD, et al: Marfan's syndrome, pregnancy, and the cardiac surgeon. Milit Med154:404-406, 1989.
6.
DeBakey ME, McCollum CH, Crawford ES, et al: Dissection and dissecting aneurysms of the aorta: Twenty-year follow-up of five hundred twenty-seven patients treated surgically. Surgery92:1118-1134, 1982.
7.
Bentall H., DeBono A.: A technique for complete replacement of the ascending aorta. Thorax23:338-339, 1968.
8.
Larson EW, Edwards WD: Risk factors for aortic dissection: A necropsy study of 161 cases. Am J Cardiol53:849-855, 1984.
9.
Roberts WC: Aortic dissection: Anatomy, consequences, and causes. Am Heart J101:195-214, 1981.
10.
Roberts WC, Honig HS: The spectrum of cardiovascular disease in the Marfan syndrome: A clinico-morphologic study of 18 necropsy patients and comparison to 151 previously reported necropsy patients. Am Heart J104:115-135, 1982.
11.
Sutinen S., Piiroinen O.: Marfan syndrome, pregnancy and fatal dissection of the aorta. Acta Obstet Gynecol Scand50:295-300, 1971.
12.
Anagnostopoulos CE, Prabhakar Mjs, Kittle CF: Aortic dissections and dissecting aneurysms . Am J Cardiol30:263-273,.1972.
13.
Pyeritz RE: Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med71:784-790, 1981.
14.
Goldman AP, Kotler MN, Scanlon MH, et al: The complementary role of magnetic resonance imaging, Doppler echocardiography, and computed tomography in the diagnosis of dissecting thoracic aneurysms. Am Heart J111:970-981, 1986.
15.
Crawford ES : Marfan's syndrome: broad spectral surgical treatment of cardiovascular manifestations. Ann Surg198:487-505, 1983.
16.
Gott VL, Pyeritz RE, Magovern GJ, et al: Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. N Engl J Med314:1070-1074, 1986.
17.
Cabrol C., Pavie A., Mesnildrey P., et al: Long-term results with total replacement of the ascending aorta and reimplantation of the coronary arteries. J Thorac Cardiovasc Surg91:17-25, 1986.