Marfan's syndrome presented with dissected thoraco-abdominal aneurism and pregnancy. A case report
Main Article Content
Keywords
Marfan Syndrome, Aortic Aneuriysm, Thoracic, Pregnancy
Abstract
A 39-year-old patient diagnosed two years previosly with Marfan Syndrome (MS) and thoraco-abdominal aneurysm, both presented with the following symptoms: occasional mild effort dyspnea and thoracic pain. The patient started her current illness at 28 weeks of pregnancy with an exacerbation of a deep, oppressive thoracic pain and orthopnea. The echocardiogram showed a 10 cm diameter aortic aneurysm with involvement of the aortic root, tho-racoabdominal and dissection. The computed tomography reported aneurysmatic dilatation of the aortic root and dissection of the thoracic and abdominal portion. Until the delivery of pregnancy the patient was treated successfully with meto-prolol, prazocin, and diuretics. A cesarean section at 29 weeks of pregnancy was practiced.
References
Dean J. Management of Marfan syndrome. Heart 2002;88:97-103.
Tomihara A, Ashizawa N, Abe K, Kinoshita N, Chihaya K, Yonekura T, et al. Risk of development of abdominal aortic aneurysm and dissection of thoracic aorta in a postpartum woman with Marfan’s syndrome. Intern Med 2006;45:1285-1289.
Immer FF, Bansi AG, Immer-Bansi AS, McDougall J, Zehr KJ, Schaff HV, et al. Aortic dissection in pregnancy: analysis of risk factors and outcome. Ann Thorac Surg 2003;76:309-314
Elkayam U, Ostrzega E, Shotan A, et al. Cardiovascular problems in pregnant women with the Marfan syndrome. Ann Intern Med 1995;123(2):117-122
Weissmann-Brenner A, Schoen R., Divon Y. Aortic dissection in pregnancy. Obstet Gynecol 2004;103:1110-1113.
Elkayam U, Rose J, Jamison M. Vascular aneury-sms and dissections during pregnancy. En: Elkayam U, Gleicher N, editores. Cardiac problems in pregnancy. New York: Alan R. Liss; 1990. p. 215 -229.
Gott VL, Pyeritz RE, Magovern GJ Jr, Cameron DE, McKusick VA. Surgical treatment of aneurysms of the ascending aorta in the Marfan syndrome. Results of composite-graft repair in 50 patients. N Engl J Med 1986;314:1070-1074.
Murgatroyd F, Child A, Poloniecki J, Treasure T, Pumphrey C. Does routine echocardiographic measurement of the aortic root diameter help predict the risk of aortic dissection in the Marfan syndrome? Eur Heart J 1991:12:410. (Abstract).
Meijboom LJ, Vos FE, Timmermans J, Beers GH, Zwinderman AH, Mulder Bj. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005;206:914-920.
Tutarel O, Lotz J, Roentgen P, Drexler H, Meyer GP, Westhoff-Bleck M. Pregnancy in a Marfan patient with pre-existing aortic dissection. Int J Cardiol 2007; 114:E36-E37.
Treasure T. Elective replacement of the aortic root in Marfan syndrome. Br Heart J. 1993;69:101-103.
Pyeritz RE. Maternal and fetal complications of pregnancy in the Marfan syndrome. Am J Med 1981; 71:784-790.
Beighton P. Pregnancy in the Marfan syndrome. Br Med J (Clin Res Ed) 1982;285:464.
Lunel A, Audra P, Plauchu H, Gaucherand P. Mar-fan’s syndrome and pregnancy. J Gynecol Obs-tet Biol Reprod 2006;35:607-613.
Lewis S, Rynder I, Lovell A.T. Peripartum presentation of an acute aortic dissection. Br J Anaesth 2005;94:496-499.
Paulus DA, Layon AJ, Mayfield WR, D’Amico R, Taylor WJ, James CF. Intrauterine pregnancy and aortic valve replacement. J Clin Anesth 1995;7:338-346.
Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J 2003;24:761-781.
Nakazawa M, Aomi S, Akagi T et al. Guidelines for indication and management of pregnancy and delivery in women with heart disease. Circ J 2005; 69:1296-1297.
Therrien J, Gatzoulis M, Graham T, Bink-Boelkens M, Connelly M, Niwa K, et al. Canadian Cardiovascular Society Consensus Conference 2001 update: Recommendations for the management of adults with congenital heart disease Part II. Can J Cardiol 2001;l17:1029-1050.
Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilatation and the benefit of long term ?-adrenergic blockade in Marfan syndrome. N Engl J Med 1994;330:1335-1341.
Milewicz DM, Dietz HC, Miller DC. Treatment of aortic disease in patients with Marfan syndrome. Circulation 2005;111:150-157.