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  • Title: [Two cases of Marfan syndrome complicated with aortic dissection during pregnancy].
    Author: Maruyama T, Totsuka N, Akahane K, Yoshioka J, Shinohara M, Kouzu S, Fujii N, Yajima H, Shimotori M.
    Journal: Kokyu To Junkan; 1993 Jan; 41(1):85-8. PubMed ID: 8434165.
    Abstract:
    Two women with Marfan syndrome who developed aortic dissection during pregnancy are reported. Case 1: A 38-year-old woman had experienced severe back pain five years before (at the age of 33) when she was in the 38th week of gestation, followed by labour with no problem. Three years ago, she was examined in our hospital for enlarged thoracic aorta pointed out in a somatoscopy and was diagnosed as having Marfan syndrome and aortic dissection of DeBakey type IIIb. Because her aortic dissection has been growing larger recently, surgical repair is scheduled electively. Case 2: A 32-year-old woman, who had been diagnosed as having Marfan syndrome because of ectopia lentis at 10 years old, was referred to our department for a painful tumor with pulsation which had emerged at the right-hand side of her neck in the 32nd week of gestation. Acute aortic dissection of DeBakey type I complicated with acute aortic regurgitation was diagnosed, and emergency operation (Cesarean section, reconstruction of ascending aorta and aortic arch, replacement of aortic valve and aorto-coronary bypass to right coronary artery) was performed. Post-operative course was uneventful and the patient and her baby were discharged safely. In general, aortic dissection during pregnancy of patients with Marfan syndrome is of poor prognosis. Full consent by the patient and her family is necessary to decide whether the pregnancy should go on, and close cooperation among participating departments is indispensable for the treatment for this condition.
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