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Case Reports in Obstetrics and Gynecology
Volume 2012 (2012), Article ID 490169, 3 pages
http://dx.doi.org/10.1155/2012/490169
Case Report

Acute Aortic Dissection in Pregnancy in a Woman with Undiagnosed Marfan Syndrome

1Department of Obstetrics and Gynaecology, Women’s and Children’s Hospital, Adelaide, SA 5006, Australia
2Department of Cardiothoracic Surgery, Flinders Medical Centre, Adelaide, SA 5142, Australia

Received 25 October 2012; Accepted 26 November 2012

Academic Editors: A. Awonuga, S. Z. A. Badawy, O. Oyesanya, and B. Piura

Copyright © 2012 Mandana Master and Gavin Day. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

We report a case of acute aortic dissection in a lady of 28 weeks of gestation with undiagnosed Marfan syndrome. The patient had been seen in our antenatal clinics. Her history documented in her pregnancy record was negative for genetic/congenital abnormalities. There was no family history documented. Subsequently, at 28 weeks of gestation, the patient presented with sudden onset chest, jaw, and back pain. Further history revealed that her father had died at the age of 27 of an aortic dissection. Echocardiography showed aortic root dissection with occlusion of aortic branches. She subsequently underwent an emergency lower segment caesarean section followed by surgical repair of type A dissection. A simultaneous type B dissection was managed conservatively. On later examination, our patient fulfilled the diagnostic criteria for phenotypic expression of Marfan syndrome. Genetic testing also confirmed that she has a mutation of the fibrillin (FBN 1) gene associated with the disease.