Acute Type A Dissection during Pregnancy with Marfan's Syndrome.

Abstract

Background  Marfan's syndrome (MFS) is a connective tissue disorder, caused by a mutation in the FBN-1 gene and affecting multiple organ systems including the vascular system. During pregnancy, women with MFS have an increased risk of aortic dissection due to changes in the cardiovascular system. Case Description  We present a 39-year-old woman that suffered from an acute aortic dissection in week 33 of her third pregnancy who urgently required lifesaving surgery. Three-dimensional reconstruction of computed tomography scan was performed with unborn child in utero. Conclusion  Monitoring of patients with suspected or confirmed MFS appears essential and interdisciplinary treatment and prevention strategies are required.