%0 Case Reports
%T Loeys-Dietz syndrome in pregnancy.
%A Thomas KE
%A Hogan J
%A Pitcher A
%A Mackillop L
%A Blair E
%A Frise CJ
%J Obstet Med
%V 14
%N 1
%D Mar 2021
%M 33995573
暂无%R 10.1177/1753495X19852819
%X Loeys-Dietz syndrome is a recently described condition which causes cardiovascular, craniofacial, neurocognitive and skeletal abnormalities due to mutations in components of the transforming growth factor-β signalling pathway. Associated vascular abnormalities include vessel tortuosity and an increased incidence of vascular dissection. Pregnancy increases the risk of aortic dissection compared to non-pregnant individuals and an underlying condition such as Loeys-Dietz syndrome increases this further. While aortic dissection is well described in pregnancy in Loeys-Dietz syndrome, some women can have uncomplicated deliveries, particularly when the risks of the condition are actively managed. Such pregnancies should be considered high-risk, and women should be counselled and managed accordingly. Here we describe two pregnancies in one woman, both with successful outcomes, followed by a summary of the key management principles.