Open Access
REVIEW
Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature
Katherine Smith, Bernard Gros
University of Central Florida College of
Medicine
* Corresponding Author: Bernard Gros, 6850 Lake Nona Blvd., Orlando, FL 32827. Email:
Congenital Heart Disease 2017, 12(3), 251-260. https://doi.org/10.1111/chd.12465
Abstract
A well-established association exists between acute aortic dissection and pregnancy, particularly in
women with Marfan syndrome. However, there is debate regarding appropriate management
guidelines. In particular, there are differing opinions regarding when prophylactic aortic root repair
should be recommended as well as the efficacy of beta blockers in this clinical scenario. The current study evaluated 10 years of published literature (2005-2015) in the PubMed/Medline
database. Fifty articles, describing 72 cases of women who presented with aortic dissection in the
antepartum or postpartum period were identified. Comparisons on demographic variables and clinical outcomes between cases of women with Marfan syndrome (n = 36) and without Marfan
syndrome (n = 36) were conducted. There were no significant differences in demographics (age,
gravidity, parity) between the Marfan and non-Marfan cases. Marfan patients presented with antepartum dissections significantly earlier in pregnancy than those without Marfan syndrome
(P = .002). However, there were no significant difference between the 2 groups in maternal mortality, fetal mortality, or obstetric outcomes (mode of delivery and gestational age at delivery).
Eight cases described events in Marfan women with an aortic root diameter ≤40 mm. Six events
occurred in Marfan women who were managed with beta blockers. Current guidelines rely on aortic root diameter for stratification of Marfan women into risk categories, but we identified several
cases that would be missed by these guidelines. Specifically, the existing literature suggest that
women with Marfan syndrome should take precautions throughout pregnancy, rather than the
third trimester.
Keywords
Cite This Article
Smith, K., Gros, B. (2017). Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature.
Congenital Heart Disease, 12(3), 251–260. https://doi.org/10.1111/chd.12465