Title:Management and Outcomes of Aortic Dissection in Pregnancy with Marfan Syndrome: A Systematic Review
Volume: 18
Issue: 3
Author(s): Hassan Al-Thani, Ahammed Mekkodathil and Ayman El-Menyar*
Affiliation:
- Clinical Research, Trauma and Vascular Surgery Section, Hamad Medical Corporation, Doha,Qatar
Keywords:
Pregnancy, CVD, Marfan syndrome, aortic root, aneurysm, dissection.
Abstract:
Background: In Marfan Syndrome (MFS), aortic dilatation is one of the main cardiovascular
manifestations which deteriorate due to the physiological changes during pregnancy. We aimed to assess
the up-to-date management and outcomes of aortic root dilation and dissection (AoD) in pregnancy
with MFS.
Patients and Methods: A systematic review was conducted and reported according to the Preferred
Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Original studies published
between January 1, 2001 and December 31, 2018 and which described the management and/or outcomes
of AoD during or after pregnancy in women with MFS were included. Literature searches were
conducted. The PubMed search was performed using terms "Marfan Syndrome" [Mesh] and "Pregnancy"
[Mesh] whereas the Google Scholar search was for “Marfan” and “Pregnancy”, all words anywhere
in the article.
Results: The literature search yielded 177 articles on PubMed and 13,900 articles on Google Scholar.
Assessment of full-text articles for eligibility after removal of duplicates from both databases yielded 12
eligible studies to be included in the final review.
Conclusion: Women with MFS are at high risk of aortic dissection during pregnancy and women with
aortic root 41-45 mm should consider avoiding pregnancy. Guideline-specific management of aortic
aneurysms in pregnancy will reduce the risk of dissection. Diagnosis and Management of MFS need
a multidisciplinary approach and team that should start working early in pregnancy. Further studies are
needed to optimize medical and surgical approaches in addition to preconception counselling in highrisk
subjects.