Key result
Pregnancy in Marfan syndrome carries high dissection risk, warranting avoidance with aortic roots 41-45 mm.
Why the study?
Aortic dilatation and dissection during pregnancy in women with Marfan Syndrome require updated assessment of management and outcomes due to physiological changes in pregnancy.
What are the management strategies and outcomes for aortic root dilation and dissection in pregnant women with Marfan Syndrome?
Systematic Review (n=12)
What are the management strategies and outcomes for aortic root dilation and dissection in pregnant women with Marfan Syndrome?
Pregnant women with Marfan Syndrome are at high risk of aortic dissection, and those with an aortic root of 41-45 mm should consider avoiding pregnancy, highlighting the need for multidisciplinary preconception counseling.
Aortic risk management in pregnant MFS patients remains incompletely defined; leaves open need for prospective outcome data.
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.
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Al-Thani et al. (2019) conducted a systematic review in Aortic root dilation and dissection in pregnancy with Marfan Syndrome (n=12). Pregnancy was evaluated on Management and outcomes of aortic root dilation and dissection. Pregnancy in women with Marfan Syndrome carries a high risk of aortic dissection, and women with an aortic root of 41-45 mm should consider avoiding pregnancy.
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