Marfan syndrome diagnosis and management

Ammash, Naser M.; Connolly, Heidi M.

doi:10.24170/4-1-2105

Cited by 4 publications

(1 citation statement)

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“…9,10 The composite risk from Marfan syndrome and pregnancy increases the likelihood of acute aortic dissection (AAD) in these patients. 11 Current recommendations from the World Health Organization (WHO) classify women with MFS into 4 risk categories based on the extent of their cardiovascular condition (Table 1). 12 The WHO classifications are primarily based on aortic root measurements, as this is believed to be the greatest risk factor for the development of AAD.…”

Section: Introductionmentioning

confidence: 99%

Pregnancy-related acute aortic dissection in Marfan syndrome: A review of the literature

Smith

Gros

2017

Congenital Heart Disease

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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.

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Section: Introductionmentioning

confidence: 99%