Maternal complication of pregnancy in Marfan syndrome

Int J Cardiol. 2009 Aug 14;136(2):156-61. doi: 10.1016/j.ijcard.2008.04.035. Epub 2008 Jul 15.


Objectives: Evaluate the risk of aortic complications (aortic dissection or prophylactic aortic surgical replacement) associated with pregnancy in Marfan syndrome in the absence of specific care.

Background: Data are scarce on aortic complications during pregnancy in Marfan syndrome.

Methods: Retrospective study on a large population (415 patients) followed up in our multidisciplinary out-patient clinic devoted to Marfan syndrome. Women over 18 years of age were divided into 2 groups: 85 had been pregnant (MFSP+) giving birth to 136 children through 160 pregnancies; 68 had not been pregnant (MFSP-). The occurrence of aortic complication was compared between the 2 groups using a piecewise discrete-time model.

Results: In MFSP+, 7 aortic complications occurred during 160 pregnancies (4.4%) and 17 aortic complications occurred during 1870 years of follow-up out of pregnancy. In MFSP-, 14 aortic complications occurred over 940 years of follow-up. Pregnancy was associated with a 5 fold increase in risk of aortic complication, which did not translate into increased risk during life, suggesting that pregnancy may act as a revealer in women prone to aortic complication.

Conclusions: Pregnancy is associated with a transient increased risk of aortic complication in the absence of specific care.

MeSH terms

  • Adolescent
  • Adult
  • Aortic Aneurysm / epidemiology*
  • Aortic Aneurysm / surgery
  • Aortic Dissection / epidemiology*
  • Aortic Dissection / surgery
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Humans
  • Marfan Syndrome / epidemiology*
  • Middle Aged
  • Mitral Valve Insufficiency / epidemiology*
  • Mitral Valve Insufficiency / surgery
  • Pregnancy
  • Pregnancy Complications, Cardiovascular / epidemiology*
  • Retrospective Studies
  • Risk Factors
  • Young Adult