Marfan syndrome is an inherited disorder that affects connective tissue throughout the body. The two most affected parts of the body are the eyes and aorta. People with Marfan syndrome tend to have vision problems due to a dislocated lens, and the aorta can become weakened and stretch. This stretching can lead to an aortic aneurysm or even a tearing of the aortic wall (aortic dissection). Both an aortic aneurysm and aortic dissection are very serious and can be life threatening. Additional problems include mitral valve prolapse and aortic valve regurgitation. These two issues can cause symptoms such as shortness of breath, fatigue and palpitations [1].

Complications:

  • Pregnancy in patients with Marfan syndrome have a 1% risk of developing serious cardiac complications. This risk increases as aortic root dimensions increase. It is important to perform an echocardiography prior to pregnancy to find these dimensions. Aortic root diameters <4.5cm is desirable. Anything more than this is associated with as high as a 10% risk of maternal mortality [2].
  • Women with Marfan syndrome have a greater chance of developing aortic dissection and/or rupture during pregnancy due to the change in hormones weakening the aortic wall. This risk is also increased during labor and delivery when pressure in the aorta increases [2].
  • Pregnancy in women with Marfan Syndrome are labeled as high risk. It is important that the aorta of the mother is evaluated at least every three months [3].
Aortic Dissection

Treatment [2]:

  • Beta blockers can be used to reduce the risk of aortic rupture and control blood pressure
  • General anesthesia and a cesarean section for delivery should be considered to maximize hemodynamic control; however, not all patients are the same, and it is important to have an obstetrician familiar with the risks of Marfan Syndrome so they can choose the best method of delivery

 Sources:

  1. Marfan syndrome. NIH Web site. https://ghr.nlm.nih.gov/condition/marfan-syndrome. Updated March, 2012. Accessed July, 2017.
  2. Naderi, S, Raymond R. Pregnancy and heart disease. Dis manag. 2014. http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/cardiology/pregnancy-and-heart-disease/.
  3. Pregnancy. The Marfan Foundation Web site. http://www.marfan.org/resources/patients/pregnancy. Accessed July, 2017.

By: Stephanie Kramer