Family planning can be very stressful and emotionally difficult when one of the parents to be has a genetic disorder of the connective tissue, like Marfan syndrome or a related disorder.
In the case of Marfan syndrome it can even be life-threatening, and appropriate steps should be taken to help prevent any issues from arising.
Prior to any decisions being made, parents to be should understand the many options now available, and the potential risk factors that exist for the child and mother. Parents to be should always consult with a genetic counselor, in order to discuss options and be better informed about the entire process.
Women who have Marfan syndrome should be closely monitored at all stages of pregnancy. Pregnancy poses additional risks to women who are affected by the disorder, because of the increased stress on the heart and blood vessels. Information has the benefit of helping to educate parents and allow them to better make an informed decision, regarding pregnancy and the risks posed to child and mother.
Women who have a history of significant heart valve problems or aortic disease should always discuss pregnancy risks with their doctors, and should consult with a genetic counselor before ever considering pregnancy.
Serious risks, regarding aortic complications such as a tear or rupture is considered significant once the aortic diameter exceeds normal more than 10 percent (4.0 centimeters) However, this does not exclude all risk for women with Marfan Syndrome when the aortic dimension is less than 4.0 centimeters at the onset of pregnancy.
Women with a history of surgical treatment, such as those who have had composite graft surgery (including an artificial aortic valve) of the aortic root need special attention and proper guidance because of the potential for harmful effects of blood thinners, such as warfarin (Coumadin) on the developing fetus. Although it is thought that prior aortic root surgery decreases the risks associated with pregnancy for women with Marfan syndrome, this does not under any circumstances eliminate all risk factors, since other aortic segments can enlarge and tear.
All women who become pregnant and who have Marfan syndrome should be considered at “high-risk” and their aorta should be evaluated by echocardiography at least every three months, and sooner when appropriate.
The processes of delivery should be by the least stressful method possible. At this time, there is controversy regarding whether a controlled vaginal delivery or a Cesarean section imposes less stress for the majority of women with Marfan syndrome. The most suitable delivery method for a particular woman should only be made after careful consultation with an obstetrician familiar with all the relevant issues, regarding pregnancy. Cesarean section is not an absolute necessity because of Marfan syndrome, but may be indictated for any of the usual reasons that would apply to any pregnancy.
Women with Marfan syndrome are often advised to complete childbearing earlier in life if possible.
Medicine and Pregnancy:
There are certain medications used in the treatment of Marfan syndrome can not be used during pregnancy due to a risk of birth defects and fetal loss. Such medications include, angiotensin converting enzyme inhibitors (such as enalapril or captopril) and angiotensin receptor blockers (such as losartan).