Pregnancy for a woman with Marfan's syndrome can be hazardous for both her and the fetus, making it a high-risk pregnancy.
Key issues:
(1) genetic and pre-pregnancy counseling
(2) risk of aortic dissection or rupture of aneurysms
(3) complications of antihypertensive medications
(4) complications of substance abuse
(5) complications of pregnancy-related hypertension
Since Marfan syndrome shows autosomal dominant inheritance the fetus will probably be affected.
There is an increased risk for aortic dissection, especially:
(1) if the maximal aortic dimension is > 4.0 cm
(2) during third trimester, delivery and the early post-partum period
Consequently, echocardiography is performed during pregnancy to monitor aortic root size.
ACE inhibitors or ARBs can be teratogenic for the fetus and need to be discontinued. Beta-blockers may be continued.
The risk of aortic dissection may increase with:
(1) substance abuse with cocaine or amphetamines
(2) pre-eclampsia or eclampsia
