Management of the pregnant woman with Marfan syndrome complicated by ascending aorta dilation.

ABSTRACT

INTRODUCTION: Marfan syndrome is a disorder of connective tissue associated with progressive dilation of the aorta and potential risk for aortic dissection. Women with Marfan syndrome who are, or wish to become, pregnant represent a unique and challenging patient population due to a risk for accelerated aortic growth and aortic dissection during pregnancy. Risk for aortic complications during pregnancy is related to the dimensions of the ascending aorta. Women with an aortic diameter ≥4.5 cm at the start of pregnancy are at higher risk for aortic dissection, and an aortic dimension >4.0 cm is considered a relative contraindication to pregnancy in the setting of Marfan syndrome. CONCLUSION: Multidisciplinary care involving specialists familiar with Marfan syndrome should be emphasized before, during, and after pregnancy with the involvement of Maternal Fetal Medicine, Genetics, Cardiology, Cardiothoracic Surgery, Anesthesia, and other specialties on a case-by-case basis. We review the important aspects of the evaluation and management of pregnant women with Marfan syndrome.