CARDIOVASCULAR PROBLEMS IN PREGNANT-WOMEN WITH THE MARFAN-SYNDROME

Purpose: To review the available information on the diagnostic, progno stic, and therapeutic aspects of cardiac complications in women with t he Marfan syndrome during the peripartum period and to develop guideli nes for the approach to these patients on the basis of this informatio n. Data Sources: A MEDLINE search and a manual search of bibliographie s from reviewed articles. Study Selection and Data Extraction: Article s that reported on pregnancy in patients with the Marfan syndrome or t hat discussed potentially relevant aspects of the syndrome. Results: P regnancy in the Marfan syndrome is associated with two primary problem s: potential catastrophic aortic dissection and the risk for having a child with the syndrome. The risk for peripartum aortic dissection is especially high in women in whom aortic root dilatation is diagnosed b efore pregnancy. Gestation seems to be safer in women without preexist ing cardiovascular disease; however, an event-free pregnancy cannot be guaranteed, The Marfan syndrome is inherited in an autosomal dominant manner, and the fetus has a 50% risk for inheriting the mutant gene. Conclusions: Women with the syndrome should be counseled before concep tion about the risks of pregnancy to both mother and fetus. Because pr econceptual dilatation of the ascending aorta seems to be an important predictor for aortic dissection, it should be excluded before pregnan cy. Transesophageal echocardiography seems to be preferable for noninv asive assessment of aortic dilatation before and during pregnancy, Pro phylactic use of beta-blockers may be useful in preventing aortic dila tation. Surgery should be considered during gestation in patients with progressive aortic dilatation when or before the aortic roof reaches. 5.5 cm. Because of the potential risk of ionizing radiation to the fe tus, noninvasive methods such as transesophageal echocardiography and magnetic resonance imaging are preferred to contrast aortography for t he diagnosis of aortic dissection during pregnancy. Vaginal delivery c an be done in patients with the Marfan syndrome who do not have cardio vascular system abnormalities. In patients with aortic dilatation, aor tic dissection, or other important cardiac abnormalities, cesarean sec tion should be the preferred method of delivery.