Maternal and fetal complications of pregnancy in the Marfan syndrome

The medical literature reports 32 women affected by the Marfan syndrome who had at least one pregnancy; 16 died of and four survived acute aortic dissection. Most of the women who suffered an aortic complication in association with pregnancy had pre-existing aortic regurgitation, aortic root dilatation or other severe cardiovascular problem. Because women with the Marfan syndrome usually desire children, our clinic records were reviewed and patients were contacted to determine a more representative estimate of maternal risks. The pregnancy histories of women with the Marfan syndrome were compared with those of spouses of men with the Marfan syndrome and those of mothers of a sporadic (new mutation) child with the Marfan syndrome. One of 26 women with the Marfan syndrome died shortly after pregnancy of endocarditis; she was the only woman to have a severe, pre-existing cardiovascular condition. The prevalences of milder pregnancy-associated cardiovascular and general complications did not differ among the study groups. The rate of early spontaneous abortion was higher in women with the Marfan syndrome than in either control group. These results suggest the risk of maternal death is low in women with the Marfan syndrome who have minimal cardiovascular disease. Women with the Marfan syndrome should be counseled regarding pregnancy risks only after review of their cardiovascular status, including an echocardiographically determined aortic root diameter.