Analysis of the Risk Associated With Pregnancy in Women With Marfan Syndrome

Abstract

regnancy in patients with Marfan syndrome (MFS) is associated with the potential for lethal cardiovascular events and transmission of mutant genes to the children.1 Although aortic dissection is the most serious complication in patients with MFS, pregnancy increases the risk of such complications2,3 because of the maternal cardiovascular changes such as increases in the blood volume, heart rate, and stroke volume.4 Previous studies demonstrated that the risk factors for aortic complications during the perinatal period were an enlarged aortic root at the beginning of pregnancy, rapid growth of the aortic root, a family history of premature aortic dissection, and the presence of significant aortic regur gitation. 5 Small aortic diameter, however, cannot preclude the occurrence of aortic dissection during the perinatal period. Article p 2545 Severity of Cardiovascular Complications During Pregnancy In the early 1990s, the maternal and fetal mortality rates in MFS patients who developed type A aortic dissection during pregnancy were 30% and 50%, respectively. 6 Although the mortality rate has improved since then, women with MFS are still at risk of aortic complications during the perinatal period. A recent review1 presented 39 women who had MFS and experienced perinatal cardiovascular complications: acute aortic dissection was reported in 29 patients, including 21 patients with type A dissection; 23 patients developed acute aortic dissection during pregnancy, and 6 patients did after delivery. In 19 patients, dilatation of the aorta was diagnosed prior to pregnancy and 4 patients had a history of aortic surgery. Surgical intervention was required in 32 patients, of whom 7 underwent emergency surgery during pregnancy, because of acute aortic dissection. Among the patients with emergency surgery, operative maternal death was reported for 2 mothers, of whom 1 had intrauterine fetal death. Six fetuses survived but 1 had brain atrophy. Among the 39 patients in the review, 5 fetal deaths were reported, including 3 intrauterine fetal deaths and 2 elective abortions. Maternal death occurred in 5 cases. There was 1 prepartum death from multiple organ failure 4 days after emergency aortic surgery for acute aortic dissection that occurred at 26 weeks of pregnancy. The baby also died in utero during the surgery. One patient died 14 days after delivery from a ruptured aortic dissection. Three patients died late after delivery, because of dissection, endocarditis, and subarachnoid hemorrhage, respectively. Incidence of Cardiovascular Complications During Pregnancy It is difficult to clarify the incidence of cardiovascular events during pregnancy in patients with MFS because the severity of the patients differs among reports. A higher incidence of cardiovascular complications may be associated with a higher proportion of high-risk patients. Therefore, there is a bias toward a higher incidence of aortic events in publications from referral cardiovascular centers. In this issue of the Journal, Katsuragi et al report a relatively high incidence of aortic events compared with previous studies;7 the reported prevalence of perinatal aortic dissection in patients with MFS was 3.9%.8–10