Clinical and cardiovascular impact of pregnancy in women with Marfan syndrome.

BACKGROUND

Marfan syndrome (MS) is associated with mutations in the FBN1 gene, which encodes fibrillin-1, an essential protein in elastic fibers. Cardiovascular damage in MS has serious complications such as aneurysms and aortic dissection (AD), which increase during pregnancy.

OBJECTIVE

To evaluate the cardiovascular and clinical status of women with Marfan syndrome and pregnancy.

MATERIALS AND

METHODS

Retrospective study in women with MS or other connective tissue diseases (CTD) classified according to the 2010 Ghent criteria.

RESULTS

One hundred women were included, 35 were admitted as pediatric patients, and 65 as adults. Ten women without medical records were excluded. Forty-eight non-pregnant women and 46 pregnant women were analyzed. 20 births, 26 cesarean sections, 9 preeclampsia, and 14 miscarriages. Of the total group, 14 had con-genital heart disease, 36 had AD, 47 had aortic regurgitation, 22 had mitral valve regurgitation, 15 had tricuspid valve regurgitation, 39 had valve prolapse, 67 had surgeries, and 15 had stents. 19 died, 5 due to aneurysm rupture, 4 had AD, 3 had cardiogenic shock, 5 had septic shock, and 2 had hypovolemic shock.

CONCLUSIONS

Cardiovascular damage in pregnancies of women with MS or CTD is complex. The diagnosis of MS is late in some cases; it is at the time of delivery, and genetic counseling is not timely. These are areas of opportunity to impact on prevention, timely gynecological and obstetric status, and surgery.