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Pregnancy in Marfan syndrome after aortic root replacement: a case report and review of the literature.

OBJECTIVE
Marfan syndrome is an inheritable connective tissue disorder with multisystem involvement and variable expression of signs and symptoms, caused by mutations within the fibrillin gene on chromosome 15q21. The reported incidence of severe cardiovascular events during pregnancy in Marfan patients' series is 3-7%, and the overall risk of fatal complication is approximately 1%. Although acute aortic dissection is a rare complication, an association between pregnancy and aortic dissection has been reported even in the absence of preconception aortic root dilatation.


DESIGN
Case report and literature review.


RESULTS
A 29-year-old pregnant woman with Marfan syndrome after replacement of ascending aorta with aortic valve conservation. She had a normal pregnancy and was delivered at term by cesarean section.


CONCLUSION
Successful pregnancy and delivery can be achieved in Marfan's patients after aortic root replacement.