RESUMO
Aortic dissection is a life-threatening disease that requires immediate surgical intervention. Marfan syndrome is a hereditary disease with an autosomaldominant transmission, which affects the connective tissue, with skeletal, cardiovascular and ocular involvement. It is one of the most prevalent connective tissue disorders, presenting a risk of aortic dissection of approximately 1% even without dilatation of the aorta. When dissectionoccurs during pregnancy and requires surgical intervention (type A dissection), maternal mortality is high (20%-30%). We report a 38 year-old woman with Marfan syndrome that hadan acute type A aortic dissection and severe aortic regurgitation at 37 weeks of gestation. The patient underwent a cesarean section and delivered a healthy baby. Afterwards, aortic valverepair and ascending aortic replacement was successfully performed under circulatory arrest with deep hypothermia. Additionally mitral valve repair for degenerative disease with posterior, autologous pericardium mitral valve ring was performed.