Disección aórtica y síndrome de Marfán en el embarazo: A propósito de un caso clínico

Resumen

Aortic dissection is a life-threatening disease that requires
immediate surgical intervention. Marfan syndrome is a hereditary disease with an autosomal
dominant 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 dissection
occurs 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 had
an 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 valve
repair 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