346. Pregnant with Marfan syndrome and aortic root aneurism: Dissection case report for the third quarter

Abstract

Introduction The main cardiovascular complications of Marfan Syndrome (MS) are valvar disease, heart failure and aortic dissection. Pregnancy in a patient with MS presents greater risk of aortic complications. Aortic root diameter greater than 45 mm is a class IV of modified World Health Organization classification of maternal pregnancy risk (high risk of maternal death or severe morbidity). We discuss a case of aortic dissection in the third trimester in a MS patient with aortic dilation previous to pregnancy. Method Review of medical charts. Case description Patient 40 years old, with MS, previous echocardiogram with aortic root of 54 mm, ascending aorta of 56 mm, and normal aortic arch. She refused therapeutic abortion. During prenatal she remained asymptomatic, under use of metoprolol for 75 mg. A new echocardiogram was made in the second trimester, without alterations. At 30 weeks of gestation, she presented with aortic dissection type Stanford A. A cesarean section was performed under general anesthesia, followed by subtotal hysterectomy, during which the patient remained stable. Subsequently, correction of dissection of the ascending aorta was initiated, with a Benton procedure and right coronary artery bypass. An intraoperative myocardial infarction caused cardiorespiratory arrest reversed during surgery, but a new episode during anesthetic lead to death. The 1370 g live neonate had a good outcome. Discussion Aortic dissection is a severe condition with high rates of mortality. Pregnancy in a patient with aortic dilation is a reason to pregnancy termination, refused by this patient. Even with medical treatment with beta-blocker, she presented a type A aortic dissection, with immediate indication of premature therapeutic cesarean section followed by surgery. Despite prompt diagnosis and treatment, an intraoperative myocardial infarction was the cause of maternal death. This case shows that in spite of optimal care, pregnancy in Marfan syndrome patient with aortic dilation may have a fatal outcome.