Background: Aortic dilatation and dissection are severe complications of pregnancy that may cause maternal death. The purpose of the present study was to investigate risk factors for aortic dilatation or dissection in pregnant Japanese women with Marfan syndrome.
Methods and Results:A total of 28 patients with Marfan syndrome were investigated retrospectively during pregnancy and after delivery at 1 institution. These patients were divided into 2 groups: those who experienced aortic dilatation or dissection (group D, n=11) and those who did not (group ND, n=17). In group D, aortic dilatation or dissection occurred in 7 cases during pregnancy (2 in the 2 nd trimester, 5 in the 3 rd trimester) and 4 cases after birth. The 2 cases in the 2 nd trimester involved aortic dilatation >60 mm and those patients underwent hemiarch replacement and a David operation, respectively. Delivery by cesarean section (64% vs. 18%, P<0.05), sinus of Valsalva ≥40 mm (86% vs. 21%, P<0.05), aortic size index (size of sinus of Valsalva/body surface area) ≥25 mm/m 2 (7/7, 100% vs. 0/14, 0%, P<0.