Pregnancy-associated aortic dilatation or dissection in Japanese women with Marfan syndrome.

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 2nd trimester involved aortic dilatation > 60mm and those patients underwent hemiarch replacement and a David operation, respectively. Delivery by cesarean section (64% vs. 18%, P < 0.05), sinus of Valsalva ≥ 40mm (86% vs. 21%, P < 0.05), aortic size index (size of sinus of Valsalva/body surface area) ≥ 25 mm/m² (7/7, 100% vs. 0/14, 0%, P < 0.0001), and faster growth of the sinus of the Valsalva (median, [interquartile range]: 0.41 mm/month [0.23-0.66 mm/month] vs. 0.05 mm/month [-0.13 to 0.22 mm/month]; P < 0.05) were significantly higher in group D than in group ND. CONCLUSIONS A large sinus of Valsalva, increased aortic size index, and rapid growth of the sinus of Valsalva are risk factors for aortic dilatation or dissection in pregnant Japanese women with Marfan syndrome.