title: Pregnancy-associated aortic dilatation or dissection in Japanese women with Marfan syndrome.
abstract: 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.
  
  
  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.
authors.affiliation: Department of Perinatology and Gynecology, National Cerebral and Cardiovascular Center, Suita, Japan. skatsura12@yahoo.co.jp
authors.forename: Shinji
authors.initials: S
authors.lastname: Katsuragi
authors.forename: Keiko
authors.initials: K
authors.lastname: Ueda
authors.forename: Kaoru
authors.initials: K
authors.lastname: Yamanaka
authors.forename: Reiko
authors.initials: R
authors.lastname: Neki
authors.forename: Chizuko
authors.initials: C
authors.lastname: Kamiya
authors.forename: Yoshihito
authors.initials: Y
authors.lastname: Sasaki
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Osato
authors.forename: Koichiro
authors.initials: K
authors.lastname: Niwa
authors.forename: Tomoaki
authors.initials: T
authors.lastname: Ikeda
mesh_terms: D000328:Adult; D001019:Aortic Rupture / Q000150:complications / Q000401:mortality* / Q000473:pathology / Q000601:surgery; D044466:Asian People; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008382:Marfan Syndrome / Q000150:complications / Q000401:mortality* / Q000473:pathology / Q000601:surgery; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000401:mortality* / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D012850:Sinus of Valsalva / Q000473:pathology