title: Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study.
abstract: OBJECTIVE
  To investigate pregnancy outcomes, especially the risk of pregnancy-related aortic dissection (AD), in patients with Marfan syndrome (MFS) after prophylactic aortic root replacement (ARR).
  
  
  METHODS
  Retrospective case series study.
  
  
  METHODS
  Tertiary perinatal care centre at a university hospital.
  
  
  METHODS
  Pregnant women fulfilling the revised Ghent nosology (2010) criteria for MFS who were managed at our institute.
  
  
  METHODS
  The pregnancy outcomes of all patients with MFS managed at our institute between 1982 and September 2016 were reviewed retrospectively based on medical records.
  
  
  METHODS
  Obstetrical management and complication including the incidence of AD throughout the peripartum period.
  
  
  RESULTS
  Among 22 patients (28 pregnancies) who had been managed as potential MFS or related disorders, 14 (17 pregnancies) fulfilled the revised Ghent nosology (2010) criteria for MFS and were enrolled in this study. Five patients (five pregnancies) had received ARR before conception: three (60%) developed type B aortic dissection [AD(B)] during the peripartum period, compared with only one of 10 patients (12 pregnancies) without ARR (P < 0.05, Chi-square test).
  
  
  CONCLUSIONS
  Our study results suggest that MFS patients after prophylactic ARR are still at high risk of AD(B) during the peripartum period. Careful pre-pregnancy counselling and multidisciplinary care throughout the peripartum period are essential for the management of MFS, even after surgical repair of an ascending aortic aneurysm.
  
  
  CONCLUSIONS
  MFS patients after prophylactic ARR are still at high risk of type B aortic dissection during the peripartum period.
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Sayama
authors.affiliation: Department of Cardiovascular Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: N
authors.initials: N
authors.lastname: Takeda
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Iriyama
authors.affiliation: Department of Pediatrics, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Inuzuka
authors.affiliation: Department of Cardiovascular Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Maemura
authors.affiliation: Department of Cardiovascular Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: D
authors.initials: D
authors.lastname: Fujita
authors.affiliation: Department of Cardiovascular Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Yamauchi
authors.affiliation: Department of Cardiovascular Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Nawata
authors.affiliation: Department of Anesthesiology and Pain Relief Center, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Bougaki
authors.affiliation: Department of Obstetrics and Gynecology, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Hyodo
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: R
authors.initials: R
authors.lastname: Shitara
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Nakayama
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Komatsu
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Nagamatsu
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Osuga
authors.affiliation: Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Fujii
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000453:epidemiology / Q000209:etiology / Q000517:prevention & control / Q000628:therapy; D001018:Aortic Diseases / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D005260:Female; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008382:Marfan Syndrome / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology; D058725:Peripartum Period; D011183:Postoperative Complications / Q000453:epidemiology / Q000517:prevention & control / Q000628:therapy; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000453:epidemiology / Q000517:prevention & control / Q000628:therapy; D011256:Pregnancy Outcome; D018566:Pregnancy, High-Risk; D012189:Retrospective Studies; D020379:Risk Adjustment / Q000379:methods; D014656:Vascular Surgical Procedures / Q000009:adverse effects* / Q000379:methods
keywords: Aortic dissection; Marfan syndrome; aortic root replacement; pregnancy