Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study

S Sayama; N Takeda; T Iriyama; R Inuzuka; S Maemura; D Fujita; H Yamauchi; K Nawata; M Bougaki; H Hyodo; R Shitara; T Nakayama; A Komatsu; T Nagamatsu; Y Osuga; T Fujii

doi:10.1111/1471-0528.14635

Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study

BJOG. 2018 Mar;125(4):487-493. doi: 10.1111/1471-0528.14635. Epub 2017 May 2.

Authors

S Sayama¹, N Takeda², T Iriyama¹, R Inuzuka³, S Maemura², D Fujita², H Yamauchi⁴, K Nawata⁴, M Bougaki⁵, H Hyodo⁶, R Shitara¹, T Nakayama¹, A Komatsu¹, T Nagamatsu¹, Y Osuga¹, T Fujii¹

Affiliations

¹ Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
² Department of Cardiovascular Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
³ Department of Pediatrics, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
⁴ Department of Cardiovascular Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
⁵ Department of Anesthesiology and Pain Relief Center, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
⁶ Department of Obstetrics and Gynecology, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan.

PMID: 28294527
DOI: 10.1111/1471-0528.14635

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).

Design: Retrospective case series study.

Setting: Tertiary perinatal care centre at a university hospital.

Population: 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.

Main outcome measures: 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.

Tweetable abstract: MFS patients after prophylactic ARR are still at high risk of type B aortic dissection during the peripartum period.

Keywords: Aortic dissection; Marfan syndrome; aortic root replacement; pregnancy.

MeSH terms

Adult
Aortic Diseases / diagnosis
Aortic Diseases / etiology
Aortic Diseases / surgery*
Aortic Dissection* / epidemiology
Aortic Dissection* / etiology
Aortic Dissection* / prevention & control
Aortic Dissection* / therapy
Female
Humans
Incidence
Japan / epidemiology
Marfan Syndrome* / complications
Marfan Syndrome* / diagnosis
Marfan Syndrome* / epidemiology
Peripartum Period
Postoperative Complications* / epidemiology
Postoperative Complications* / prevention & control
Postoperative Complications* / therapy
Pregnancy
Pregnancy Complications, Cardiovascular* / epidemiology
Pregnancy Complications, Cardiovascular* / prevention & control
Pregnancy Complications, Cardiovascular* / therapy
Pregnancy Outcome
Pregnancy, High-Risk
Retrospective Studies
Risk Adjustment / methods
Vascular Surgical Procedures / adverse effects*
Vascular Surgical Procedures / methods