Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study S Sayama, a * N Takeda, b * T Iriyama, a R Inuzuka, c S Maemura, b D Fujita, b H Yamauchi, d K Nawata, d M Bougaki, e H Hyodo, f R Shitara, a T Nakayama, a A Komatsu, a T Nagamatsu, a Y Osuga, a T Fujii a a Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan b Department of Cardiovascular Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan c Department of Pediatrics, Faculty of Medicine, University of Tokyo, Tokyo, Japan d Department of Cardiovascular Surgery, Faculty of Medicine, University of Tokyo, Tokyo, Japan e Department of Anesthesiology and Pain Relief Center, Faculty of Medicine, University of Tokyo, Tokyo, Japan f Department of Obstetrics and Gynecology, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan Correspondence: T Iriyama, Department of Obstetrics and Gynecology, Faculty of Medicine, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan. Email iriyama-tky@umin.ac.jp Accepted 6 March 2017. Published Online 28 April 2017. 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. Keywords Aortic dissection, aortic root replacement, Marfan syndrome, pregnancy. Tweetable abstract MFS patients after prophylactic ARR are still at high risk of type B aortic dissection during the peripartum period. Linked article This article is commented on by MR Johnson and JW Roos Hesselink, p. 494 in this issue. To view this mini commentary visit https://doi.org/10.1111/1471-0528.14781. Please cite this paper as: Sayama S, Takeda N, Iriyama T, Inuzuka R, Maemura S, Fujita D, Yamauchi H, Nawata K, Bougaki M, Hyodo H, Shitara R, Nakayama T, Komatsu A, Nagamatsu T, Osuga Y, Fujii T. Peripartum type B aortic dissection in patients with Marfan syndrome who underwent aortic root replacement: a case series study. BJOG 2018;125:487–493. Introduction Marfan syndrome (MFS) is an autosomal dominant disor- der affecting ocular, cardiovascular, and musculoskeletal systems. 1,2 Mutations in FBN1 (15q21.1) are known to cause MFS. 3 FBN1 encodes fibrillin-1, a major extracellular matrix component of both elastic and non-elastic tissues. 4–6 The major cardiovascular manifestation of MFS is progres- sive dilation of the ascending aorta, and the subsequent Stanford type A aortic dissection (AD), which involves the *These authors contributed equally to this work. 487 ª 2017 Royal College of Obstetricians and Gynaecologists DOI: 10.1111/1471-0528.14635 www.bjog.org Intrapartum care