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Objective: Loeys-Dietz syndrome (LDS) is associated with a higher risk of aortic dissections (ADs) during pregnancy and postpartum. However, there is limited evidence about the perinatal management of LDS patients who have undergone prophylactic aortic root replacements (ARRs) before conception. Case report: We present the case of a 28-year-old nulliparous pregnant woman with LDS with a pathogenic variant within exon 5 of TGFBR2 (c.1379G > T, p.[Arg460Leu]), who underwent an ARR at 20 years of age. Cardiac echocardiography did not show any significant changes in the aorta during pregnancy, and her blood pressure remained normal. She had a cesarean section at 37 weeks of gestation. She developed an acute Stanford type B AD extending from the aortic arch to the infrarenal aorta 8 days postpartum and underwent a total arch replacement. Conclusion: This case report suggests that patients with LDS after prophylactic ARRs still possess a risk for Stanford type B ADs.

Objectives: Patients with Marfan syndrome (MFS) show a substantially increased risk of pregnancy-related aortic dissection (AD). Prophylactic aortic root replacement (ARR) is recommended for patients with aortic root dilatation to decrease cardiovascular risk during the peripartum period. However, evidence supporting the management of parturients with MFS who have received ARR is limited. This study was aimed 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: The pregnancy outcomes of all patients with MFS managed at our institute between 1982 and September 2016 were retrospectively reviewed based on medical records. 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 (5 pregnancies) had received ARR before conception, of which 3 (60%) developed type B aortic dissection [AD(B)] during the peripartum period, whereas only one of 10 patients (12 pregnancies) without ARR (8.3% of pregnancies) developed AD(B) in the peripartum period (p < 0.05, chi-square test). Conclusions: Our study results suggested that MFS patients after prophylactic ARR are still at high risk of AD(B) during the peripartum period. Careful pre-pregnancy counseling and multidisciplinary care throughout the peripartum period are essential for the management of MFS, even after surgical repair of ascending aortic aneurysm. Key Words: Marfan syndrome, pregnancy, aortic dissection, aortic root replacement. [ABSTRACT FROM AUTHOR]