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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.

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

Renal hypouricemia is associated with urinary calculi and severe acute renal failure after exercise. The epidemiology of renal hypouricemia is not yet sufficiently understood, and there is no report of it occurring during pregnancy. We report the case of a pregnant woman with renal hypouricemia. At her first pregnancy, she developed preeclampsia with severe features at the 34th week of gestation. After parturition, she developed acute renal failure and was diagnosed with renal hypouricemia. During the second pregnancy, when she was referred to our hospital, care was taken to ensure adequate hydration by infusion of liquids and water at the time of labour. Consequently, she did not have onset of renal hypouricemia. We suggest that acute renal failure may be avoided in pregnant women with renal hypouricemia by preventing dehydration via drinking enough during pregnancy postpartum period and by infusion during labour.