ABSTRACT
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.
Subject(s)
Aorta, Thoracic/transplantation , Aortic Aneurysm, Thoracic/surgery , Aortic Dissection/surgery , Loeys-Dietz Syndrome/surgery , Puerperal Disorders/surgery , Adult , Aortic Dissection/congenital , Aortic Dissection/prevention & control , Aortic Aneurysm, Thoracic/etiology , Aortic Aneurysm, Thoracic/prevention & control , Female , Humans , Loeys-Dietz Syndrome/complications , Medical Illustration , Preconception Care/methods , Pregnancy , Prophylactic Surgical Procedures/methods , Puerperal Disorders/etiology , Puerperal Disorders/prevention & controlABSTRACT
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.
