• Melissa L Russo, Neelima Sukhavasi, Veena Mathur, and Shaine A Morris.
• Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Warren Alpert Medical School of Brown University, Women and Infants Hospital of Rhode Island, Providence, Rhode Island; and the Department of Obstetrics and Gynecology, Baylor College of Medicine, and the Departments of Molecular and Human Genetics and Pediatric Cardiology, Baylor College of Medicine, Texas Children's Pavilion for Women, Houston, Texas.
• Obstet Gynecol. 2018 Jun 1; 131 (6): 1080-1084.

BackgroundLoeys-Dietz syndrome is associated with arterial tortuosity and aortic dissection. Pregnancy may be a period of increased risk for aortic dissection.CaseA 16-year-old primigravid girl was referred to our center with a family history of aortic dissection. Loeys-Dietz syndrome was suspected, and genetic testing confirmed the TGFβ2 (c.988C>T) mutation. A cesarean delivery was performed at 36 weeks of gestation, with no cardiovascular complications. In this case, the uterine vessels were significantly tortuous; this may be an additional finding in Loeys-Dietz syndrome.ConclusionWomen with Loeys-Dietz syndrome warrant special consideration in obstetric management secondary to the risk for aortic dissection. It is recommended that a multidisciplinary team with knowledge about connective tissue disorders and expertise in aortic surgery coordinate maternal obstetric and cardiovascular care.

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