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Obstetric Management of Loeys-Dietz Syndrome

Russo, Melissa, L., MD; Sukhavasi, Neelima, MD; Mathur, Veena, MS, CGC; Morris, Shaine, A., MD, MPH

doi: 10.1097/AOG.0000000000002615
Contents: Maternal-Fetal Medicine: Case Report

BACKGROUND: Loeys-Dietz syndrome is associated with arterial tortuosity and aortic dissection. Pregnancy may be a period of increased risk for aortic dissection.

CASE: A 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.

CONCLUSION: Women 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.

Among pregnant women with Loeys-Dietz syndrome, anticipation of and preparation for potential aortic dissection are paramount.

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.

Corresponding author: Melissa L. Russo, MD, Division of Maternal-Fetal Medicine, Department of Obstetrics and Gynecology, Warren Alpert Medical School of Brown University, Women and Infants Hospital of Rhode Island, 101 Dudley Street, Providence, RI 02905; email: mlrusso@wihri.org.

Supported by the National Institutes of Health K23HL127266 award.

Financial Disclosure The authors did not report any potential conflicts of interest.

Presented at the 5th Annual Resident Research Day, June 7, 2017, Baylor College of Medicine, Houston, Texas.

Each author has indicated that she has met the journal's requirements for authorship.

© 2018 by The American College of Obstetricians and Gynecologists. Published by Wolters Kluwer Health, Inc. All rights reserved.