title: Pregnancy after aortic root replacement in Loeys-Dietz syndrome: High risk of aortic dissection.
abstract: Loeys-Dietz syndrome due to mutations in TGFBR1 and 2 is associated with early and aggressive aortic aneurysm and branch vessel disease. There are reports of uncomplicated pregnancy in this condition, but there is an increased risk of aortic dissection and uterine rupture. Women with underlying aortic root aneurysm are cautioned about the risk of pregnancy-related aortic dissection. Prophylactic aortic root replacement is recommended in women with aortopathy and aortic root dilatation to lessen the risk of pregnancy. There is limited information in the literature about the outcomes of pregnancy after root replacement in Loeys-Dietz syndrome. We present a case series of three women with Loeys-Dietz syndrome who underwent elective aortic root replacement for aneurysm disease and subsequently became pregnant and underwent Cesarean section delivery. Each of these women were treated with beta blockers throughout pregnancy. Surveillance echocardiograms and noncontrast MRA studies during pregnancy remained stable demonstrating no evidence for aortic enlargement. Despite the normal aortic imaging and careful observation, two of the three women suffered acute aortic dissection in the postpartum period. These cases highlight the high risk of pregnancy following aortic root replacement in Loeys-Dietz syndrome. Women with this disorder are recommended to be counseled accordingly. © 2016 Wiley Periodicals, Inc.
authors.affiliation: Cardiovascular Division, Department of Medicine, Washington University of Medicine, Saint Louis, Missouri.
authors.forename: Alan C
authors.initials: AC
authors.lastname: Braverman
authors.affiliation: Division of Cardiothoracic Surgery, Department of Surgery, Washington University of Medicine, Saint Louis, Missouri.
authors.forename: Marc R
authors.initials: MR
authors.lastname: Moon
authors.affiliation: Division of Vascular Surgery, Department of Surgery, Washington University of Medicine, Saint Louis, Missouri.
authors.forename: Patrick
authors.initials: P
authors.lastname: Geraghty
authors.affiliation: Department of Medical Genetics, Washington University School of Medicine, Saint Louis, Missouri.
authors.forename: Marcia
authors.initials: M
authors.lastname: Willing
authors.affiliation: Cardiovascular Division, Department of Medicine, Washington University of Medicine, Saint Louis, Missouri.
authors.forename: Christopher
authors.initials: C
authors.lastname: Bach
authors.affiliation: Missouri Baptist Medical Center, Saint Louis, Missouri.
authors.forename: Nicholas T
authors.initials: NT
authors.lastname: Kouchoukos
mesh_terms: D000328:Adult; D000792:Angiography; D001011:Aorta / Q000473:pathology* / Q000601:surgery; D002585:Cesarean Section; D004452:Echocardiography; D005260:Female; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000175:diagnosis* / Q000601:surgery; D011247:Pregnancy; D011248:Pregnancy Complications; D011256:Pregnancy Outcome; D012306:Risk; D014057:Tomography, X-Ray Computed
keywords: Loeys-Dietz syndrome; aneurysm; aortic dissection; genetics; pregnancy