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Original Investigation
October 14, 2020

Clinical Features and Outcomes of Pregnancy-Related Acute Aortic Dissection

Author Affiliations
  • 1Cardiovascular Division, Department of Medicine, Washington University School of Medicine in St Louis, St Louis, Missouri
  • 2Medical student, Washington University School of Medicine in St Louis, St Louis, Missouri
  • 3Minneapolis Heart Institute at Abbott-Northwestern Hospital, Minneapolis, Minnesota
  • 4University Hospital Vall d'Hebron, Barcelona, Spain
  • 5Division of Translational Medicine and Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia
  • 6Donald and Barbara Zucker School of Medicine at Hofstra/Northwell Health, New York, New York
  • 7Department of Anesthesiology, University of Virginia Medical School, Charlottesville
  • 8Department of Cardiovascular Sciences, University of Leicester, Leicester, United Kingdom
  • 9Cardiovascular Division, Department of Medicine, University of Minnesota School of Medicine, Minneapolis
  • 10Department of Surgery University of Toronto, Toronto General Hospital, Toronto, Ontario, Canada
  • 11Division of Cardiothoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, Georgia
  • 12Department of Clinical Medicine, Tromsø University Hospital, Tromsø, Norway
  • 13University Hospital of Heidelberg, Heidelberg, Germany
  • 14Libin Cardiovascular Institute, University of Calgary, Calgary, Alberta, Canada
  • 15Division of Cardiothoracic Surgery, Department of Baylor College of Medicine, Houston, Texas
  • 16Department of Cardiology, Hadassah University Medical Center, Jerusalem, Israel
  • 17Cardiovascular Division, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
  • 18Deputy Editor, JAMA Cardiology
  • 19Cardiovascular Division, Department of Medicine, University of Michigan School of Medicine, Ann Arbor
  • 20Cardiovascular Division, Department of Medicine, Massachusetts General Hospital, Harvard Medical School, Boston
JAMA Cardiol. Published online October 14, 2020. doi:10.1001/jamacardio.2020.4876
Key Points

Question  What are the clinical features in women who experience aortic dissection related to pregnancy?

Findings  In this cohort study, 29 women, representing 1% of women in the International Registry of Acute Aortic Dissection, experienced aortic dissection related to pregnancy. Most women had an underlying aortopathy condition, but many were previously unaware of this diagnosis.

Meaning  Aortic dissection is a rare complication of pregnancy that occurs in women with aortopathy conditions; the findings of this study suggest that these conditions are often not recognized until after the aortic dissection occurs.

Abstract

Importance  Women with aortopathy conditions are at risk for pregnancy-related aortic dissection, and these conditions may not be recognized until after the aortic dissection occurs.

Objective  To examine the clinical characteristics, imaging features, and outcomes in women with pregnancy-related acute aortic dissection.

Design, Setting, and Participants  A cohort study, comprising data from the International Registry of Acute Aortic Dissection (IRAD) (February 1, 1998, to February 28, 2018). The multicenter referral center study included 29 women with aortic dissection during pregnancy or less than 12 weeks post partum in IRAD from 1998 to 2018.

Main Outcomes and Measures  Clinical features of pregnancy-related aortic dissection to be studied included underlying aortopathy, aortic size, type of aortic dissection, timing of dissection, hypertension, and previous aortic surgery.

Results  A total of 29 women (mean [SD] age, 32 [6] years) had pregnancy-related aortic dissection, representing 0.3% of all aortic dissections and 1% of aortic dissection in women in the IRAD. Among women younger than 35 years, aortic dissection was related to pregnancy in 20 of 105 women (19%). Thirteen women (45%) had type A aortic dissection, and 16 women (55%) had type B. Aortic dissection onset was known in 27 women (93%): 15 during pregnancy, 4 in the first trimester, and 11 in the third trimester; 12 were post partum, occurring a mean (SD) of 12.5 (14) days post partum. At type A aortic dissection diagnosis, the mean (SD) aortic diameters were sinus of Valsalva, 54.5 (5) mm and ascending aorta, 54.7 (6) mm. At type B aortic dissection diagnosis, the mean (SD) descending aortic diameter was 32.5 (5) mm. Twenty women (69%) had an aortopathy condition or a positive family history: 13 women (65%) with Marfan syndrome, 2 women (10%) with Loeys-Dietz syndrome, 2 women (10%) with bicuspid aortic valves, 2 women (10%) with a family history of aortic disease, and 1 woman (5%) with familial thoracic aortic aneurysm. Aortopathy was not recognized until after aortic dissection in 47% of the women. Twenty-eight women (97%) survived aortic dissection hospitalization.

Conclusions and Relevance  Aortic dissection complicating pregnancy is rare. Most pregnancy-related aortic dissection is due to an aortopathy often not diagnosed until after aortic dissection. In this study, type A aortic dissections were associated with a dilated aorta, and type B aortic dissections often were not. Recognition of underlying conditions and risks for aortic dissection may improve management of pregnancy in women with aortopathy.

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