Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC)

Author:

Roman Mary J.1,Pugh Norma L.2,Hendershot Tabitha P.2,Devereux Richard B.1,Dietz Hal3,Holmes Kathryn4,Eagle Kim A.5,LeMaire Scott A.6,Milewicz Dianna M.7,Morris Shaine A.8,Pyeritz Reed E.9,Ravekes William J.3,Shohet Ralph V.10,Silberbach Michael4,Dietz Harry C.,Habashi Jennifer,Prakash Siddharth K.,Maslen Cheryl L.,Song Howard K.,Bavaria Joseph E.,Milewski Karianna,Weinsaft Jonathan W.,McDonnell Nazli,Asch Federico M.,Tolunay H. Eser,Desvigne‐Nickens Patrice,Tseng Hung,Kroner Barbara L.,

Affiliation:

1. Division of Cardiology, Weill Cornell Medical College, New York, NY

2. Biostatistics and Epidemiology Division, RTI International, Rockville, MD

3. Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD

4. Department of Pediatrics, Oregon Health & Sciences University, Portland, OR

5. Division of Cardiology, University of Michigan Health System, Ann Arbor, MI

6. Division of Cardiothoracic Surgery, Baylor College of Medicine, Houston, TX

7. Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center, Houston, TX

8. Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, TX

9. Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA

10. Department of Medicine, John A. Burns School of Medicine, Honolulu, HI

Abstract

Background The risk of aortic complications associated with pregnancy in women with Marfan syndrome ( MFS ) is not fully understood. Methods and Results MFS women participating in the large National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (Gen TAC ) were evaluated. Among 184 women with MFS in whom pregnancy information was available, 94 (51%) had a total of 227 pregnancies. Among the women with pregnancies, 10 (10.6%) experienced a pregnancy‐related aortic complication (4 type A and 3 type B dissections, 1 coronary artery dissection, and 2 with significant [≥3 mm] aortic growth). Five of 7 aortic dissections, including all 3 type B, and the coronary dissection (75% of all dissections) occurred in the postpartum period. Only 5 of 8 women with pregnancy‐associated dissection were aware of their MFS diagnosis. The rate of aortic dissection was higher during the pregnancy and postpartum period (5.4 per 100 person‐years vs 0.6 per 100 person‐years of nonpregnancy; rate ratio, 8.4 [95% CI=3.9, 18.4]; P <0.0001). Conclusions Pregnancy in MFS is associated with an increased risk of aortic dissection, both types A and B, particularly in the immediate postpartum period. Lack of knowledge of underlying MFS diagnosis before aortic dissection is a major contributing factor. These findings underscore the need for early diagnosis, prepregnancy risk counseling, and multidisciplinary peripartum management.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Cardiology and Cardiovascular Medicine

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