title: Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC).
abstract: The risk of aortic complications associated with pregnancy in women with Marfan syndrome (MFS) is not fully understood.
  
  
  
  MFS women participating in the large National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) 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).
  
  
  
  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.
authors: Division of Cardiology, Weill Cornell Medical College, New York, NY mroman@med.cornell.edu.
authors: Mary J
authors: MJ
authors: Roman
authors: Biostatistics and Epidemiology Division, RTI International, Rockville, MD.
authors: Norma L
authors: NL
authors: Pugh
authors: Biostatistics and Epidemiology Division, RTI International, Rockville, MD.
authors: Tabitha P
authors: TP
authors: Hendershot
authors: Division of Cardiology, Weill Cornell Medical College, New York, NY.
authors: Richard B
authors: RB
authors: Devereux
authors: Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD.
authors: Hal
authors: H
authors: Dietz
authors: Department of Pediatrics, Oregon Health & Sciences University, Portland, OR.
authors: Kathryn
authors: K
authors: Holmes
authors: Division of Cardiology, University of Michigan Health System, Ann Arbor, MI.
authors: Kim A
authors: KA
authors: Eagle
authors: Division of Cardiothoracic Surgery, Baylor College of Medicine, Houston, TX.
authors: Scott A
authors: SA
authors: LeMaire
authors: Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center, Houston, TX.
authors: Dianna M
authors: DM
authors: Milewicz
authors: Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, TX.
authors: Shaine A
authors: SA
authors: Morris
authors: Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
authors: Reed E
authors: RE
authors: Pyeritz
authors: Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD.
authors: William J
authors: WJ
authors: Ravekes
authors: Department of Medicine, John A. Burns School of Medicine, Honolulu, HI.
authors: Ralph V
authors: RV
authors: Shohet
authors: Department of Pediatrics, Oregon Health & Sciences University, Portland, OR.
authors: Michael
authors: M
authors: Silberbach
mesh_terms: D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000209:etiology / Q000235:genetics; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology* / Q000235:genetics; D001018:Aortic Diseases / Q000209:etiology* / Q000235:genetics; D005260:Female; D006801:Humans; D008382:Marfan Syndrome / Q000150:complications*; D049590:Postpartum Period; D011247:Pregnancy; D011248:Pregnancy Complications / Q000453:epidemiology / Q000235:genetics*; D012042:Registries; D012307:Risk Factors; D014481:United States; D055815:Young Adult
keywords: Marfan syndrome; aneurysm; aortic disease; aortic dissection; pregnancy and postpartum