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

Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease

Kim A. Eagle¹  Norma L. Pugh²  Tabitha P. Hendershot²  Shaine A. Morris³  Michael Silberbach⁴  William J. Ravekes⁵  Hal Dietz⁶  Ralph V. Shohet⁶  Kathryn Holmes⁷  Scott A. LeMaire⁸  Richard B. Devereux⁹  Mary J. Roman⁹  Dianna M. Milewicz^1,10  Reed E. Pyeritz^1,11 

[1] Sciences University, Portland, OR;Biostatistics and Epidemiology Division, RTI International, Rockville, MD;Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center, Houston, TX;Department of Medicine, John A. Burns School of Medicine, Honolulu, HI;Department of Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA;Department of Pediatrics, Johns Hopkins University School of Medicine, Baltimore, MD;;Department of Pediatrics, Oregon Health &Division of Cardiology, University of Michigan Health System, Ann Arbor, MI;Division of Cardiology, Weill Cornell Medical College, New York, NY;Division of Cardiothoracic Surgery, Baylor College of Medicine, Houston, TX;Division of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine, Houston, TX;

关键词: aneurysm;    aortic disease;    aortic dissection;    Marfan syndrome;    pregnancy and postpartum;   

DOI  :  10.1161/JAHA.116.004052

来源: DOAJ

【 摘 要 】

BackgroundThe risk of aortic complications associated with pregnancy in women with Marfan syndrome (MFS) is not fully understood. Methods and ResultsMFS 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). ConclusionsPregnancy 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.

【 授权许可】

Unknown