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

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 (GenTAC) were...

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Veröffentlicht in:Journal of the American Heart Association 2016-08, Vol.5 (8), p.n/a
Hauptverfasser: Roman, Mary J., Pugh, Norma L., Hendershot, Tabitha P., Devereux, Richard B., Dietz, Hal, Holmes, Kathryn, Eagle, Kim A., LeMaire, Scott A., Milewicz, Dianna M., Morris, Shaine A., Pyeritz, Reed E., Ravekes, William J., Shohet, Ralph V., Silberbach, Michael, 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.
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Sprache:eng
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Zusammenfassung: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 (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
ISSN:2047-9980
2047-9980
DOI:10.1161/JAHA.116.004052