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

Mary J Roman, Norma L Pugh, Tabitha P Hendershot, Richard B Devereux, Hal Dietz, Kathryn Holmes, Kim A Eagle, Scott A LeMaire, Dianna M Milewicz, Shaine A Morris, Reed E Pyeritz, William J Ravekes, Ralph V Shohet, Michael Silberbach, GenTAC InvestigatorsDietzHarry C.HabashiJenniferPrakashSiddharth K.MaslenCheryl L.SongHoward K.BavariaJoseph E.MilewskiKariannaWeinsaftJonathan W.McDonnellNazliAschFederico M.TolunayH. EserDesvigne‐NickensPatriceTsengHungKronerBarbara L, Harry C Dietz, Jennifer Habashi, Siddharth K Prakash, Cheryl L Maslen, Howard K Song, Joseph E Bavaria, Karianna Milewski, Jonathan W Weinsaft, Nazli McDonnell, Federico M Asch, H Eser Tolunay, Patrice Desvigne-Nickens, Hung Tseng, Barbara L Kroner, Mary J Roman, Norma L Pugh, Tabitha P Hendershot, Richard B Devereux, Hal Dietz, Kathryn Holmes, Kim A Eagle, Scott A LeMaire, Dianna M Milewicz, Shaine A Morris, Reed E Pyeritz, William J Ravekes, Ralph V Shohet, Michael Silberbach, GenTAC InvestigatorsDietzHarry C.HabashiJenniferPrakashSiddharth K.MaslenCheryl L.SongHoward K.BavariaJoseph E.MilewskiKariannaWeinsaftJonathan W.McDonnellNazliAschFederico M.TolunayH. EserDesvigne‐NickensPatriceTsengHungKronerBarbara L, Harry C Dietz, Jennifer Habashi, Siddharth K Prakash, Cheryl L Maslen, Howard K Song, Joseph E Bavaria, Karianna Milewski, Jonathan W Weinsaft, Nazli McDonnell, Federico M Asch, H Eser Tolunay, Patrice Desvigne-Nickens, Hung Tseng, Barbara L Kroner

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 (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).

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.

Keywords: Marfan syndrome; aneurysm; aortic disease; aortic dissection; pregnancy and postpartum.

© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

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