Open AccessAortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (Gen TAC )
Author(s) -
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
Publication year - 2016
Publication title -
journal of the american heart association
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.494
H-Index - 85
ISSN - 2047-9980
DOI - 10.1161/jaha.116.004052
Subject(s) - medicine , pregnancy , marfan syndrome , aortic dissection , postpartum period , dissection (medical) , cardiology , aortic aneurysm , risk factor , aneurysm , obstetrics , surgery , aorta , genetics , biology
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.