OBJECTIVES: Evaluate the risk of aortic complications (aortic dissection or prophylactic aortic surgical replacement) associated with pregnancy in Marfan syndrome in the absence of specific care. BACKGROUND: Data are scarce on aortic complications during pregnancy in Marfan syndrome. METHODS: Retrospective study on a large population (415 patients) followed up in our multidisciplinary out-patient clinic devoted to Marfan syndrome. Women over 18 years of age were divided into 2 groups: 85 had been pregnant (MFSP+) giving birth to 136 children through 160 pregnancies; 68 had not been pregnant (MFSP-). The occurrence of aortic complication was compared between the 2 groups using a piecewise discrete-time model. RESULTS: In MFSP+, 7 aortic complications occurred during 160 pregnancies (4.4%) and 17 aortic complications occurred during 1870 years of follow-up out of pregnancy. In MFSP-, 14 aortic complications occurred over 940 years of follow-up. Pregnancy was associated with a 5 fold increase in risk of aortic complication, which did not translate into increased risk during life, suggesting that pregnancy may act as a revealer in women prone to aortic complication. CONCLUSIONS: Pregnancy is associated with a transient increased risk of aortic complication in the absence of specific care.
OBJECTIVES: Evaluate the risk of aortic complications (aortic dissection or prophylactic aortic surgical replacement) associated with pregnancy in Marfan syndrome in the absence of specific care. BACKGROUND: Data are scarce on aortic complications during pregnancy in Marfan syndrome. METHODS: Retrospective study on a large population (415 patients) followed up in our multidisciplinary out-patient clinic devoted to Marfan syndrome. Women over 18 years of age were divided into 2 groups: 85 had been pregnant (MFSP+) giving birth to 136 children through 160 pregnancies; 68 had not been pregnant (MFSP-). The occurrence of aortic complication was compared between the 2 groups using a piecewise discrete-time model. RESULTS: In MFSP+, 7 aortic complications occurred during 160 pregnancies (4.4%) and 17 aortic complications occurred during 1870 years of follow-up out of pregnancy. In MFSP-, 14 aortic complications occurred over 940 years of follow-up. Pregnancy was associated with a 5 fold increase in risk of aortic complication, which did not translate into increased risk during life, suggesting that pregnancy may act as a revealer in women prone to aortic complication. CONCLUSIONS: Pregnancy is associated with a transient increased risk of aortic complication in the absence of specific care.
Authors: Jennifer Pardo Habashi; Elena Gallo MacFarlane; Rustam Bagirzadeh; Caitlin Bowen; Nicholas Huso; Yichun Chen; Djahida Bedja; Tyler J Creamer; Graham Rykiel; Maurice Manning; David Huso; Harry C Dietz Journal: Sci Transl Med Date: 2019-05-01 Impact factor: 17.956
Authors: Ellen S Regalado; Dong-chuan Guo; Anthony L Estrera; L Maximilian Buja; Dianna M Milewicz Journal: Am J Med Genet A Date: 2013-11-15 Impact factor: 2.802
Authors: Dianna M Milewicz; Alan C Braverman; Julie De Backer; Shaine A Morris; Catherine Boileau; Irene H Maumenee; Guillaume Jondeau; Arturo Evangelista; Reed E Pyeritz Journal: Nat Rev Dis Primers Date: 2021-09-02 Impact factor: 65.038
Authors: 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 Journal: J Am Heart Assoc Date: 2016-08-11 Impact factor: 5.501