Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys-Dietz syndrome
File(s)
Accepted version
Author(s)
Type
Journal Article
Abstract
Objective
Pregnancies in women with Loeys–Dietz syndrome (LDS) are rare and are typically documented in case reports only. Early reports suggested high rates of maternal complications during pregnancy and the puerperium, including aortic dissection and uterine rupture, but information on fetal outcomes was very limited.
Design
A retrospective cohort study.
Setting
Eight specialist UK centres.
Sample
Pregnant women with LDS.
Methods
Data was collated on cardiac, obstetric, and neonatal outcomes.
Main outcome measures
Maternal and perinatal outcomes in pregnancies complicated by LDS.
Results
Twenty pregnancies in 13 women with LDS were identified. There was one miscarriage, one termination of pregnancy, and 18 livebirths. In eight women the diagnosis was known prior to pregnancy but only one woman had preconception counselling. In four women the diagnosis was made during pregnancy through positive genotyping, and the other was diagnosed following delivery. Five women had a family history of aortic dissection. There were no aortic dissections in our cohort during pregnancy or postpartum. Obstetric complications were common, including postpartum haemorrhage (33%) and preterm delivery (50%). In all, 14/18 (78%) of deliveries were by elective caesarean section, at a median gestational age at delivery of 37 weeks. Over half the infants (56%) were admitted to the neonatal unit following delivery.
Conclusion
Women with LDS require multidisciplinary specialist management throughout pregnancy. Women should be referred for preconception counselling to make informed decisions around pregnancy risk and outcomes. Early elective preterm delivery needs to be balanced against a high infant admission rate to the neonatal unit.
Pregnancies in women with Loeys–Dietz syndrome (LDS) are rare and are typically documented in case reports only. Early reports suggested high rates of maternal complications during pregnancy and the puerperium, including aortic dissection and uterine rupture, but information on fetal outcomes was very limited.
Design
A retrospective cohort study.
Setting
Eight specialist UK centres.
Sample
Pregnant women with LDS.
Methods
Data was collated on cardiac, obstetric, and neonatal outcomes.
Main outcome measures
Maternal and perinatal outcomes in pregnancies complicated by LDS.
Results
Twenty pregnancies in 13 women with LDS were identified. There was one miscarriage, one termination of pregnancy, and 18 livebirths. In eight women the diagnosis was known prior to pregnancy but only one woman had preconception counselling. In four women the diagnosis was made during pregnancy through positive genotyping, and the other was diagnosed following delivery. Five women had a family history of aortic dissection. There were no aortic dissections in our cohort during pregnancy or postpartum. Obstetric complications were common, including postpartum haemorrhage (33%) and preterm delivery (50%). In all, 14/18 (78%) of deliveries were by elective caesarean section, at a median gestational age at delivery of 37 weeks. Over half the infants (56%) were admitted to the neonatal unit following delivery.
Conclusion
Women with LDS require multidisciplinary specialist management throughout pregnancy. Women should be referred for preconception counselling to make informed decisions around pregnancy risk and outcomes. Early elective preterm delivery needs to be balanced against a high infant admission rate to the neonatal unit.
Date Issued
2019-02-27
Date Acceptance
2019-02-27
Citation
BJOG: An International Journal of Obstetrics and Gynaecology, 2019, 126 (8), pp.1025-1031
ISSN
1470-0328
Publisher
Wiley
Start Page
1025
End Page
1031
Journal / Book Title
BJOG: An International Journal of Obstetrics and Gynaecology
Volume
126
Issue
8
Copyright Statement
© 2019 Royal College of Obstetricians and Gynaecologists. This is the peer reviewed version of the following article: Cauldwell, M, Steer, PJ, Curtis, S, Mohan, AR, Dockree, S, Mackillop, L, Parry, H, Oliver, J, Sterrenburg, M, Bolger, A, Siddiqui, F, Simpson, M, Walker, N, Bredaki, F, Walker, F, Johnson, MR. Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys–Dietz syndrome. BJOG 2019; 126: 1025– 1031, which has been published in final form at https://doi.org/10.1111/1471-0528.15670. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions.
Identifier
http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000470936600019&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=1ba7043ffcc86c417c072aa74d649202
Subjects
Science & Technology
Life Sciences & Biomedicine
Obstetrics & Gynecology
Congenital heart disease
pregnancy
HEART-DISEASE
MARFAN-SYNDROME
MUTATIONS
TGFBR1
MANAGEMENT
ANEURYSMS
WOMEN
Publication Status
Published
Date Publish Online
2019-06-05