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Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys–Dietz syndrome

Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys–Dietz syndrome
Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys–Dietz syndrome

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.

Tweetable abstract: Pregnancy outcomes in women with Loeys–Dietz syndrome.

Congenital heart disease, pregnancy
1470-0328
Cauldwell, M.
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Steer, P.J.
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Curtis, S.
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Mohan, A.R.
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Dockree, S.
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Mackillop, L.
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Parry, H.
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Oliver, J.
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Sterrenburg, M.
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Bolger, A.
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Siddiqui, F.
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Simpson, M.
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Walker, N.
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Bredaki, F.
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Walker, F.
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Johnson, M.R.
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Cauldwell, M.
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Steer, P.J.
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Curtis, S.
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Mohan, A.R.
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Dockree, S.
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Mackillop, L.
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Parry, H.
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Oliver, J.
269a97b8-9e3b-4c26-87b9-169bd3cb63fc
Sterrenburg, M.
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Bolger, A.
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Siddiqui, F.
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Simpson, M.
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Walker, N.
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Bredaki, F.
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Walker, F.
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Johnson, M.R.
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Cauldwell, M., Steer, P.J., Curtis, S., Mohan, A.R., Dockree, S., Mackillop, L., Parry, H., Oliver, J., Sterrenburg, M., Bolger, A., Siddiqui, F., Simpson, M., Walker, N., Bredaki, F., Walker, F. and Johnson, M.R. (2019) Maternal and fetal outcomes in pregnancies complicated by the inherited aortopathy Loeys–Dietz syndrome. BJOG: An International Journal of Obstetrics and Gynaecology. (doi:10.1111/1471-0528.15670).

Record type: 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.

Tweetable abstract: Pregnancy outcomes in women with Loeys–Dietz syndrome.

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More information

Accepted/In Press date: 1 January 2019
e-pub ahead of print date: 20 February 2019
Keywords: Congenital heart disease, pregnancy

Identifiers

Local EPrints ID: 431203
URI: http://eprints.soton.ac.uk/id/eprint/431203
ISSN: 1470-0328
PURE UUID: be80d57d-ee85-40b8-a0e4-dc6ffe8c4347
ORCID for M. Sterrenburg: ORCID iD orcid.org/0000-0001-7283-7764

Catalogue record

Date deposited: 24 May 2019 16:30
Last modified: 16 Mar 2024 01:25

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Contributors

Author: M. Cauldwell
Author: P.J. Steer
Author: S. Curtis
Author: A.R. Mohan
Author: S. Dockree
Author: L. Mackillop
Author: H. Parry
Author: J. Oliver
Author: M. Sterrenburg ORCID iD
Author: A. Bolger
Author: F. Siddiqui
Author: M. Simpson
Author: N. Walker
Author: F. Bredaki
Author: F. Walker
Author: M.R. Johnson

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