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. 2024 Nov;131(12):1620-1629.
doi: 10.1111/1471-0528.17893. Epub 2024 Jun 26.

Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review

Théo Haem  1 , Betty Benson  2 , Amandine Dernoncourt  3   4 , Jean Gondry  1   4 , Jean Schmidt  3   4 , Arthur Foulon  1   4
Affiliations

Vascular Ehlers-Danlos syndrome and pregnancy: A systematic review

Théo Haem et al. BJOG. 2024 Nov.

Abstract

Background: Vascular Ehlers-Danlos syndrome (vEDS) is a hereditary connective tissue disorder associated with an elevated risk of vascular, uterine and digestive complications. Managing pregnancy in this context can be a challenge.

Objectives: To systematically review the literature data on the complications in pregnancy associated with vEDS.

Search strategy: We searched the Pubmed Medline and Embase databases for articles using the following terms "vascular Ehlers-Danlos syndrome" or "vEDS" AND "pregnancy".

Selection criteria: Women with vEDS.

Data collection and analysis: We searched the PubMed® MEDLINE® database for publications evaluating obstetric outcomes in women with vEDS.

Main results: A total of 121 publications were screened, with six (accounting for 412 pregnancies) included in our review. Of the women included in this sample, 30% were infertile. The miscarriage rate was 13.8% (57/412) and 8.8% of the live births were premature. Obstetric anal sphincter injuries occurred in 11.3% (23/203) of the deliveries. The maternal mortality rate per pregnancy was 5.7%.

Conclusions: Women with vEDS present an elevated risk of uterine rupture, vascular events, digestive events and death during pregnancy. Women appear to be most at risk during the peripartum period; to avoid expulsive efforts, a caesarean section should be scheduled at 37 weeks of gestation.

Keywords: obstetrics; pregnancy; vascular Ehlers‐Danlos syndrome.

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References

REFERENCES

    1. Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J, et al. The 2017 international classification of the Ehlers‐Danlos syndromes. Am J Med Genet C Semin Med Genet. 2017;175:8–26. https://doi.org/10.1002/ajmg.c.31552
    1. Kulas Søborg ML, Leganger J, Quitzau Mortensen L, Rosenberg J, Burcharth J. Establishment and baseline characteristics of a nationwide Danish cohort of patients with Ehlers–Danlos syndrome. Rheumatology. 2017;56:763–767. https://doi.org/10.1093/rheumatology/kew478
    1. Pepin M, Schwarze U, Superti‐Furga A, Byers PH. Clinical and genetic features of Ehlers‐Danlos syndrome type IV, the vascular type. N Engl J Med. 2000;342:673–680. https://doi.org/10.1056/NEJM200003093421001
    1. Frank M, Albuisson J, Ranque B, Golmard L, Mazzella JM, Bal‐Theoleyre L, et al. The type of variants at the COL3A1 gene associates with the phenotype and severity of vascular Ehlers‐Danlos syndrome. Eur J Hum Genet. 2015;23(12):1657–1664. https://doi.org/10.1038/ejhg.2015.32
    1. Murray ML, Pepin M, Peterson S, Byers PH. Pregnancy‐related deaths and complications in women with vascular Ehlers‐Danlos syndrome. Genet Med. 2014;16:874–880. https://doi.org/10.1038/gim.2014.53

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