Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS)
File(s)
Published version
Author(s)
Type
Journal Article
Abstract
Aortopathy including Marfan (MFS) and Loeys-Dietz syndrome (LDS) poses a high risk of aortic dissection, particularly during pregnancy and the puerperium. Current preventive measures of aortic root dilatation include medical therapy and prophylactic aortic root replacement. The Personalised External Aortic Root Support (PEARS) operation has been developed as an alternative surgical strategy to prevent aortic root dilatation and is now an established procedure with a good prognosis. However, outcomes in pregnant women are unknown. We present case series of nine successful pregnancies in seven women with aortopathy (6 MFS and 1 LDS) who underwent PEARS procedure prior to conception. At a mean follow-up of 4.3 years after delivery, there was no type A or B aortic dissections. Aortic dimensions remained stable, and no hypertensive disorders were observed. Although this is a small retrospective study, PEARS procedure may be a viable pre-conception surgical strategy for women with aortopathy, as an alternative to conventional aortic root surgery. Further studies are needed to conclude that PEARS could be a non-inferior or superior alternative to conventional aortic root surgery in these patients.
Date Issued
2025-07-04
Date Acceptance
2025-06-17
Citation
Nature Communications, 2025, 16 (1)
ISSN
2041-1723
Publisher
Nature Portfolio
Journal / Book Title
Nature Communications
Volume
16
Issue
1
Copyright Statement
© The Author(s) 2025. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.
License URL
Identifier
10.1038/s41467-025-61241-y
Publication Status
Published
Article Number
6165
Date Publish Online
2025-07-04