Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS)
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...
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Nature Portfolio
2025-07-01
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| Series: | Nature Communications |
| Online Access: | https://doi.org/10.1038/s41467-025-61241-y |
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| author | Claudia Montanaro Polona Kacar Giulia Iannaccone John Pepper Gurleen Wander Christoph A. Nienaber Andreas Hoschtitzky Mario Petrou Hannah Douglas Mark R. Johnson Roshni R. Patel Isma Rafiq Michael A. Gatzoulis |
| author_facet | Claudia Montanaro Polona Kacar Giulia Iannaccone John Pepper Gurleen Wander Christoph A. Nienaber Andreas Hoschtitzky Mario Petrou Hannah Douglas Mark R. Johnson Roshni R. Patel Isma Rafiq Michael A. Gatzoulis |
| author_sort | Claudia Montanaro |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-ad4097f6af0f429b8e5dbfb473796bcf |
| institution | DOAJ |
| issn | 2041-1723 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | Nature Portfolio |
| record_format | Article |
| series | Nature Communications |
| spelling | doaj-art-ad4097f6af0f429b8e5dbfb473796bcf2025-08-20T03:03:37ZengNature PortfolioNature Communications2041-17232025-07-011611510.1038/s41467-025-61241-yConnective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS)Claudia Montanaro0Polona Kacar1Giulia Iannaccone2John Pepper3Gurleen Wander4Christoph A. Nienaber5Andreas Hoschtitzky6Mario Petrou7Hannah Douglas8Mark R. Johnson9Roshni R. Patel10Isma Rafiq11Michael A. Gatzoulis12Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’s NHS TrustAdult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’s NHS TrustAdult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’s NHS TrustNational Heart and Lung Institute, Imperial CollegeDepartment of Obstetrics, Chelsea and Westminster HospitalNational Heart and Lung Institute, Imperial CollegeNational Heart and Lung Institute, Imperial CollegeNational Heart and Lung Institute, Imperial CollegeDepartment of Cardiology, St Thomas’ HospitalNational Heart and Lung Institute, Imperial CollegeDepartment of Obstetrics, Chelsea and Westminster HospitalAdult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’s NHS TrustAdult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’s NHS TrustAbstract 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.https://doi.org/10.1038/s41467-025-61241-y |
| spellingShingle | Claudia Montanaro Polona Kacar Giulia Iannaccone John Pepper Gurleen Wander Christoph A. Nienaber Andreas Hoschtitzky Mario Petrou Hannah Douglas Mark R. Johnson Roshni R. Patel Isma Rafiq Michael A. Gatzoulis Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS) Nature Communications |
| title | Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS) |
| title_full | Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS) |
| title_fullStr | Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS) |
| title_full_unstemmed | Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS) |
| title_short | Connective tissue disorder and high risk pregnancy: a case series with personalised external aortic root support (PEARS) |
| title_sort | connective tissue disorder and high risk pregnancy a case series with personalised external aortic root support pears |
| url | https://doi.org/10.1038/s41467-025-61241-y |
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