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Multicenter Study
. 2025 Nov 4;11(7):1091-1107.
doi: 10.1093/ehjqcco/qcaf038.

Pregnancy outcomes in women with heritable thoracic aortic disease: data from the EORP ESC registry of pregnancy and cardiac disease (ROPAC) III

Puck N J Peters  1 , Johanna A van der Zande  1   2 , Julie De Backer  3   4 , Guillaume Jondeau  4   5 , Osama Ahmad  6 , Marjorie Richardson  7 , Francesca M Comoglio  8 , Heleen van der Zwaan  9 , Siddharth K Prakash  10 , Christina Christersson  11 , Karishma P Ramlakhan  1   2 , Roger Hall  12 , Mark R Johnson  13 , Jolien W Roos-Hesselink  1   4 ; ROPAC investigators
Collaborators, Affiliations
Multicenter Study

Pregnancy outcomes in women with heritable thoracic aortic disease: data from the EORP ESC registry of pregnancy and cardiac disease (ROPAC) III

Puck N J Peters et al. Eur Heart J Qual Care Clin Outcomes. .

Abstract

Aims: The risk of pregnancy in women with heritable thoracic aortic disease (HTAD) is estimated to be high, but supporting data are scarce. The aim of this study is to prospectively investigate pregnancy outcomes to improve patient management and care.

Methods and results: The Registry of Pregnancy and Cardiac disease (ROPAC) III is a prospective global registry including pregnant women with known aortic pathology between 2018 and 2023. Cardiac, obstetric and fetal outcomes, beta-blocker use, and the impact of breastfeeding were investigated. Additionally, changes in aortic diameters were assessed. In total, 176 pregnancies in 170 women (mean age 32 years, 56% primigravida) with HTAD were included: 122 with Marfan syndrome, 14 with Loeys-Dietz syndrome, 10 with ACTA2 variants, and 30 with other diagnoses. There was no maternal or neonatal mortality, while six (3.4%) fetal deaths occurred. Thirteen (7.6%) women suffered a major adverse cardiac event (MACE), including six (3.5%) aortic dissections (three during and three after pregnancy). Beta-blockers were used throughout pregnancy by 83 (47%) women. Women taking beta-blockers did not experience less MACE, aortic dissection, or aortic growth. Breastfeeding women had a significantly lower occurrence of MACE compared with non-breastfeeding women. The aortic diameter showed significant growth during pregnancy.

Conclusion: The aortic dissection rate in this cohort of women with HTAD diagnosis prior to pregnancy, under surveillance in specialized clinics, was lower than previously reported. Our results suggest that pregnancy might have some effect on aortic growth and dissections did occur. This warrants close monitoring, also after delivery. Importantly, we found no association between breastfeeding and post-partum complications.

Keywords: ACTA2; Aortic dissection; Heritable thoracic aortic disease; Loeys-Dietz; Marfan; Pregnancy.

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Conflict of interest statement

Conflict of interest: J.W.R.-H. declares financial support for salary of PhD candidate from Zabawas Foundation and De Hoop Foundation; Leadership role as chairperson for the ROPAC study; Managing Director Dutch Cardiovascular Alliance. R.H. declares being the Chair of EASY-AS DSMB. J.D.B. declares being a co-chair of ESC 2025 pregnancy guidelines (no payment received); Vascern Transversal pregnancy group (no payment received). Guillaume Jondeau declares grants of contracts from the Federation Francaise de Cardiologie (his institution); Leadership or fiduciary role for the Coordination of VASCERN. P.N.J.P. declares receiving Support for attending meetings and/or travel from Erasmus MC, Department of Cardiology. S.K.P. was Scientific Advisory Board (unpaid) for the Turner Syndrome Society of the United States; participation in Collaborative Clinical Science Working Group (unpaid) for the GenTAC Alliance, Marfan Foundation. All other authors have no conflict of interest to declare.

Figures

Graphical Abstract
Graphical Abstract
Figure 1
Figure 1
Imaging data on the aortic diameter before, during, and after pregnancy stratified per segment. Statistical analysis has been conducted using a mixed-models approach to analyse the trend overtime for the whole cohort.
Figure 2
Figure 2
Imaging data on the ascending aorta diameter before, during, and after pregnancy stratified per diagnostic group. Statistical analysis has been conducted using a mixed-models approach to analyse the trend overtime for each diagnostic group. PV, pathogenic variant.
Figure 3
Figure 3
Imaging data on the sinus of Valsalva diameter before, during and after pregnancy stratified per diagnostic group. Statistical analysis has been conducted using a mixed-models approach to analyze the trend overtime for each diagnostic group. PV, pathogenic variant.
See this image and copyright information in PMC

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