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The aim of this study is to prospectively investigate pregnancy outcomes to improve patient management and care.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods and results<\/jats:title>\n                    <jats:p>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\u2013Dietz 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>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. 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