Research Information System
International Journal of Cardiology Congenital Heart Disease, vol.24, 2026 (ESCI, Scopus)
Background Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder associated with high pregnancy-related risks. Current guidelines consider vEDS an mWHO IV high-risk pregnancy, but data are limited and often retrospective. Methods This prospective case series reports pregnancy outcomes and aortic measurements in six women with confirmed or suspected vEDS enrolled in the ROPAC III registry. Results No maternal deaths or aortic dissections occurred. One fetal death was reported. Obstetric complications were common, including preterm birth and hemorrhage. Most women delivered via cesarean section. No significant aortic growth was observed during pregnancy. Conclusions In women diagnosed with vEDS prior to pregnancy, the absence of life-threatening vascular events suggests pregnancy may be safer than previously reported. These findings support more individualized counseling and underscore the need for further prospective studies.