AUTHOR=Nunez-Ordonez Nicolas , Amado-Olivares Andrés F. , Jimenez-Ordonez Andrés F. , Obando Carlos , Chalela Tomas , Senosiain Julián , Sandoval Nestor , Camacho-Mackenzie Jaime , Villa-Hincapié Carlos TITLE=Case Report: A usual procedure in an unusual situation: a patient with a rare Ehlers Danlos/osteogenesis imperfecta overlap undergoing aortic valve replacement JOURNAL=Frontiers in Cardiovascular Medicine VOLUME=Volume 12 - 2025 YEAR=2025 URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2025.1480363 DOI=10.3389/fcvm.2025.1480363 ISSN=2297-055X ABSTRACT=Connective tissue disorders are known to cause cardiac and vascular complications. We present the case of a 37-year-old female patient with a rare Ehlers Danlos/Osteogenesis Imperfecta Overlap Syndrome, referred to cardiac surgery with aortic valve regurgitation, who underwent a successful Biological Surgical Aortic Valve Replacement (SAVR). A multidisciplinary, patient-centered, heart-team approach is essential in managing patients with rare genetic disorders to optimize postoperative outcomes. Adult cardiac surgeons must become familiar with genetic syndromes and their implications for improving perioperative outcomes.