Vascular Ehlers-Danlos syndrome with ruptured thoracic aortic aneurysm complicated by vascular graft and endograft infection and aortobronchial fistula

The combination of vascular graft or endograft infection and aortic hereditary disease is extremely rare and just as challenging. We report the case of a 40-year-old man presenting with a ruptured saccular aortic isthmus aneurysm treated with thoracic endovascular aortic repair, complicated by vascular graft or endograft infection with an aortobronchial fistula and multiple pseudoaneurysms. Despite complete graft explantation, prolonged antibiotic therapy, and additional endovascular and open interventions, he developed recurrent pseudoaneurysms with an ultimately fatal outcome. Genetic testing revealed a pathogenic <i>COL3A1</i> mutation consistent with vascular Ehlers-Danlos syndrome. This case illustrates the diagnostic and therapeutic dilemma at the intersection of infection and heritable aortic diseases, underscoring the need for multidisciplinary management in complex aortic pathology.