The Impact of Connective Tissue Disorders on Patients with Thoracic Aortic Pseudoaneurysms

ABSTRACT Introduction We sought to review the clinical presentation, etiologies, diagnostic methods, surgical techniques, and long-term outcomes of patients with thoracic aortic false aneurysms (TAFA) treated at our institution. Although connective tissue disorders (CTDs) are recognized risk factors for aortic aneurysms, their role in the development and management of false aneurysms is less well established. The aim of this study was to compare cohorts of patients with and without CTD. Methods A total of 116 patients were diagnosed with a thoracic aortic pseudoaneurysm between January 1996 and August 2024 at our institute. Genetic screening confirmed a CTD in 18 patients. Follow-up was obtained for all patients through echocardiographic or computed tomography assessment. Results In all cases, TAFA developed after prior cardiovascular surgery. In the CTD cohort, the most common initial diagnosis was aortic dissection (72%), and the most frequent primary operation was the Bentall procedure (88%). Ten patients with CTD were indicated for reintervention (surgical reoperation, endoluminal graft implantation, or occluder implantation). Conclusion One-year survival was higher in patients without CTD (98%) than in those with CTD (89%); however, ten-year survival was higher in the CTD cohort (89% vs. 68%). After surgical reoperation, secondary TAFA recurrence was lower in patients without CTD (16% vs. 22%), although recurrence occurred sooner in this group. Operative mortality was 11.1% in patients with CTD and 5.1% in those without CTD.