Long-term Outcomes of Open Thoracoabdominal Aortic Repair in Connective Tissue Disease Patients.

Abstract

This study aims to evaluate the short- and long-term outcomes of open thoracoabdominal aortic repair in patients with connective tissue disease. Retrospective single-institutional review of patients with clinically or genetically diagnosed connective tissue disease who underwent extent I-V open thoracoabdominal aortic repair between 2013 and 2025. The primary outcomes were 30-day and 1-year mortality. Secondary outcomes included neurologic, renal, cardiac, and pulmonary complications, visceral ischemia, long-term branch-graft patency, graft-related complications, and survival. 146 patients underwent open thoracoabdominal aortic repair during the study period, 56 had connective tissue disease and were included in the analysis. Average age was 42 ±12 years, and 68% (n=38) were male. Most of the patients were diagnosed with Marfan (68%, n=38), followed by Loeys-Dietz syndrome (14%, n=8). 91% of repairs were done electively for post-dissection aneurysms (n=51) requiring extent II thoracoabdominal aortic repair (68%, n=38). Visceral and/or renal artery reconstructions were performed in nearly all patients (96%, n=54). Most patients had prior aortic surgery performed (89%, n=50). The 30-day mortality was 2% (n=1), with one patient dying intraoperatively secondary to failure to wean from cardiopulmonary bypass. The 1-year mortality was 5% (n=3). Prolonged ventilator support >48 hours occurred in four patients (7%), with two (4%) requiring tracheostomy placement. Visceral ischemia occurred in four patients (7%). One patient (2%) had spinal cord ischemia with transient paraparesis, and two (4%) had renal failure requiring dialysis at discharge. At a mean imaging follow-up time of 59±48 months, the primary patency of the celiac, superior mesenteric, left renal and right renal arteries were 98%, 100%, 100%, and 94%, respectively. Graft-related complications were mainly secondary to intercostal patch aneurysms, which occurred in eight patients (15%), five of whom necessitated endovascular repair. At a mean follow-up of 68 ±47 months, the survival of operated cohort was 89%. Open thoracoabdominal aortic repair in connective tissue disease patients can be performed safely with good short- and long-term outcomes, and durable visceral and renal branch-graft patency. However, ongoing surveillance is critical in detecting graft related complications, especially intercostal patch aneurysms, which may require intervention.