Long-term outcomes of open thoracoabdominal aortic repair in connective tissue disease patients

This study aims to evaluate the short- and long-term outcomes of open thoracoabdominal aortic repair in patients with connective tissue disease (CTD).

Retrospective single-institutional review of patients with clinically or genetically diagnosed CTD 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.

During the study period, 146 patients underwent open thoracoabdominal aortic repair, 56 patients with CTD were included in the analysis. Average age was 42 ± 12 years, and 68% patients (n = 38) were male. Most of the patients were diagnosed with Marfan syndrome (68%, n = 38), followed by Loeys-Dietz syndrome (14%, n = 8). Ninety-one percent of repairs were done electively for postdissection 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 previous 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 CTD 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.