Risk Profile And Operative Outcomes In Marfan Syndrome Versus Non-Marfan Patients Undergoing Thoracoabdominal Aortic Aneurysm Repair.
Academic Article
Overview
abstract
OBJECTIVES: To compare operative and long-term outcomes between Marfan and non-Marfan patients undergoing thoracoabdominal aortic aneurysm (TAAA) repair. METHODS: We identified all consecutive patients undergoing TAAA repair between 1997 and 2022. Primary outcome was composite of major adverse events (MAE). Secondary outcomes were individual complications and long-term survival. Inverse probability of treatment weighting was performed. Weighted Kaplan-Meier (KM) curves were used to estimate long-term survival. Multivariable analysis identified factors associated with MAE. RESULTS: 684 patients underwent open TAAA repair. 90 (13.1%) had Marfan syndrome, while 594 (86.9%) did not. Marfan patients were younger (46[36-56] vs 69[61-76], p<0.001). Extent II-III aneurysms (57/90, 63.3% vs 211/594, 35.6%, p<0.001) and type I or III chronic dissection (77/90, 85.3% vs 242/594, 40.8%, p<0.001) were more common. Cardiovascular risk factors were less frequent in Marfan patients. There was no difference in MAE between groups (12/90, 13.3% vs 100/594, 16.8%, p=0.49). Operative mortality was similar between groups (3/90, 3.3% vs 28/594, 4.7%, p=0.75). Unweighted survival at 10 years was 78.7% vs 46.8% (p=0.001). Weighted KM curves showed no difference in long-term survival (adjusted HR 0.79; CI 0.32-1.99, p=0.62; Log-rank p=0.12). At multivariable analysis, renal insufficiency (OR 2.29; CI 1.43-3.68, p<0.01) and urgent/emergent procedure (OR 2.17; CI 1.35-3.48, p<0.01) were associated with MAE, while Marfan syndrome was not (OR 1.56; CI 0.69-3.49, p=0.28). CONCLUSIONS: Open TAAA repair can be performed with similar operative outcomes in Marfan and non-Marfan patients despite differing risk profiles. Operative/perioperative strategies must be tailored to specific needs of the patient to optimize outcomes.