Results of Open Surgical Repair in Patients With Marfan Syndrome and Distal Aortic Dissection

Abstract

Background: In patients with Marfan syndrome (MFS), distal aortic dissection can necessitate thoracoabdominal aortic aneurysm (TAAA) repair in survivors of acute DeBakey type I dissection and those with DeBakey type III dissection. We examined outcomes of surgical repair of TAAA in patients with MFS with distal aortic dissection.

Methods: Data were analyzed for 127 consecutive TAAA repairs performed between January 2004 and June 2014 in patients with MFS and distal aortic dissection-DeBakey types I (n = 73) and III (n = 54). The median time from dissection onset to TAAA repair was 5.2 years (interquartile range [IQR]: 2.1 to 9.8 years) for the overall group and was longer in patients with DeBakey I (6.5 years, IQR: 3.5 to 13.9 years) than patients with DeBakey III (2.9 years, IQR: 0.6 to 6.0 years, p < 0.001). Eleven patients (9%) had acute or subacute dissection at the time of repair. Sixty-six patients (52%) underwent Crawford extent II TAAA repair. A composite end point, adverse event, was defined as operative death or permanent stroke, renal failure, paraplegia, or paraparesis.

Results: Eight patients had adverse events (6%), including 5 operative deaths (4%). There was no permanent stroke and 1 case each of permanent paraplegia and paraparesis. At discharge, 2 early survivors (2%) had renal failure. Extent II repairs did not have substantially different outcomes from other repairs.

Conclusions: In these patients with MFS with aortic dissection, open TAAA repair incurred reasonable operative risk, but improvements are needed to reduce rates of renal failure. Extent II TAAA repair does not appear to increase operative risk in patients with MFS.