Outcomes of endovascular therapy for Stanford type B aortic dissection in patients with Marfan syndrome.

OBJECTIVE

To evaluate the mid-term outcomes of thoracic endovascular aorta repair (TEVAR) for Stanford type B aortic dissection (TBAD) in patients with Marfan syndrome (MFS).

METHODS

Between January 2009 and December 2019, patients with MFS who underwent TEVAR for TBAD were enrolled. Demographic data, preoperative and perioperative clinical profiles, and follow-up data were collected and analyzed. The cumulative survival and freedom from reintervention rates were calculated with Kaplan-Meier analysis.

RESULTS

A total of 26 patients were enrolled. The mean age was 38.5 ± 10.7 (range, 24-64 years). The in-hospital mortality was 0. The cumulative survival rate was 88.1% (95% confidence interval [CI], 67.5%-98.5%) at 5 years and 82.9% (95% CI, 60.2%-93.3%) at 10 years. Patients with a thrombosed false lumen (FL) along the length of the stent had a significantly higher cumulative survival rate (P < .05) and freedom from reintervention (P = .01) than patients with patent FL. The freedom from reintervention was 83.4% (95% CI, 61.4% to 93.4%) at 5 years and 50.3% (95% CI, 21.4%-97.3%) at 10 years. There was no significant difference freedom from reintervention in freedom from reintervention between patients with and those without a previous aortic root procedure (P = .46).

CONCLUSIONS

TEVAR can be performed safely and effectively for TBAD in patients with MFS. Perioperative mortality and morbidity were low; however, lifelong close follow-up in the clinic and imaging are mandatory to prevent aortic rupture. Patients with a patent FL were at high risk of late death.