Endovascular therapy for Stanford B aortic dissection for patients with Marfan Syndrome: systematic review and...

The role of thoracic endovascular aortic repair (TEVAR) in patients with Marfan Syndrome with Stanford type B aortic dissection (TBAD) remains under debate.

MEDLINE and EMBASE were searched through December 2021 to identify studies that investigated outcomes in MFS patients with TBAD who underwent TEVAR. Data regarding patient characteristics, perioperative and late outcomes were extracted.

Twelve studies were identified including 120 patients. The mean age was 40.2 years (95% confidence interval [CI], 36.8-43.6). 40.4% (95% CI: 10.8-70.0) of cases were performed emergently. 76.2% (95% CI: 64.6-87.8) of patients had a history of previous aortic surgery. In-hospital mortality was 3.7% (95% CI: 0.6-6.8). Primary endoleak occurred in 15.2% (95% CI: 8.6-21.8), which was comprised of type 1 (9.3% [95% CI: 3.9-14.6]) and type 2 (7.1% [95% CI: 2.3-12.0]) endoleaks. During mean follow-up period of 37.4 months (95% CI: 24.1-50.7), secondary endoleak was reported in 14.1% (95% CI: 7.1-21.1), which was comprised of type 1 (7.4% [95% CI: 2.4-12.5]) and type 2 (4.0% [95% CI: 0.3-7.7]) endoleak. Repeat TEVAR was performed in 15.5% (95% CI: 9.3-21.8) and open aortic surgery in 18.6% (95% CI: 9.6-27.5). Long-term mortality was 11.9% (95% CI: 6.5-17.3).

Our analysis showed that TEVAR for TBAD in patients with MFS has low perioperative morbidity and mortality but was associated with a high rate of late reintervention. This treatment option should be limited to emergent cases and to patients deemed unsuitable for open repair. Lifelong follow-up with imaging is mandatory in this population.