Endovascular Treatment of Aortic Pathologies in Patients With Marfan Syndrome: Single-Center Experience

Objectives: To study the outcome of endovascular treatment of aortic pathologies in patients with Marfan syndrome (MFS) at a single institution. Methods: Consecutive MFS patients who underwent endovascular repair or hybrid procedures for aortic pathologies from January 2010 to May 2020 were identified. Several endovascular and hybrid strategies have been used. Technical success, short- and mid-term survival, complications, and re-interventions were retrospectively analyzed. Results: During the study period, 24 patients with MFS (median age, 48 [13-78] years; 58% males) were treated. Indications for intervention were chronic aortic dissection with aneurysm degeneration in 16 patients (67%), acute type B aortic dissection in 4 patients (17%), aortic aneurysm without any dissection in 3 patients (13%), and aortic intramural hematoma in 1 patient (4%). Most patients were asymptomatic (83%), three (13%) were symptomatic and one (4%) had a contained rupture. The median aneurysm diameter was 56 (35-86) mm. Hybrid procedures were performed in 7 (29%) patients. Thoracic endovascular repair was performed in 12 (50%) patients, a fenestrated or branched endovascular aortic repair in 4 (17%) patients, and placement of an iliac artery stent-graft in 1 (4%) patient. Procedures were staged in 12 (50%) patients. Technical success was achieved in all patients. The median intensive care unit stay was 6 (range, 1-30) days, and the median hospital stay was 23 (range, 3-112) days. Early mortality was reported in 1 (4%) patient. Wound infection was seen in 7 (29%) patients and gastrointestinal complications in 3 (13%) patients. The median follow-up was 42 (range, 1-127) months. The cumulative survival rate was 87% at 24 months. The cumulative freedom from re-intervention was 77% at 12 months. Conclusions: Endovascular treatment of aortic pathologies in patients with MFS appears feasible with acceptable early and mid-term outcomes in terms of mortality and re-intervention rates. Endovascular therapy plays an increasing role in MFS patients with aortic pathology.

Automated summary

The study showed that endovascular treatment of aortic pathologies in patients with Marfan syndrome is feasible with high technical success rates, acceptable survival rates, and freedom from re-intervention rates. Endovascular therapy plays an increasingly important role in managing aortic pathology in Marfan syndrome patients.