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

Abstract

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.

Outcomes After Endovascular Aortic Intervention in Patients With Connective Tissue Disease

“…[30][31][32][33] In accordance with previous reports of endovascular treatment of thoracic, thoracoabdominal, and abdominal aortic disease in patients with CTD, the primary technical success was excellent in the EVICTUS cohort. [34][35][36][37][38][39] The perioperative mortality and morbidity were acceptable for patients with Marfan syndrome or Loeys-Dietz syndrome, especially given that compared with the reports of open surgery, this cohort featured patients with an older median age and more patients emergently treated for complicated dissections, as well as symptomatic or ruptured aneurysms. These results are also consistent with a recent report of thoracic endovascular repairs in patients with CTD of similar age with an analogous proportion of emergent procedures.…”

Secondary Endovascular Conversions for Failed Open Repair

“… 35 In cases already submitted to open repair, allowing for proximal or proximal and distal landing zones in synthetic grafts, endovascular repair may be a good option by overcoming this limitation. 35 36 Clough et al 35 showed that this strategy was both feasible and safe, although more mid- and long-term follow-up is needed to correctly assess durability of these repairs. Connective tissue disease patients are frequently burdened with multiple reinterventions, which significantly impairs their quality of life via anxiety related to surgeries, which may lead them to seek less invasive options.…”

Comparison of Immediate, Medium, and Long-Term Postoperative Results of Open Surgery and Fenestrated/Branched Stent Grafts for Extended Thoracoabdominal Aortic Aneurysms

“…Data on F/BEVAR for TAAAs related to connective tissue disorders are still lacking. Only a few studies with limited numbers have been published [99][100][101]. In a recent literature review [102], 26 patients with TAAAs related to connective tissue disorders and treated by F/BEVAR could be identified.…”