Department of Vascular Medicine, German Aortic Center Hamburg, University Heart & Vascular Center Hamburg, Hamburg, Germany.
J Endovasc Ther. 2022 Aug;29(4):602-610. doi: 10.1177/15266028211067733. Epub 2021 Dec 30.
To study the outcome of endovascular treatment of aortic pathologies in patients with Marfan syndrome (MFS) at a single institution.
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.
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.
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.
研究单中心马凡综合征（MFS）患者主动脉病变的血管内治疗结果。
连续纳入 2010 年 1 月至 2020 年 5 月期间接受血管内修复或杂交手术治疗主动脉病变的 MFS 患者。采用了多种血管内和杂交策略。回顾性分析技术成功率、短期和中期存活率、并发症和再干预情况。
研究期间，共治疗 24 例 MFS 患者（中位年龄 48 [13-78] 岁；58%为男性）。干预指征为慢性主动脉夹层伴动脉瘤退行性变 16 例（67%），急性 B 型主动脉夹层 4 例（17%），无夹层主动脉瘤 3 例（13%），主动脉壁内血肿 1 例（4%）。大多数患者无症状（83%），3 例有症状（13%），1 例有破裂（4%）。中位动脉瘤直径为 56（35-86）mm。7 例（29%）患者行杂交手术。12 例（50%）患者行胸主动脉腔内修复术，4 例（17%）患者行开窗或分支主动脉腔内修复术，1 例（4%）患者行髂动脉支架置入术。12 例（50%）患者分期进行手术。所有患者均达到技术成功。患者 ICU 住院中位时间为 6（1-30）天，中位住院时间为 23（3-112）天。早期死亡率为 1 例（4%）。7 例（29%）患者发生伤口感染，3 例（13%）患者发生胃肠道并发症。中位随访时间为 42（1-127）个月。24 个月时累积生存率为 87%。12 个月时无再干预率为 77%。
血管内治疗 MFS 患者的主动脉病变在死亡率和再干预率方面具有良好的早期和中期效果，是可行的。血管内治疗在 MFS 患者的主动脉病变中发挥着越来越重要的作用。
