Departments of Cardiac and Vascular Surgery, University Hospital Birmingham NHS Foundation Trust, Birmingham, UK.
Eur J Cardiothorac Surg. 2022 Aug 3;62(3). doi: 10.1093/ejcts/ezac137.
A relatively small proportion of patients with heritable thoracic aortic disease require open surgical replacement of the distal thoracic aorta. We reviewed the outcome in patients with Marfan and Loeys-Dietz syndromes treated in an aortic centre in the United Kingdom.
We performed a single-centre retrospective study of consecutive patients treated between October 1999 and December 2019. The primary end point was 30-day mortality. Secondary end points were Kaplan-Meier estimates of medium-term survival and freedom from distal reintervention. Data are presented as median (interquartile range).
A total of 58 patients [33 men; 51 with Marfan syndrome; median age 41 years (35-48); median aneurysm diameter 60 mm (55-74)] underwent open descending (n = 21) or thoracoabdominal aortic replacement (n = 37). All repairs were performed using cardiopulmonary bypass with hypothermic circulatory arrest in 31 patients. The 30-day mortality was 5.2% (n = 3, including 2 patients ≥ 60 years with significant comorbidity). Major non-fatal complications included early reoperation (n = 7), tracheostomy (n = 9), temporary renal replacement therapy (n = 3), permanent spinal cord deficit (n = 2) and permanent stroke (n = 1). Median follow-up was 81 months (48-127). Estimated (±standard error) 5-year survival was 85% ±5%. Seven patients had distal aortic reintervention with no deaths or spinal cord deficit: estimated 5-year freedom from distal reintervention was 94% ±3%. There was no difference in survival or freedom from distal reintervention comparing: elective vs. non-elective; type of heritable thoracic aortic disease; DeBakey type; or extent of surgical repair.
Descending thoracic and thoracoabdominal aortic replacement in patients with heritable thoracic aortic disease can be performed with low perioperative morbidity and mortality, satisfactory long-term survival and low requirement for distal reintervention.
遗传性胸主动脉疾病患者中，仅有一小部分需要进行开放性外科手术置换胸主动脉远端。我们回顾了在英国一家主动脉中心接受治疗的马凡综合征和洛伊茨-迪茨综合征患者的治疗结果。
我们对 1999 年 10 月至 2019 年 12 月期间连续收治的患者进行了单中心回顾性研究。主要终点是 30 天死亡率。次要终点是中期生存和免于远端再干预的 Kaplan-Meier 估计。数据以中位数（四分位距）表示。
共 58 例患者[33 例男性；51 例马凡综合征；中位年龄 41 岁（35-48）；中位瘤径 60mm（55-74）]接受了开放性降主动脉（n=21）或胸腹主动脉置换术（n=37）。所有手术均在体外循环下进行，其中 31 例采用深低温循环中断。30 天死亡率为 5.2%（n=3，包括 2 例≥60 岁且合并严重合并症的患者）。主要非致命性并发症包括早期再次手术（n=7）、气管切开术（n=9）、临时肾脏替代治疗（n=3）、永久性脊髓损伤（n=2）和永久性中风（n=1）。中位随访时间为 81 个月（48-127）。估计（±标准误差）5 年生存率为 85%±5%。7 例患者行远端主动脉再干预，无死亡或脊髓损伤：估计 5 年免于远端再干预率为 94%±3%。在生存或免于远端再干预方面，择期与非择期、遗传性胸主动脉疾病类型、DeBakey 分型或手术修复范围之间无差异。
遗传性胸主动脉疾病患者行降主动脉和胸腹主动脉置换术，围手术期发病率和死亡率较低，长期生存满意，远端再干预需求较低。
