Faculty of Medicine, Imperial College School of Medicine, Imperial College London, London, UK.
Leicester Medical School, College of Life Sciences, University of Leicester, Leicester, UK.
J Card Surg. 2021 Mar;36(3):1083-1090. doi: 10.1111/jocs.15340. Epub 2021 Jan 21.
Patients with connective tissue diseases are at high lifetime risk of developing thoracic aortic aneurysms (TAAs) due to defects in extracellular matrix composition which compromise the structural integrity of the aortic wall. It is vital to identify and manage aneurysms early to prevent fatal complications such as dissection or rupture.
This review synthesises information obtained from a thorough literature search regarding the pathophysiology of TAAs in those with heritable connective tissue diseases (HCTDs), the investigations for timely diagnosis and current operative strategies.
Major complications of open repair (OR) include pneumonia (32%), haemorrhage (31%) and tracheostomy (18%), with a minor risk of vocal cord paresis (9%). For thoracic endovascular aortic repair (TEVAR), high rates of endoleak were documented (38-66.6%). Reintervention rates for TEVAR are also high at 38-44%. Mortality rates were documented as 25% for open repair and vary from 14% to 44% for TEVAR.
OR remains the mainstay of surgical management. While TEVAR use is expanding, it remains the alternative choice due to concerns over endograft durability, limited long-term outcome data and the lack of high-quality evidence regarding its use in HCTD patients.
由于细胞外基质组成的缺陷，结缔组织疾病患者终生患胸主动脉瘤（TAA）的风险很高，这会损害主动脉壁的结构完整性。早期发现和管理动脉瘤对于预防夹层或破裂等致命并发症至关重要。
本综述综合了从全面文献检索中获得的关于遗传性结缔组织疾病（HCTD）患者 TAA 的病理生理学、及时诊断的检查方法和当前手术策略的信息。
开放性修复（OR）的主要并发症包括肺炎（32%）、出血（31%）和气管造口术（18%），声带麻痹的风险较小（9%）。对于胸主动脉腔内修复术（TEVAR），记录到较高的内漏率（38-66.6%）。TEVAR 的再次介入率也很高，为 38-44%。OR 的死亡率为 25%，TEVAR 的死亡率从 14%到 44%不等。
OR 仍然是手术治疗的主要方法。虽然 TEVAR 的使用正在扩大，但由于对移植物耐久性的担忧、有限的长期结果数据以及缺乏关于其在 HCTD 患者中使用的高质量证据，它仍然是替代选择。
