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Abstract：
Non-atherosclerotic aortic arch pathologies (NA-AAPs) and anatomical variants are characterized as rare cardiovascular diseases with a low incidence rate, below 1 case per 2000 population, but enormous heterogeneity in terms of anatomical variants, i.e., Takayasu disease (TAK) and fibromuscular dysplasia (FMD). In specific clinical scenarios, NA-AAPs constitute life-threatening disorders.
METHODS: In this study, 82 (1.07%) consecutive patients with NA-AAPs (including 38 TAKs, 26 FMDs, and 18 other AAPs) out of 7645 patients who underwent endovascular treatment (EVT) for the aortic arch and its side-branch diseases at a single institution between 2002 and 2022 were retrospectively reviewed. The recorded demographic, biochemical, diagnostic, operative, and postoperative factors were reviewed, and the functional outcomes were determined during follow-up. A systematic review of the literature was also performed.
RESULTS: The study group comprised 65 (79.3%) female and 17 (21.7%) male subjects with a mean age of 46.1 ± 14.9 years. Overall, 62 (75.6%) patients were diagnosed with either cerebral ischemia symptoms or aortic arch dissection on admission. The EVT was feasible in 59 (72%) patients, whereas 23 (28%) patients were referred for medical treatment. In EVT patients, severe periprocedural complications occurred in two (3.39%) patients, including one periprocedural death and one cerebral hyperperfusion syndrome. During a median follow-up period of 64 months, cardiovascular events occurred in 24 (29.6%) patients (5 deaths, 13 ISs, and 6 myocardial infarctions). Repeated EVT for the index lesion was performed in 21/59 (35.6%) patients, including 19/33 (57.6%) in TAK and 2/13 (15.4%) in FMD. In the AAP group, one patient required additional stent-graft implantation for progressing dissection to the iliac arteries at 12 months. A baseline white blood count (odds ratio [HR]: 1.25, 95% confidence interval [CI]: 1.11-1.39; p < 0.001) was the only independent prognostic factor for recurrent stenosis, while a baseline hemoglobin level (HR: 0.73, 95%CI: 0.59-0.89; p = 0.002) and coronary involvement (HR: 4.11, 95%CI: 1.74-9.71; p = 0.001) were independently associated with a risk of major cardiac and cerebral events according to the multivariate Cox proportional hazards regression analysis.
CONCLUSIONS: This study showed that AAPs should not be neglected in clinical settings, as it can be a life-threatening condition requiring a multidisciplinary approach. The knowledge of prognostic risk factors for adverse outcomes may improve surveillance in this group of patients.
METHODS: In this study, 82 (1.07%) consecutive patients with NA-AAPs (including 38 TAKs, 26 FMDs, and 18 other AAPs) out of 7645 patients who underwent endovascular treatment (EVT) for the aortic arch and its side-branch diseases at a single institution between 2002 and 2022 were retrospectively reviewed. The recorded demographic, biochemical, diagnostic, operative, and postoperative factors were reviewed, and the functional outcomes were determined during follow-up. A systematic review of the literature was also performed.
RESULTS: The study group comprised 65 (79.3%) female and 17 (21.7%) male subjects with a mean age of 46.1 ± 14.9 years. Overall, 62 (75.6%) patients were diagnosed with either cerebral ischemia symptoms or aortic arch dissection on admission. The EVT was feasible in 59 (72%) patients, whereas 23 (28%) patients were referred for medical treatment. In EVT patients, severe periprocedural complications occurred in two (3.39%) patients, including one periprocedural death and one cerebral hyperperfusion syndrome. During a median follow-up period of 64 months, cardiovascular events occurred in 24 (29.6%) patients (5 deaths, 13 ISs, and 6 myocardial infarctions). Repeated EVT for the index lesion was performed in 21/59 (35.6%) patients, including 19/33 (57.6%) in TAK and 2/13 (15.4%) in FMD. In the AAP group, one patient required additional stent-graft implantation for progressing dissection to the iliac arteries at 12 months. A baseline white blood count (odds ratio [HR]: 1.25, 95% confidence interval [CI]: 1.11-1.39; p < 0.001) was the only independent prognostic factor for recurrent stenosis, while a baseline hemoglobin level (HR: 0.73, 95%CI: 0.59-0.89; p = 0.002) and coronary involvement (HR: 4.11, 95%CI: 1.74-9.71; p = 0.001) were independently associated with a risk of major cardiac and cerebral events according to the multivariate Cox proportional hazards regression analysis.
CONCLUSIONS: This study showed that AAPs should not be neglected in clinical settings, as it can be a life-threatening condition requiring a multidisciplinary approach. The knowledge of prognostic risk factors for adverse outcomes may improve surveillance in this group of patients.
摘要：
非动脉粥样硬化性主动脉弓病变（NA-AAP）和解剖变异是罕见的心血管疾病，发病率低，每2000人低于1例，但是解剖变异方面的巨大异质性，即，Takayasu病（TAK）和纤维肌性发育不良（FMD）。在特定的临床场景中，NA-AAP构成危及生命的疾病。
方法：在本研究中，82例（1.07%）连续的NA-AAP患者（包括38例TAKs，26个FMD,对2002年至2022年间在一家机构接受主动脉弓及其侧支疾病血管内治疗(EVT)的7645例患者中的18例其他AAP)进行了回顾性分析.记录的人口统计，生物化学，诊断,Operative,并对术后因素进行了回顾，并在随访期间确定功能结局.还对文献进行了系统回顾。
结果：研究组包括65名（79.3％）女性和17名（21.7％）男性受试者，平均年龄为46.1±14.9岁。总的来说,62例（75.6％）患者入院时被诊断为脑缺血症状或主动脉弓夹层。59例（72%）患者的EVT是可行的，而23例(28%)患者被转诊接受治疗。在EVT患者中，严重的围手术期并发症发生在两名（3.39%）患者中，包括一例围手术期死亡和一例脑高灌注综合征.在64个月的中位随访期内，24例（29.6%）患者发生心血管事件（5例死亡，13IS，和6次心肌梗塞)。在21/59（35.6％）的患者中，对指示性病变进行了重复EVT，包括TAK中的19/33（57.6％）和口蹄疫中的2/13（15.4％）。在AAP组中，1例患者在12个月时需要额外的支架移植物植入术,以进行髂动脉夹层.基线白细胞计数（比值比[HR]：1.25，95％置信区间[CI]：1.11-1.39;p<0.001）是复发性狭窄的唯一独立预后因素，根据多变量Cox比例风险回归分析,基线血红蛋白水平(HR:0.73,95CI:0.59-0.89;p=0.002)和冠状动脉受累(HR:4.11,95CI:1.74-9.71;p=0.001)与主要心脑事件的风险独立相关.
结论：这项研究表明，在临床环境中不应忽略AAP，因为这可能是一种危及生命的疾病，需要多学科的方法。了解不良结局的预后危险因素可能会改善该组患者的监测。
方法：在本研究中，82例（1.07%）连续的NA-AAP患者（包括38例TAKs，26个FMD,对2002年至2022年间在一家机构接受主动脉弓及其侧支疾病血管内治疗(EVT)的7645例患者中的18例其他AAP)进行了回顾性分析.记录的人口统计，生物化学，诊断,Operative,并对术后因素进行了回顾，并在随访期间确定功能结局.还对文献进行了系统回顾。
结果：研究组包括65名（79.3％）女性和17名（21.7％）男性受试者，平均年龄为46.1±14.9岁。总的来说,62例（75.6％）患者入院时被诊断为脑缺血症状或主动脉弓夹层。59例（72%）患者的EVT是可行的，而23例(28%)患者被转诊接受治疗。在EVT患者中，严重的围手术期并发症发生在两名（3.39%）患者中，包括一例围手术期死亡和一例脑高灌注综合征.在64个月的中位随访期内，24例（29.6%）患者发生心血管事件（5例死亡，13IS，和6次心肌梗塞)。在21/59（35.6％）的患者中，对指示性病变进行了重复EVT，包括TAK中的19/33（57.6％）和口蹄疫中的2/13（15.4％）。在AAP组中，1例患者在12个月时需要额外的支架移植物植入术,以进行髂动脉夹层.基线白细胞计数（比值比[HR]：1.25，95％置信区间[CI]：1.11-1.39;p<0.001）是复发性狭窄的唯一独立预后因素，根据多变量Cox比例风险回归分析,基线血红蛋白水平(HR:0.73,95CI:0.59-0.89;p=0.002)和冠状动脉受累(HR:4.11,95CI:1.74-9.71;p=0.001)与主要心脑事件的风险独立相关.
结论：这项研究表明，在临床环境中不应忽略AAP，因为这可能是一种危及生命的疾病，需要多学科的方法。了解不良结局的预后危险因素可能会改善该组患者的监测。
