Delis Konstantinos T, Gloviczki Peter
Division of Vascular Surgery, Mayo Clinic.
Perspect Vasc Surg Endovasc Ther. 2005 Sep;17(3):187-203. doi: 10.1177/153100350501700302.
Middle aortic syndrome (MAS) is a clinical condition generated by segmental narrowing of the abdominal or distal descending thoracic aorta. MAS may be acquired, caused by Takayasu's or temporal arteritis (giant cell arteritides), neurofibromatosis, fibromuscular dysplasia, retroperitoneal fibrosis, mucopolysaccharidosis, and the Williams syndrome, or congenital, ascribed to a developmental anomaly in the fusion and maturation of the paired embryonic dorsal aortas. Segmental aortic stenosis may be located at the suprarenal, inter-renal or infrarenal aorta, with a high propensity for concomitant stenoses in both the renal (63%) and visceral (33%) arteries. Hypertension proximal to the aortic stenosis, and relative hypotension distal to it, are characteristic findings in MAS. Typical manifestations include headache, early fatigue on exertion, and bilateral lower-limb claudication. The severity of hypertension is the primary indication for intervention and the factor determining procedural timing. As a great proportion of patients with MAS are children or teenagers, the clinical benefits of early surgical intervention to reverse refractory hypertension have to be weighed against the repercussions pertaining to the insult of surgery on the developing aorta. Open surgery is the primary treatment of tubular aortic narrowing (MAS) associated with renovascular hypertension and visceral artery stenosis. This entails aortoaortic bypass of the diseased segment or, less often, patch aortoplasty and usually bypass grafting of the stenosed renal and visceral arteries performed with autologous conduits, particularly in the youngest of patients. Endovascular therapy may provide a sound minimally invasive treatment in MAS caused by discrete aortic stenoses that do not encompass the mesenteric and renal arteries. Hypertension is thus improved or cured in more than 70% of patients. Prognosis after uncompromised surgical reconstruction is rewarding in the mid and long term in patients with congenital aortic coarctation but deteriorates in patients with aortoarteritis and recurrent inflammatory activity.
中段主动脉综合征（MAS）是一种由腹主动脉或胸降主动脉远端节段性狭窄引起的临床病症。MAS可能是后天性的，由高安动脉炎或颞动脉炎（巨细胞动脉炎）、神经纤维瘤病、纤维肌发育异常、腹膜后纤维化、黏多糖贮积症和威廉姆斯综合征所致；也可能是先天性的，归因于成对的胚胎背主动脉融合和成熟过程中的发育异常。节段性主动脉狭窄可位于肾上腺上、肾间或肾下主动脉，肾动脉（63%）和内脏动脉（33%）同时出现狭窄的倾向较高。主动脉狭窄近端的高血压和远端的相对低血压是MAS的典型表现。典型症状包括头痛、运动早期疲劳和双侧下肢间歇性跛行。高血压的严重程度是干预的主要指征及决定手术时机的因素。由于很大一部分MAS患者是儿童或青少年，早期手术干预逆转难治性高血压的临床益处必须与手术对发育中的主动脉造成损伤的影响相权衡。开放手术是治疗与肾血管性高血压和内脏动脉狭窄相关的管状主动脉狭窄（MAS）的主要方法。这需要对病变节段进行主动脉-主动脉旁路移植术，较少情况下采用补片主动脉成形术，通常还需用自体血管对狭窄的肾动脉和内脏动脉进行旁路移植，尤其是最年幼的患者。对于由不涉及肠系膜和肾动脉的离散性主动脉狭窄引起的MAS，血管内治疗可能提供一种可靠的微创治疗方法。因此，超过70%的患者高血压得到改善或治愈。先天性主动脉缩窄患者在进行无并发症的手术重建后，中长期预后良好，但大动脉炎和反复炎症活动患者的预后会恶化。
