• 摘要: 中主动脉综合征(MAS)是一种少见的血管性疾病,是由先天发育异常或后天因素导致的降主动脉下段及腹主动脉上段(即中段主动脉)的节段性狭窄,常累及内脏动脉及肾动脉,导致高血压、间歇性跛行及心力衰竭等表现的临床综合征。MAS占主动脉疾病的0.5%~2.0%,常发生在儿童青少年和青年人。由于其影响因素复杂,国内医师对MAS导致高血压的认识不足,给诊断和及时治疗带来了一定的困难,未经治疗的患者约80%在40岁前死于心力衰竭、脑出血或主动脉破裂等并发症。该病的诊治常需要多学科合作与个性化。为提高临床医师对该疾病的诊治能力,本共识由心血管内科、血管外科、风湿免疫科、儿科和医学影像科专家共同撰写制定,系统阐述了MAS的概念、病理生理机制、分类、临床特点,对MAS导致高血压的诊断、评估及治疗提出了推荐意见,旨在规范MAS的诊断标准和治疗策略。强调早期识别和多学科协作的重要性,提出根据病因、狭窄类型和年龄等特点选择个体化治疗方案。本共识明确提出了围手术期管理要点和长期随访方案,旨在改善临床医师对MAS导致高血压的重视程度以及提高其诊治能力。

     

    Abstract: Middle aortic syndrome (MAS) is a rare vascular disease characterized by segmental narrowing of the distal descending aorta and the upper abdominal aorta (i.e., the middle section of the aorta) due to congenital developmental abnormalities or acquired factors. It often involves visceral arteries and renal arteries, leading to a clinical syndrome manifested by hypertension, intermittent claudication, and heart failure. MAS accounts for 0.5% to 2.0% of aortic diseases and commonly occurs in children, adolescents, and young adults. Due to its complex influencing factors, domestic physicians have insufficient understanding of hypertension caused by MAS, which poses certain difficulties in diagnosis and timely treatment. Approximately 80% of untreated patients die from complications such as heart failure, cerebral hemorrhage, or aortic rupture before the age of 40. The diagnosis and treatment of this disease often require multidisciplinary cooperation and individualized approaches. To improve clinical physicians' ability to diagnose and treat this condition, this consensus was jointly drafted by experts from cardiology, vascular surgery, rheumatology and immunology, pediatrics, and medical imaging. It systematically elaborates on the concept, pathophysiological mechanisms, classification, and clinical characteristics of MAS, and provides recommendations for the diagnosis, evaluation, and treatment of hypertension caused by MAS, aiming to standardize diagnostic criteria and treatment strategies. Emphasis is placed on early recognition and multidisciplinary collaboration, with individualized treatment strategies proposed based on etiology, type of stenosis, and age. This consensus clearly outlines perioperative management key points and long-term follow-up plans, aiming to raise clinical physicians' awareness of hypertension caused by MAS and enhance their diagnostic and therapeutic capabilities.

     

/

返回文章
返回