冠状动脉不适窦起源(anomalous aortic origin of a coronary artery,AAOCA)是一种罕见的冠状动脉先天发育畸形,属于冠状动脉起源异常,指冠状动脉异位开口于对侧冠状动脉窦.该病临床表现隐匿,不同类型临床表现差异较大,异常起源的冠状动脉走行于主肺动脉间(合并壁内段走行),可导致运动诱发的心绞痛、晕厥,甚至猝死.冠状动脉CT血管造影能清晰显示冠状动脉起源位置及走行,是诊断的有效手段.有心肌缺血证据及潜在风险时,推荐手术矫正治疗,术后远期预后好.AAOCA为罕见病,发病率低,医师诊治经验有限,但高危型潜在危险性高,早期诊治有重要临床意义.该文对AAOCA病理生理、临床表现、诊断与治疗的研究进展进行综述,旨在提高儿科医师对AAOCA的认识,减少误诊漏诊以及由此造成的儿童心源性猝死的发生.
Advances in anomalous aortic origin of coronary artery in children
Anomalous aortic origin of a coronary artery(AAOCA)is a rare congenital malformation of the coronary artery,characterized by the coronary orifice origin from the inappropriate sinus of valsalva.Clinical presentations of this condition are occult,and different types present with various clinical manifestations,including exercise-induced angina,syncope,and even sudden death,particularly when the coronary artery follows an interarterial(intramural)course.Coronary computed comography angiography is an effective diagnostic method for precisely delineating the origin and shape of the coronary artery.Surgical correction is recommended upon evidence of myocardial ischemia and potential risk,and offers favorable long-term prognosis post-surgery.Despite low morbidity,limited experience in diagnosis and treatment,early diagnosis and timely treatment are of great importance because of the high potential risk related to the high-risk types.This paper aims to review the advances in the pathophysiology,clinical manifestations,diagnosis,and treatment of AAOCA to enhance recognition among pediatricians and reduce the incidence rate of sudden cardiac death in children with AAOCA.
ChildrenCoronary arteryAnomalous aortic originSudden death
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