左冠状动脉异常起源于肺动脉综合征:临床表现及CTA影像学特征

Clinical manifestations and computed tomography angiography features of anomalous left coronary artery from the pulmonary artery syndrome

目的:左冠状动脉异常起源于肺动脉(anomalous left coronary artery from the pulmonary artery,ALCAPA)综合征是一种罕见的先天性冠状动脉畸形,临床漏诊及误诊率高,目前鲜见ALCAPA综合征的临床表现及冠状动脉CT血管成像(computed tomography angiography,CTA)影像特点的相关报道,本研究旨在总结ALCAPA综合征的临床表现及冠状动脉CTA影像特征,提升临床诊疗水平。方法:回顾性收集2013年1月1日至2023年2月1日中南大学湘雅二医院收治的24例ALCAPA综合征患者的临床资料,根据左、右冠状动脉侧支循环情况分为婴儿型(7例)和成人型(17例),比较两型患者的临床表现及CTA影像特点。结果:24例ALCAPA综合征患者的男女比例为1꞉5,婴儿型中位发病年龄为3.22个月,成人型中位发病年龄为22.0岁。婴儿型早期即出现左心功能不全的症状;而成人型临床表现多样,7例患者无症状因体检发现心脏杂音而就诊,6例患者出现慢性心肌缺血症状,2例患者出现恶性心律失常。CTA示11例患者左冠状动脉起源于肺动脉左后窦;7例婴儿型患者均出现左心扩大、左室肥厚、左心功能下降,左、右冠状动脉间缺乏侧支循环,冠状动脉管径正常。17例成人型患者均出现冠状动脉扩张、迂曲及发达的侧支循环;7例成人型患者左心功能保留。结论:两型ALCAPA患者的临床及影像学特点不同,CTA表现具有特征性,可作为ALCAPA综合征明确诊断、分型、手术评估及术后随访的手段。

Objective:Anomalous left coronary artery from the pulmonary artery(ALCAPA)syndrome is a rare congenital coronary artery malformation with a high rate of clinical missed diagnosis and misdiagnosis.At present,there is a lack of reports on the clinical manifestations of ALCAPA and the imaging features of coronary computed tomography angiography(CTA).This study aims to summarize the clinical characteristics and coronary CTA imaging features of ALCAPA to improve the clinical diagnosis and treatment.Methods:The clinical data of 24 ALCAPA syndrome patients were retrospectively collected,analyzed and summarized from January 1,2013 to February 1,2023 in the Second Xiangya Hospital of Central South University.According to the left and right coronary collateral circulation,the patients were classified into infantile type(7 cases)and adult type(17 cases).The differences of clinical manifestations and CTA imaging features were compared between the 2 types of patients.Results:Of the 24 patients,a male-to-female ratio was at 1꞉5,and the median age of onset was 3.22 months for the infant type and 22.0 years for the adult type.The infantile type showed symptoms of left heart insufficiency at an early stage,while the adult type had a variety of clinical manifestations in 7 patients being asymptomatic and presented with a cardiac murmur on physical examination,6 with symptoms of chronic myocardial ischemia,and 2 with malignant arrhythmias.CTA showed that 11 patients’the left coronary artery originated from the left posterior sinus of the pulmonary artery.All 7 infantile type patients had an enlarged left heart,left ventricular hypertrophy,reduced left heart function,lack of collateral circulation between the left and right coronary arteries,and normal coronary artery diameter.All 17 adult type patients showed dilated and tortuous coronary arteries with rich collateral circulation,and 7 adult type patients had preserved left heart function.Conclusion:The clinical manifestations and CTA imaging features of patients with the 2 types of ALCAPA are different,while CTA performance is characteristic and can be used as a means of definitive diagnosis,staging,surgical evaluation,and postoperative follow-up of ALCAPA syndrome.