研究动态
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异常起源于肺动脉的左冠状动脉综合征的临床表现及计算机断层血管造影特征。

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

发表日期:2023 Jul 28
作者: Chunhua Fan, Mu Zeng, Ting Huang, Xilong Mei, Bo Jiang, Jun Liu, Dehong Peng
来源: HEART & LUNG

摘要:

肺动脉起源异常的左冠状动脉(ALCAPA)综合征是一种罕见的先天性冠状动脉畸形,具有临床错诊和误诊的高发率。目前,对ALCAPA的临床表现和冠状动脉计算机断层扫描血管造影(CTA)的影像特征的报道尚缺乏。本研究旨在总结ALCAPA的临床特征和冠状动脉CTA的影像特征,以改善临床诊断和治疗。回顾性收集、分析和总结了2023年1月1日至2023年2月1日在中南大学湘雅二医院诊治的24例ALCAPA综合征患者的临床数据。根据左、右冠状血管侧支循环,将患者分为幼儿型(7例)和成人型(17例)。比较了两种类型患者的临床表现和CTA影像特征的差异。在24例患者中,男女比例为1:5,幼儿型的中位发病年龄为3.22个月,成人型为22.0岁。幼儿型在早期表现为左心功能不全的症状,而成人型的临床表现多样,其中7例无症状,在体格检查中出现心脏杂音,6例出现慢性心肌缺血的症状,2例出现恶性心律失常。CTA显示,11例患者的左冠状动脉起源于肺动脉的左后窦。所有7例幼儿型患者均表现为左心增大、左室肥厚、左心功能减退、左、右冠状动脉之间无相互血运,并且冠状动脉直径正常。所有17例成人型患者均表现为扩张扭曲的冠状动脉,具有丰富的侧支循环,其中7例成人型患者左心功能正常。两种类型ALCAPA患者的临床表现和CTA影像特征不同,而CTA表现具有特征性,可作为ALCAPA综合征的确诊、分期、手术评估和术后随访的手段。
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.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.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.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.