Yi Dong, Xia Juan, Zhou Xiang, Liu Chengwei, Liu Li, Yan Hua, Ma Xiaojing
Department of Cardiology, Wuhan Asia Heart Hospital, Wuhan, China.
Department of Echocardiography, Wuhan Asia Heart Hospital, Wuhan, China.
Front Cardiovasc Med. 2023 Apr 20;10:1160893. doi: 10.3389/fcvm.2023.1160893. eCollection 2023.
Both the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and unilateral absence of the pulmonary artery (UAPA) are rare congenital malformations, ALCAPA accompanied with UAPA is extremely rare. Here, we reported a middle-aged man admitted to our department for evaluation of chest pain during exercise. Physical examination and lab tests did not unveil obvious abnormality; however, transthoracic echocardiogram (TTE) revealed multivessel myocardial collateral blood flow signals in the left ventricular wall and ventricular septum, a shunting flow from the left coronary artery into the pulmonary artery and dilated right coronary artery (RCA), which supported but did not confirm the diagnosis of ALCAPA. Coronary angiography (CAG) showed an absent left coronary ostium and a dilated RCA, with extensive collaterals supplying the left coronary system. Multidetector computed tomography angiography (MDCTA) was then performed and revealed the anomalous origin of the left main coronary artery (LMCA) arising from the pulmonary artery, and it incidentally unveiled another rare congenital malformation of UAPA. The patient underwent surgical correction of ALCAPA by reimplantation of the LMCA to the aorta, without surgical treatment of UAPA. The patient had been in good clinical condition and remained angina free with good exercise tolerance during follow-up (∼6 months so far). In this case, we discussed the diagnostic value of TTE, CAG, and MDCTA on rare abnormalities as ALCAPA and UAPA. We highlighted the role of multiple non-invasive imaging modalities in diagnosing rare causes of angina in adult patients, and the importance of careful examination in avoiding misdiagnosis. To our best knowledge, this is the first report of ALCAPA accompanied with UAPA in an adult patient.
左冠状动脉起源于肺动脉（ALCAPA）和单侧肺动脉缺如（UAPA）均为罕见的先天性畸形，ALCAPA合并UAPA极为罕见。在此，我们报告一名中年男性因运动时胸痛入院。体格检查和实验室检查未发现明显异常；然而，经胸超声心动图（TTE）显示左心室壁和室间隔有多支心肌侧支血流信号、左冠状动脉向肺动脉的分流以及右冠状动脉（RCA）扩张，这支持但未确诊ALCAPA。冠状动脉造影（CAG）显示左冠状动脉开口缺如且RCA扩张，有广泛的侧支血管供应左冠状动脉系统。随后进行了多排螺旋CT血管造影（MDCTA），显示左冠状动脉主干（LMCA）起源于肺动脉异常，且偶然发现了另一种罕见的先天性畸形UAPA。患者接受了将LMCA重新植入主动脉的ALCAPA手术矫正，未对UAPA进行手术治疗。患者临床状况良好，随访期间（迄今约6个月）未再发生心绞痛，运动耐量良好。在本病例中，我们讨论了TTE、CAG和MDCTA对ALCAPA和UAPA等罕见异常的诊断价值。我们强调了多种非侵入性成像方式在诊断成年患者罕见心绞痛病因中的作用，以及仔细检查以避免误诊的重要性。据我们所知，这是首例成年患者中ALCAPA合并UAPA的报告。
