Kandel Devraj, Mustafa Irfa, Rajlawot Kritisha, Neupane Nirmal Prasad, Sitaula Asim
Department of Radiodiagnosis and Imaging, Shahid Gangalal National Heart Centre, Bansbari, Bagmati +977, Kathmandu, Nepal.
Radiol Case Rep. 2022 Jul 6;17(9):3432-3435. doi: 10.1016/j.radcr.2022.06.017. eCollection 2022 Sep.
Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) also known as Bland-White-Garland Syndrome is a rare anomaly of coronary arteries comprising of 0.25%-0.5% of all congenital heart defects with a prevalence of 1 in every 300,000 live births. Its clinical significance lies in the possibility of resultant coronary steal phenomenon with a left-to-right shunt causing aberrant left ventricular perfusion which may ultimately lead to myocardial ischemia and infarction in children having the abnormality. ALCAPA may manifest as an isolated defect but in 5% of cases it may be associated with other cardiac anomalies such as atrial septal defect, ventricular septal defect, and aortic coarctation. We present a case of 7 years female with ALCAPA with collaterals between RCA and LCA and additional findings of juxtaposition of left atrial appendage. Juxtaposition of atrial appendage is associated with some major congenital heart diseases, transposition of great vessels being the common one. In our case, however, juxtaposition of left atrial appendage is associated with ALCAPA. Surgery is the definite treatment modality for ALCAPA available till date. Early diagnosis of ALCAPA with the help of multislice CT angiography is always good for the patient to prevent the possible grave consequences.
左冠状动脉起源于肺动脉（ALCAPA），又称布兰德-怀特-加兰综合征，是一种罕见的冠状动脉异常，占所有先天性心脏病的0.25%-0.5%，每30万活产婴儿中约有1例发病。其临床意义在于可能导致冠状动脉窃血现象，出现左向右分流，引起左心室灌注异常，最终可能导致患有该异常的儿童发生心肌缺血和梗死。ALCAPA可能表现为孤立性缺陷，但在5%的病例中可能与其他心脏异常有关，如房间隔缺损、室间隔缺损和主动脉缩窄。我们报告一例7岁女性患有ALCAPA，右冠状动脉和左冠状动脉之间有侧支循环，并有左心耳并列的额外发现。心耳并列与一些主要的先天性心脏病有关，最常见的是大动脉转位。然而，在我们的病例中，左心耳并列与ALCAPA有关。手术是迄今为止治疗ALCAPA的明确治疗方式。借助多层CT血管造影早期诊断ALCAPA对患者总是有益的，可预防可能的严重后果。
