Bornemeier Renee A, Eble Brian K, Jaquiss Robert D B, Imamura Michiaki
Department of Pediatrics, University of Arkansas for Medical Sciences, Division of Cardiology, Little Rock, Arkansas Children's Hospital, Little Rock, AR, USA.
Congenit Heart Dis. 2008 Nov-Dec;3(6):435-9. doi: 10.1111/j.1747-0803.2008.00216.x.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. It is typically diagnosed at a few months of age. The infant presents with cardiac failure secondary to left ventricular dysfunction from the coronary steal that develops as pulmonary vascular resistance (PVR) falls. With the diagnosis made, surgical repair is typically performed expeditiously in order to try and restore left ventricular perfusion and stop the ongoing coronary steal. We present an unusual case of a preterm infant undergoing a routine echocardiogram, who was incidentally found to have an ALCAPA. Management strategies between neonatology and cardiology may differ in this setting. In this case, elevated PVR helps to preserve myocardial perfusion prior to surgical repair. Therefore, common neonatal management strategies typically utilized for respiratory distress, elevated PVR, and cardiac dysfunction may have potential for detrimental effects on myocardial perfusion in this specific lesion. This case also emphasizes the importance of identifying the coronary origins as a routine part of a complete pediatric echocardiogram. It also presents a rarely encountered problem: when to repair an ALCAPA in an as yet asymptomatic patient. We believe this to be the youngest and smallest patient reported with the diagnosis and successful surgical correction of an ALCAPA.
左冠状动脉起源于肺动脉（ALCAPA）是一种罕见的先天性心脏缺陷。通常在几个月大时被诊断出来。由于随着肺血管阻力（PVR）下降而发生的冠状动脉窃血，婴儿会出现继发于左心室功能障碍的心力衰竭。一旦确诊，通常会迅速进行手术修复，以尝试恢复左心室灌注并停止持续的冠状动脉窃血。我们报告了一例接受常规超声心动图检查的早产儿的不寻常病例，该婴儿偶然被发现患有ALCAPA。在这种情况下，新生儿科和心脏科之间的管理策略可能会有所不同。在本病例中，升高的PVR有助于在手术修复前维持心肌灌注。因此，通常用于治疗呼吸窘迫、PVR升高和心脏功能障碍的常见新生儿管理策略可能会对这种特定病变的心肌灌注产生潜在的有害影响。本病例还强调了将冠状动脉起源的识别作为完整儿科超声心动图常规部分的重要性。它还提出了一个很少遇到的问题：何时对尚无症状的患者进行ALCAPA修复。我们认为这是报告中诊断并成功进行手术矫正ALCAPA的最年轻、最小的患者。
