Ferreras Cristina, Mota Sílvia, Sarmento João Antunes, Granja Sofia, Grilo Marta, Ribeiro Augusto
Department of Pediatrics, Centro Hospitalar Universitário de São João, Porto, Portugal.
Department of Pediatrics, Hospital de Braga, Braga, Portugal.
Int J Pediatr Adolesc Med. 2021 Dec;8(4):271-272. doi: 10.1016/j.ijpam.2021.04.001. Epub 2021 Apr 23.
ALCAPA syndrome is a rare congenital heart disease and a cause of myocardial ischemia in pediatric population. The authors present the case of a 10-year-old girl admitted to the emergency room after experiencing cardiac arrest at school. In the echocardiogram, the inability to identify the origin of the left coronary artery raised the hypothesis of abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA). A CT-scan and a cardiac catheterization were performed confirming the diagnosis. Infants with the syndrome may have myocardial infarction and congestive heart failure She successfully underwent cardiac surgery. Most undiagnosed patients die within the first year of life, and it is necessary to develop an extensive network of collaterals to ensure survival. ALCAPA syndrome rarely manifests in late childhood, teenagers, and adults and may be an important cause of sudden cardiac arrest.
左冠状动脉起源于肺动脉综合征（ALCAPA综合征）是一种罕见的先天性心脏病，是小儿群体中心肌缺血的一个病因。作者介绍了一名10岁女孩的病例，该女孩在学校发生心脏骤停后被送往急诊室。在超声心动图检查中，无法确定左冠状动脉的起源，这引发了左冠状动脉起源于肺动脉异常（ALCAPA）的假说。随后进行了CT扫描和心导管检查，确诊了该疾病。患有该综合征的婴儿可能会出现心肌梗死和充血性心力衰竭。她成功接受了心脏手术。大多数未被诊断出的患者在出生后第一年内死亡，并且需要形成广泛的侧支循环网络以确保存活。ALCAPA综合征在儿童晚期、青少年和成人中很少出现，可能是心脏骤停的一个重要原因。
