Department of Surgery, University of California Davis, Sacramento, California; Department of Cardiothoracic Surgery, David Grant Medical Center, Travis Air Force Base, California.
Department of Surgery, University of California Davis, Sacramento, California; Department of Cardiothoracic Surgery, David Grant Medical Center, Travis Air Force Base, California.
Ann Thorac Surg. 2020 Sep;110(3):1063-1071. doi: 10.1016/j.athoracsur.2020.01.082. Epub 2020 Mar 7.
Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital cardiac lesion that has been diagnosed in both children and adults with symptoms ranging from an asymptomatic murmur to sudden cardiac death. The aim of this review was to characterize published cases of ARCAPA to better understand this rare congenital coronary anomaly.
A systematic review was performed using PubMed, Embase, and Google Scholar for cases of ARCAPA. Keywords searched included: "anomalous origin of the right coronary artery from the pulmonary artery" and "ARCAPA."
A total of 223 cases of ARCAPA were identified in 193 case reports. There was a slight male predominance (54.5%) and the median age at presentation was 14.0 years. Thirty-eight percent of patients were asymptomatic and most commonly identified during evaluation of a murmur. Angina and dyspnea were the most common presenting symptoms (22.4% and 17.0%, respectively). In symptomatic patients, a bimodal distribution of age at presentation was observed with a peak near birth and another between ages 40 and 60 years. The condition was most commonly diagnosed with coronary angiography (40.4%). Most cases were repaired surgically (72.6%) and reimplantation of the right coronary artery onto the aorta was the most common method of repair (62.3%).
ARCAPA represents a rare coronary anomaly with great variability in clinical presentation. An understanding of the pathophysiology associated with the lesion is critical when determining treatment strategies.
右冠状动脉起自肺动脉（ARCAPA）是一种罕见的先天性心脏病，可发生于儿童和成人，其临床表现从无症状杂音到心脏性猝死不等。本综述的目的是对 ARCAPA 的病例进行特征描述，以更好地了解这种罕见的先天性冠状动脉异常。
使用 PubMed、Embase 和 Google Scholar 对 ARCAPA 病例进行了系统回顾。搜索的关键词包括：“右冠状动脉起自肺动脉”和“ARCAPA”。
共在 193 例病例报告中确定了 223 例 ARCAPA。男性略占优势（54.5%），发病时的中位年龄为 14.0 岁。38%的患者无症状，最常见于听诊杂音时发现。胸痛和呼吸困难是最常见的症状（分别为 22.4%和 17.0%）。在有症状的患者中，观察到发病年龄呈双峰分布，一个高峰出现在出生前后，另一个高峰出现在 40 至 60 岁之间。最常见的诊断方法是冠状动脉造影（40.4%）。大多数病例采用手术修复（72.6%），最常见的修复方法是将右冠状动脉重新植入主动脉（62.3%）。
ARCAPA 是一种罕见的冠状动脉异常，临床表现差异很大。了解与病变相关的病理生理学对于确定治疗策略至关重要。
