Department of Cardiovascular Surgery, the First Affiliated Hospital, College of Medicine, Zhejiang University, Postal Address: 79#, Qingchun Road, Hangzhou, 310000, Zhejiang, China.
J Cardiothorac Surg. 2021 Jan 6;16(1):3. doi: 10.1186/s13019-020-01374-x.
Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital heart disease affecting about 0.002% of the population. Knowledge of ARCAPA is almost collected from case reports. The aim of this study was to provide a rare case to better understand this rare congenital coronary anomaly.
We report a rare case of an 18-year-old male who was initially referred because of heart murmur. Dilated and tortuous coronary arteries were detected by echocardiography and congenital coronary anomaly was suggested. Further coronary CT angiography confirmed the diagnosis of ARCAPA. Although dual coronary system provides favorable long-term outcome, bypass surgery was considered technically difficult due to the huge mismatch of caliber between the right coronary artery and graft vessels. Eventually, simple right coronary artery ligation was performed. The patient was followed up for about 5 years without evidence of atherosclerosis or myocardial ischemia.
ARCAPA presents as a rare congenital heart disease with variable clinical manifestations. Surgical treatment is highly recommended to re-establish dual coronary system and prevent further complications. To our best knowledge, only about 200 cases of ARCAPA has been reported.
右冠状动脉起自肺动脉（ARCAPA）是一种罕见的先天性心脏病，影响约 0.002%的人群。对 ARCAPA 的认识几乎都是从病例报告中收集的。本研究的目的是提供一个罕见的病例，以更好地了解这种罕见的先天性冠状动脉异常。
我们报告了一例罕见的 18 岁男性病例，最初因心脏杂音就诊。超声心动图检测到扩张和扭曲的冠状动脉，并提示存在先天性冠状动脉异常。进一步的冠状动脉 CT 血管造影证实了 ARCAPA 的诊断。尽管双冠状动脉系统提供了良好的长期预后，但由于右冠状动脉和移植血管之间的口径差异巨大，旁路手术被认为技术上困难。最终，仅进行了简单的右冠状动脉结扎术。该患者随访约 5 年，无动脉粥样硬化或心肌缺血证据。
ARCAPA 表现为一种罕见的先天性心脏病，临床表现多样。强烈建议进行手术治疗，以重建双冠状动脉系统并预防进一步的并发症。据我们所知，仅有约 200 例 ARCAPA 病例报告。
