Ganta Srujan, Vanderploeg Megan, Kavarana Minoo
1 Division of Cardiothoracic Surgery, Department of Surgery, Medical University of South Carolina Children's Hospital, Charleston, SC, USA.
World J Pediatr Congenit Heart Surg. 2019 Mar;10(2):192-196. doi: 10.1177/2150135118822472.
Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is rare. Unique anatomical characteristics observed include tethering secondary to the extensive collateral vessels, severe native coronary tortuosity, and massive dilation of the coronary arteries. This requires specific technical consideration to ensure safe translocation.
A single-center retrospective review of six patients with ARCAPA was performed. Echocardiographic and computerized tomography scan data were analyzed for anatomical and functional cardiac characteristics. Operative techniques were analyzed, which reflected an evolution toward a modified-trapdoor technique.
Five children presented with asymptomatic murmurs and one adult patient with unstable angina. All patients underwent successful surgical correction. The modified trapdoor technique provided the most ideal geometry for coronary transfer secondary to its anatomical characteristics. Two patients had coronary button transfers above the sinotubular junction using vertical stab incisions, one had the button implanted after excising part of the aortic wall, and last three patients had modified trapdoor incisions. Mean cardiopulmonary bypass and cross-clamp times were 170 ± 27 minutes and 99.5 ± 29 minutes respectively. The average hospital stay was five days and there were no mortalities.
Anomalous right coronary from the pulmonary artery's unique anatomical characteristics require a coronary transfer technique different from that performed in aortic root replacement. In some respects, our modified technique resembles coronary transfers used in difficult arterial switch operations. The use of a modified trapdoor incision simplifies coronary transfer and may minimize coronary kinking and subsequent complications related to coronary transfer.
右冠状动脉起源于肺动脉（ARCAPA）较为罕见。观察到的独特解剖特征包括因广泛侧支血管导致的牵拉、严重的固有冠状动脉迂曲以及冠状动脉的大量扩张。这需要特定的技术考量以确保安全移位。
对6例ARCAPA患者进行单中心回顾性研究。分析超声心动图和计算机断层扫描数据以了解心脏的解剖和功能特征。分析手术技术，其反映了向改良活板门技术的演变。
5名儿童表现为无症状杂音，1名成年患者表现为不稳定型心绞痛。所有患者均成功接受手术矫正。改良活板门技术因其解剖特征为冠状动脉移位提供了最理想的几何形状。2例患者使用垂直刺状切口在窦管交界处上方进行冠状动脉纽扣移位，1例在切除部分主动脉壁后植入纽扣，最后3例患者采用改良活板门切口。平均体外循环和主动脉阻断时间分别为170±27分钟和99.5±29分钟。平均住院时间为5天，无死亡病例。
肺动脉起源的异常右冠状动脉的独特解剖特征需要一种不同于主动脉根部置换术的冠状动脉移位技术。在某些方面，我们的改良技术类似于困难动脉调转手术中使用的冠状动脉移位。改良活板门切口的使用简化了冠状动脉移位，并可能使冠状动脉扭结及随后与冠状动脉移位相关的并发症降至最低。
