Li Xin, An Jun, Wang Shuai, Lu Wanli, Liu Zhigang, Wu Yili, Jiao Fengjuan
Department of Pediatrics, TEDA International Cardiovascular Hospital, TEDA, Tianjin, China.
Department of Pediatric Cardiac Surgery, TEDA International Cardiovascular Hospital, TEDA, Tianjin, China.
Front Pediatr. 2020 Feb 20;8:51. doi: 10.3389/fped.2020.00051. eCollection 2020.
Isolated congenital coronary artery fistula (ICCAF) is an exceedingly rare anomaly in which there is a direct abnormal connection between a coronary artery and other cardiac chambers or any of great vessels. The left circumflex artery (LCX) is the least common source of ICCAF. Here we reported a rare case of large ICCAF originated from the LCX in a 9-year-old boy. He presented fatigability, murmurs and NYHA class II. Echocardiography and cardiac CT revealed that an aneurysmal dilatation of the LCX along with the dilated coronary sinus entered into the right atrium (RA) through the great cardiac vein. However, it showed that the dilated LCX directly drained into the RA by coronary angiography, which was confirmed by the surgery. During the surgical procedure, the LCX fistula was identified in a 33 cm bulbous structure, the aneurysmal dilation of RA tissue. The end of fistula was located in the lower-middle interatrial septum, which was near the coronary sinus and above the opening of inferior vena cava (IVC). Transcardiac chamber closure with cardiopulmonary bypass (CPB) was successfully performed for the correction of the fistula. It indicated that preoperative angiography is essential to define the details of large ICCAF with aneurysmal dilation. Moreover, transcardiac chamber closure with CPB is the optimal procedure for the treatment of large ICCAF, while interventional catheterization is not feasible due to the presence of aneurysmal dilation of the LCX. The description of this rare case might have great value for the diagnosis and treatment of large ICCAF originated from the LCX.
孤立性先天性冠状动脉瘘（ICCAF）是一种极为罕见的异常情况，即冠状动脉与其他心腔或任何大血管之间存在直接的异常连接。左旋支动脉（LCX）是ICCAF最不常见的起源部位。在此，我们报告了一例罕见的源自左旋支动脉的大型ICCAF病例，患者为一名9岁男孩。他表现出疲劳、杂音，纽约心脏协会（NYHA）心功能分级为II级。超声心动图和心脏CT显示，左旋支动脉呈瘤样扩张，扩张的冠状窦经心大静脉进入右心房（RA）。然而，冠状动脉造影显示扩张的左旋支动脉直接引流至右心房，手术证实了这一点。在手术过程中，在一个33厘米的球囊状结构（右心房组织的瘤样扩张）中发现了左旋支动脉瘘。瘘管末端位于房间隔中下部位，靠近冠状窦且在下腔静脉（IVC）开口上方。通过体外循环（CPB）成功实施经心腔闭合术以矫正瘘管。这表明术前血管造影对于明确伴有瘤样扩张的大型ICCAF的细节至关重要。此外，对于大型ICCAF的治疗，经心腔CPB闭合术是最佳术式，而由于左旋支动脉存在瘤样扩张，介入导管治疗不可行。对这一罕见病例的描述可能对源自左旋支动脉的大型ICCAF的诊断和治疗具有重要价值。
