Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) has a low incidence and is very rare in clinical practice. Due to the pathophysiological characteristics of this disorder, approximately 90% of patients die naturally in infancy. In the early years, the treatment effect was poor in China due to the lack of knowledge and experience in the treatment of this condition. In recent years, with the rapid development of the level of technology in the diagnosis and treatment of precocious heart disease in China, the success rate of the treatment of this disease in major surgical centers for precocious heart disease has also increased significantly. In our hospital, 21 cases were treated surgically from 1993 to 2008. In the last 3 years, 5 cases were treated by direct coronary artery grafting method, which are reported as follows: Clinical data: 5 cases were treated by direct coronary artery grafting method from October 2006 to October 2008. The diagnosis was determined mainly by electrocardiography, echocardiography and multi-row CT, and one case was treated by coronary angiography. In three cases, the opening of the left coronary artery was seen in the left posterior sinus of the pulmonary artery; in case 3, the opening was in the right posterior sinus of the main pulmonary artery; and in case 5, the opening was in the right upper side near the bifurcation of the pulmonary artery, and it emanated about 3 mm and then traveled inside the left wall of the aorta and then wrapped around to the left behind the pulmonary artery. All of them were grafted directly to the left side of the aorta. The aorta was incised in a “U” shape, forming a portal flap. In case 1, the anastomosis was repaired with a Gore-Tex vascular sheet due to high anterior wall tension. The duration of extracorporeal circulation was 113 C 267 minutes, and the duration of myocardial blood flow block was 72 C 146 minutes. RESULTS: All patients had automatic cardiac resumption after open aortic block clamp with no electrocardiographic or clinical manifestations of myocardial ischemia. Case 3 died 8 days postoperatively due to anastomotic bleeding eventually leading to multiple organ failure. The four surviving cases were followed up for 26 months, and all patients had coronary artery flow patency. DISCUSSION: With the development of surgery for precordial disease, there have been different surgical approaches to the treatment of ALCAPA throughout history. The earliest surgical treatment was to create a main, pulmonary artery anastomosis to increase pulmonary blood flow and improve oxygen saturation in the left coronary artery. Thereafter, simple ligation of ALCAPA was adopted. mustard had utilized an end-to-end anastomosis of the left common carotid artery to the left coronary artery, which allowed for a more physiological outcome. in 1966, Cooley used the saphenous vein graft method to create a double coronary artery system repair. Subsequently, Meyer et al. reported the use of an anastomosis of the left subclavian artery to the beginning of the ALCAPA. 1974, Neches et al. first described a surgical technique to re-graft a left coronary artery with an anomalous origin in the wall of the pulmonary artery to the aorta. Takeuchi reported in 1979 that when direct coronary grafting was difficult, the use of tunneling first in the pulmonary artery and then to the aortic artery, good results have also been achieved. Left coronary artery ligation alone is only suitable for those with abundant collateral circulation, and the early and late mortality rates are not low; the long-term results of Takeuchi’s procedure are not yet stable, with problems of coronary stenosis, suprapulmonary stenosis and baffle leakage; left subclavian artery, saphenous vein and internal mammary artery bypass grafting also have problems of long-term stenosis and reduced patency rate. Currently, most scholars advocate the use of a dual coronary artery system with intact arteries for the treatment of this condition, and there are several coronary grafting techniques. We have used direct ALCAPA grafting to the aorta with good clinical results. For mitral regurgitation, most scholars believe that simultaneous repair during surgery is not necessary and can be gradually restored to normal after surgery. However, in our case 4, mitral regurgitation was worse than preoperative at 8 months of postoperative follow-up. We believe that simultaneous surgical repair of mitral regurgitation above moderate should be considered, which also facilitates a smooth perioperative recovery. There are few reports in the literature on the surgical treatment of infants and children with this condition combined with left ventricular wall tumors. We have performed surgical resection in a young child with a large combined left ventricular wall tumor with good postoperative results.