ObjectiveTo summarize the surgical experience of patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) and intramural left coronary artery and analyze the early and mid-term clinical outcomes. Methods The infants with ALCAPA and intramural left coronary artery who underwent surgical treatment in Fuwai Hospital from January 2014 to September 2020 were retrospectively enrolled, and the clinical data of the patients were analyzed. Results A total of 10 patients were included. There were 8 males and 2 females, with a median age of 7.5 (3-46) months at surgery. The surgical techniques included coronary unroofing in 7 patients, coronary unroofing with coronary reimplantation in 2 patients, and coronary unroofing with ligation of left coronary artery ostium in 1 patient. Seven infants received additional procedures, including 5 mitral valve repair. Delayed chest closure was required in 2 infants, and no operative death or major complications occurred in the whole group. Postoperative chest radiograph showed that the mean cardiothoracic ratio was lower than that before surgery (0.62±0.05 vs. 0.67±0.06, P=0.006). Postoperative echocardiography indicated that the mean left ventricular ejection fraction was increased than that before surgery, but there was no statistical difference (38.7%±15.9% vs. 30.0%±16.1%, P=0.066). The follow-up was available for all 10 survivors, with an average follow-up time of 13-92 (46.6±25.0) months. During the follow-up period, the patients had no obvious symptoms, death, coronary complications or other major complications. The chest radiograph at last follow-up showed that the mean cardiothoracic ratio was further decreased (0.60±0.07 vs. 0.62±0.05, P=0.024). The echocardiography at last follow-up showed that the mean left ventricular ejection fraction was further improved (60.1%±9.3% vs. 38.7%±15.9%, P=0.002). Conclusion ALCAPA with intramural left coronary artery is a rare malformation. It can be treated with different surgical techniques with satisfactory early and mid-term outcomes.
