ObjectiveTo explore surgical methods and risk factors of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). MethodsClinical data of 28 ALCAPA patients who underwent surgical repair from October 1993 to September 2013 in Beijing Anzhen Hospital were retrospectively reviewed. There were 8 male and 20 female patients with their age of 0.6-l6.8 (4.3±0.7)years including 10 patients less than 1 years old. Surgical procedures included simple ligation of left coronary artery, intrapulmonary tunnel procedure (Takeuchi)and direct coronary reimplantation of the anomalous artery. Postoperative death, complication and cardiac function were observed. ResultsAmong the 28 patients, 1 patient received simple ligation of left coronary artery, and 7 patients received intrapulmonary tunnel procedure (Takeuchi), among whom 2 patients died postoperatively. Twenty patients received direct implantation of the anomalous artery into the ascending aorta, and 3 patients died postoperatively. Five patients who died postoperatively were 10.20±3.27 months old, including 3 patients with moderate mitral regurgitation (MR)and 2 patients with mild MR preoperatively. Preoperative heart function of the patients who died postoperatively was significantly reduced. Preoperative left ventricular ejection fraction of the patients who died postoperatively was significantly lower than that of the patients who survived (36.6%±8.5% vs. 60.9%±10.7%, P=0.000). Low cardiac output syndrome was the reason for all postoperative death. All survival patients were followed up from 1 month to 18 years. One patient who underwent intra-pulmonary tunnel procedure (Takeuchi)received pulmonary artery balloon dilatation for pulmonary supravalvular stenosis 15 years after discharge. None of the other patients received a secondary operation. During follow-up, left ventricular function was improved. Growth and development of all the patients was normal. MR did not significantly aggravate in all the patients. ConclusionPatients with younger age and worse left ventricular function have greater surgical risks of ALCAPA.
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.