Abstract: Background: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a rare but lifethreatening congenital heart disease which can lead to changes in left ventricular configuration and affect left ventricular function. Therefore, it's necessary to evaluate the left ventricular configuration of the patients with ALCAPA before and after the surgical operation. Objective: To evaluate the changes of left ventricular configuration in patients with ALCAPA before and after the operation by echocardiographic spherical index (SI). Design: Casecontrol study. Methods: Consecutive simple infantile ALCAPA patients diagnosed by echocardiography were enrolled who underwent left coronary artery transplantation and received echocardiography 1 week before and after the operation. Children receiving echocardiography because of the physical examination were taken as the normal controls. The pre and postoperative ALCAPA patients and the normal control group were measured by SI and left ventricular ejection fraction (LVEF) derived by echocardiography to evaluate the left ventricular configuration and left ventricular systolic function. Main outcome measures: Changes of left ventricular configuration before and after ALCAPA operation. Results: From February 2006 to October 2021, a total of 15 patients with simple infantile ALCAPA aged 7.5±6.6 months were included. In the group of ALCAPA patients, the preoperative and postoperative left ventricular SId was 1.22±0.11 and 1.42±0.15, and the preoperative and postoperative left ventricular SIs was 1.35±0.17 and 1.54±0.17, respectively. In the control group with the average age of 7.4±6.6 months for 15 patients, the left ventricular SId and SIs was 1.49±0.15 and 1.55±0.15, respectively. In the group of ALCAPA patients, the preoperative and postoperative LVEF was (44.7±13.8)% and (63.9±13.8)%（P<0.01）. There was a correlation between left ventricular SId, SIs and LVEF before the operation (P＞0.01). Conclusion: SI derived by echocardiography can be used to evaluate the changes of left ventricular configuration in patients with ALCAPA before and after the operation. In patients with ALCAPA, the left ventricular spheroidization was distinct before the operation, and the left ventricular remodeling and configuration were improved after operation.

Key words: Anomalous left coronary artery from pulmonary artery, Echocardiography, Spherical index (SI), Left ventricular configuration