Analysis of relevant factors associated with post-operative recovery after anomalous origin of the left coronary artery from the pulmonary artery surgery in children

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Methods Patients with ALCAPA admitted from January 2015 to December 2024 were retrospectively reviewed. Clinical data of the patients were retrieved from the record. The follow-up data include mortality rate and complications. The Kaplan-Meier survival curve was used to analyze the occurrence of death and reoperation during the follow-up of the patients with ALCAPA, and the Log-rank test was employed for comparison between groups. The Cox regression model was used to analyze the risk factors for adverse events in patients with unfavourable prognosis. Results Of 48 patients meeting the study criteria. The post-operative death or reoperation of the patients are defined as unfavourable prognosis. Patients are divided into two groups: the favourable prognosis (n = 41) and the unfavourable prognosis (n = 7). The differences among Kaplan-Meier survival curves for different subgroups were statistically significant. Patients with feeding difficulties ( p 4 ( p = 0.05), pre-operative mechanical ventilation ( p < 0.001), pre-operative vasoactive drugs ( p < 0.001), ECMO ( p < 0.001) and delayed sternal closure ( p < 0.001) are more likely to have an unfavorable prognosis. On multivariate Cox proportional hazard analysis, the age at surgical < 1y (HR 16.521, 95%CI: 1.084, 251.718, p = 0.044), pre-operative LVEF (HR: 0.708, 95%CI: 0.536, 0.937, p = 0.016), CPB time (HR: 1.024, 95%CI: 1.006, 1.042, p = 0.01) and length of stay (HR: 1.074, 95%CI: 1.02, 1.132, p = 0.007) may have a higher risk of unfavourable prognosis for patients. Conclusions Younger surgical age, reduced pre-operative LVEF, longer CPB time and length of stay are the key risk factors that affect the unfavourable prognosis of patients. ALCAPA children the unfavourable prognosis Figures Figure 1 Figure 2 Figure 3 Introduction Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly of coronary artery anatomy, with an incidence rate of 1 in 300,000 [ 1 ] . As pulmonary vascular resistance decreases during the first few weeks after birth, myocardial ischemia and/or infarction may occur. ALCAPA causes severe myocardial ischemia, overall left ventricular dysfunction, and dilation of the mitral valve annulus, resulting in varying degrees of mitral regurgitation (MR) [ 2 ] . ALCAPA is usually diagnosed in infancy, but adults may also be affected by this deformity, although it is extremely rare. Most infants who do not undergo surgical treatment die within one year, with a mortality rate varies from 35–85% [ 3 ] . Some patients may show no symptoms, while others may present with manifestations such as left ventricular dysfunction, malignant arrhythmias, or sudden cardiac death [ 4 ] . Therefore, early diagnosis and surgical can significantly reduce the mortality rate. Previous studies have reported that a low pre-operative left ventricular ejection fraction (LVEF), age at surgery, and severe pre-operative MR are risk factors for post-operative mortality after ALCAPA surgery [ 5 ] . This study conducted long-term follow-up on patients with ALCAPA, aiming to analyze the factors influencing the prognosis in children and to provide a strong basis for clinical treatment decisions and prognosis assessment. Method Study population Patients with ALCAPA admitted to Guangzhou Women and Children’s Medical Center, Guangzhou Medical University from January 2015 to December 2024 were retrospectively reviewed. The inclusion criteria were: age < 18 years, diagnosed with ALCAPA through echocardiography and enhanced cardiac CT, and exclusion of cardiomyopathy, complex congenital heart disease, and other coronary artery origin abnormalities. Clinical data collection Clinical data of the patients were retrieved from the record, including sex, weight Z-score, symptoms and signs, age of surgery, perioperative conditions, whether it was a second surgery, prognosis, and follow-up data after discharge. The follow-up data include mortality rate and complications. The echocardiographic parameters include the LVEF, the degree of MR and left ventricular end diastolic diameter (LVEDD). Z-scores were extrapolated based on age and body surface area [ 6 ] . Ethical approval for the present study was granted by the Ethics Committee of Guangzhou Women and Children’s Medical Center, Guangzhou Medical University (No.212A01), and the parents of the patients all signed the informed consent form. Statistical analysis All statistical analyses were performed using SPSS software, version 29.0 for windows. Graphpad Prism 10 was used for plotting. Continuous variables are reported as mean ± SD or median and interquartile range, according to normal or nonnormal distribution as per the Shapiro-Wilk normality test. Groups were compared by use of the unpaired Student t test when normally distribution, whereas the Mann-Whitney U test was applied to those with non-normal distribution. The Wilcoxon matched-paired signed-rank test was used to analyze paired data at different time points. Categorical variables were compared via the Fisher exact test. The Kaplan-Meier survival curve was used to analyze the occurrence of death and reoperation during the follow-up of the patients with ALCAPA, and the Log-rank test was employed for comparison between groups. The Cox regression model was used to analyze the risk factors for adverse events in patients with unfavourable prognosis. A two-tailed P-value of less than 0.05 was considered statistically significant. Results Patients baseline data Of 48 patients meeting the study criteria, the median age at surgery was 5 months (range 0–13 years old), median weight Z-score was − 1.7 ± 1.2. Among all patients, 4 patients were accompanied by other cardiac malformations, including ventricular septal defect, tetralogy of Fallot, transitional atrioventricular septal defect, and aortic coarctation. All the patients underwent left coronary artery reimplantation, and among them, 24 patients underwent mitral valvuloplasty simultaneously. The post-operative death or reoperation of the patients are defined as unfavourable prognosis. Patients are divided into two groups: the favourable prognosis (n = 41) and the unfavourable prognosis (n = 7). The baseline characteristics are described in Table 1 . Patients in the unfavourable prognosis group were more likely to have feeding difficulties ( p = 0.008). Their pre-operative LVEF ( p < 0.001) and pre-operative LVEDD Z-score ( p = 0.011) were also significantly lower than those in the favourable prognosis group. The pre-operative mechanical ventilation and vasoactive drugs in the unfavourable prognosis group was significantly more commonly used than that in the favourable prognosis group ( p = 0.018). Table 1 Demographic characteristics and perioperative data with ALCAPA Favourable prognosis group (n = 41) Unfavourable prognosis group (n = 7) P Demographic data, n (%) Sex 0.412 Male 15 (36.6) 4 (57.1) Female 26(63.4) 3(42.9) Weight Z-score -1.7 ± 1.0 -1.54 ± 1.55 0.707 Age at surgery, months 5(3.5, 33) 5(2, 13) 0.509 Presenting symptoms, n (%) Shortness of breath 24(58.5) 3(42.9) 0.683 Feeding difficulties 1(2.4) 1(6.3) 0.008* Growth redardation 11(26.8) 1(14.3) 0.662 Pre-operative NT-proBNP, pg/mL 4979 (353.5, 21303.5) 33739 (26355, 35000) 0.013* Electrocardiograph, at admission, n (%) Abnormal Q wave 7(17.1) 1(14.3) 1.0 ST-T change 19(46.3) 3(42.9) 1.0 T-wave change 13(31.7) 3(42.9) 0.672 Pre-operative echocardiogram, n (%) LVEF, % 39 (30.5, 59.5) 26 (20, 28) < 0.001* Moderate-severe MR 23 (56.1) 6 (85.7) 0.219 LVEDD Z-score 4.22 ± 2.28 6.58 ± 1.18 0.011* Pre-operative mechanical ventilation, n (%) 2 (4.9) 3 (42.9) 0.018* Pre-operative vasoactive drugs, n (%) 2 (4.9) 3 (42.9) 0.018* CPB time, min 140 (119.5, 169) 157 (121, 205) 0.267 Aortic cross-clamp time, min 68 (59.5, 86) 70 (60, 90) 0.599 MR repair, n (%) 19 (46.3) 4 (57.1) 0.696 Delayed sternal closure, n (%) 3 (7.3) 3 (42.9) 0.033* ECMO, n (%) 1 (2.4) 2 (28.6) 0.052 Length of stay, days 17(13, 21) 18(9, 49) 0.988 NT-proBNP: N-terminal pro-B-type natriuretic peptide; LVEF: Left ventricular ejection fraction; MR: Mitral regurgitation; LVEDD: Left ventricular end-diastolic diameter; CPB: Cardiopulmonary bypass; ECMO:Extracorporeal membrane oxygenation Mortality and complications Mortality of patients was 10.42% (5/48). 2 patients died of heart failure (respectively on the 9th and 49th days after surgery, among which there was one patient received repetitive mitral valvuloplasty due to severe MR). 2 died of malignant arrhythmia (respectively on the 7th and 18th days after surgery) and 1 died of respiratory failure due to a severe pulmonary infection (49 days after surgery). All of them were post-operative death. Survival analysis Follow-up was available for 43 patients, median follow-up was 687 days (range 1-2961 days), and there were no patients who died after discharge. Reoperation was required in 2 patients. Reasons for reoperation included MR re-repair for recurrent MR, and pulmonary artery balloon dilation for pulmonary artery trunk and right pulmonary artery stenosis. The estimated rates of freedom from unfavourable prognosis at 1, 3 and 5 years were 89.6%, 87.5%, and 87.5%, respectively (Fig. 1 ). Echocardiograms during the follow-up period showed that LVEF of the surviving patients with pre-operative low LVEF was completely restored. The median time to normalization of left ventricular function was 118 days (range 7-604 days) (Fig. 3 ). The differences among Kaplan-Meier survival curves for different subgroups were statistically significant (Fig. 2 A-F). Patients with feeding difficulties ( p 4 ( p = 0.05), pre-operative mechanical ventilation ( p < 0.001), pre-operative vasoactive drugs ( p < 0.001), ECMO ( p < 0.001) and delayed sternal closure ( p < 0.001) are more likely to have an unfavoural prognosis. Cox proportional hazard analyses Univariate and multivariate Cox proportional hazard analyses for independent risk factors for the prognosis are shown in Table 2 . On multivariate Cox proportional hazard analysis, the surgical age < 1 year (HR 16.521, 95%CI:1.084, 251.718, p = 0.044), pre-operative LVEF (HR: 0.708, 95%CI: 0.536, 0.937, p = 0.016), CPB time (HR: 1.024, 95%CI:1.006, 1.042, p = 0.01) and length of stay (HR: 1.074, 95%CI: 1.02, 1.132, p = 0.007) may have a higher risk of unfavourable prognosis for patients. Figure 2 shows the influence of variable on the unfavourable prognosis. Table 2 Cox proportional hazard analysis for factors for the prognosis among ALCAPA patients Risk factor Univariate Analysis Multivariate Analysis Hazzard Ratio (95% CI) P Hazzard Ratio (95% CI) P Sex 0.467 (0.104, 2.091) 0.32 Weight, Z-score 1.334 (0.632, 2.814) 0.45 Age at surgery 5000 pg/ml, n (%) 6.064 (0.726, 50.637) 0.096 Pre-operative mechanical ventilation, n (%) 16.252 (3.043, 86.789) 0.001 Pre-operative vasoactive drugs, n (%) 16.252 (3.043, 86.789) 0.001 Pre-operative LVEDD Z-score 1.813 (1.105, 2.975) 0.019 Pre-operative LVEF, n (%) 0.82 (0.721, 0.933) 0.003 0.708 (0.536, 0.937) 0.016 Pre-operative moderat-severe MR, n (%) 3.656 (0.436, 30.670) 0.232 Abnormal Q wave, n (%) 0.767 (0.092, 6.374) 0.806 ST-T change, n (%) 0.72 (0.153, 3.392) 0.678 T-wave change, n (%) 1.326 (0.295, 5.954) 0.712 CPB time, min 1.009 (1, 1.017) 0.038 1.024 (1.006. 1.042) 0.01 Aortic cross-clamp time, min 1.008 (0.982, 1.035) 0.548 Delayed sternal closure, n (%) 12.056 (2.34, 62.113) 0.003 ECMO, n (%) 15.849 (2.529, 99.31) 0.003 Length of stay, days 1.031 (1.003, 1.059) 0.027 1.074 (1.02., 1.132) 0.007 Discussion Fetuses with ALCAPA usually have no symptoms because the diastolic pressures of the pulmonary artery and aorta are similar during the prenatal circulation. However, after birth, when pulmonary vascular resistance begins to decline, most infants start to show clinical symptoms due to the retrograde flow in the left coronary artery, such as poor development, excessive sweating, breathing difficulties, pallor and feeding difficulties [ 7 ] . At the same time, coronary artery retrograde flow can lead to coronary steal, which further aggravate myocardial ischemia and thereby worsens symptoms. Prompt surgical correction of ALCAPA is required as soon as clinical diagnosis is confirmed, regardless of the patient's age and cardiac function. The anomalous coronary artery is reimplanted into the aorta to reconstruct the dual coronary artery [ 8 ] . This operation usually has a good prognosis [ 9 ] . The post-operative mortality rate ranges from 0–16% [ 10 , 11 ] , and 3% of patients require long-term re-interventions [ 1 ] . MR and pulmonary artery stenosis are the main reasons for reoperation surgery [ 1 , 7 ] .In this study, the post-operative mortality rate was 10.4% (5/48), and the rate of reoperation during the follow-up period was 4.7% (2/43), which are consistent with previous reports. Since the clinical manifestations of this disease are not specific, our study found that most patients presented with symptoms such as shortness of breath, feeding difficulties, and developmental delays. However, in the group with unfavourable prognosis, feeding difficulties was more common. It has been reported in the literature that younger patients are more likely to experience respiratory distress ( p = 0.04), while older patients are more likely to have chest pain as the initial symptom ( p = 0.004) [ 12 ] . In some centers, younger surgical age may also be associated with death, possibly due to insufficient development of coronary artery collateral vessels, or more severe ventricular ischemia or dysfunction, or both [ 13 ] . In the multivariate Cox regression analysis, it was found that age at surgical < 1 year was statistically significant for unfavourable prognosis of the patients.. During ALCAPA surgery, there has always been controversy over whether to simultaneously repair the mitral valve. Brown et al. [ 14 ] believe that patients with ALCAPA do not need to undergo mitral valve intervention simultaneously. However, Bicer et al. [ 15 ] pointed out that although the rate of mitral valve re-intervention is relatively low, it is still necessary to pay attention if MR exceeds moderate severity before the operation. For moderate and severe MR, the degree of damage to the mitral valve is very high. Simply correcting the abnormal coronary arteries and restoring the blood flow of the dual coronary arteries is not sufficient to restore the function of the mitral valve; mitral valve repair needs to be carried out simultaneously [ 1 ] . Early implementation of mitral valve repair can improve early post-operative cardiac output and facilitate the early recovery of cardiac function [ 16 , 17 ] . Although some literature has reported that MR is a risk factor for post-operative death [ 14 , 18 ] , the degree of MR did not predict any of our post-operative outcomes. In this study, 29 patients had moderate or severe MR before the operation. Among them, 24 patients underwent mitral valve repair simultaneously. Only 1 patient had severe MR due to gradually worsening mitral regurgitation 6 years after the operation and thus underwent MR repair again. 5 patients did not undergo mitral valve repair, 2 of them died, and 3 survived. During the follow-up period, MR recovered to a mild state in these 3 patients. In this studies, longer CBP time ( p = 0.01) and length of stay ( p = 0.007) were risk factors for unfavourable prognosis. This is the first report indicating that the longer CPB time and the length of stay may be related to the post-operative prognosis. Early mortality is significantly associated with left ventricular dysfunction. While advancements in surgical techniques and pharmacological therapies have mitigated the adverse effects on left ventricular function, numerous studies have demonstrated that pre-operative left ventricular dysfunction remains an independent predictor of early mortality [ 15 , 19 ] . This study corroborates these findings, showing that lower pre-operative LVEF values adversely affect patient prognosis. If there is no left ventricular dysfunction, the prognosis after the surgery will be favourable [ 20 ] . Kudumula's [ 21 ] reported in a retrospective study of infants and children with ALCAPA hat 88% of patients achieved left ventricular function recovery during long-term follow-up of 20 years. Existing literature reports a median left ventricular recovery duration of 4 months, with some cases requiring nearly one year for complete functional restoration [ 22 ] . Occasional prolonged recovery may indicate the gradual reversal of these adaptive cellular changes. Post-operative myocardial depression may sharply exacerbate cardiac dysfunction, that is, the persistent transient mechanical dysfunction that persists after reperfusion and restoration of normal coronary artery blood flow [ 18 ] . In this study, 5 patients died, all of whom presented with significantly reduced pre-operative LVEF. During the post-operative follow-up period, echocardiography showed that the left ventricular function of all the surviving patients, especially those with a low LVEF before the operation, had fully recovered. The median time to LVEF recovery was 181 days, and the longest was 604 days. Limitations The main limitations of this study include its retrospective design, single-center nature, and relatively small sample size. Additionally, the exclusive reliance on echocardiography for cardiac function assessment may not provide comprehensive evaluation of left ventricular contractility. Conclusions ALCAPA is a rare congenital anomaly of coronary artery anatomy. Early surgical intervention achieves satisfactory outcomes and improves the left ventricular function in survivors. Younger surgical age, reduced pre-operative LVEF, longer CPB time and length of stay are the key risk factors that affect the unfavourable prognosis of patients. Abbreviations ALCAPA Anomalous origin of the left coronary artery from the pulmonary artery MR Mitral regurgitation LVEF Left ventricular ejection fraction LVEDD Left ventricular end diastolic diameter NT-proBNP N-terminal pro-B-type natriuretic peptide CPB Cardiopulmonary bypass ECMO Extracorporeal membrane oxygenation Declarations Competing interests The authors declare no competing interests. Ethics approval and consent to participate This study was performed with the ethics approval from the Institutional Consent for publication Not applicable. Funding Guangzhou Major clinical technology project (2023C-ZD10). Guanghzou Science and Technology Bureau Project (2024A03J1165). The Basic and Applied Basic Research Project of Guangzhou Municiple Sccience and Technology Bureau (2025A03J4265) Author Contribution J Y was responsible for the writing of the article. Yanxing Lv responsible for the analyzed data. Xinyuan Ding performed the collected pre-operative data. L M responsible for the surgery. Hang Yang and Lin Jiang responsible for registering the post-operative follow-up data of patients. N Z designed the research study. ALL authors have read and approved the final version of this manuscript. Acknowledgements We would like to thank the Guangzhou Women and Children’s Medical Center, Guangzhou Medical University for proving support and guidance. The above opinions are those of the authors alone. Data availability The data used in this manuscript are available upon reasonable request by contacting the corresponding author via email. References Yu J, Ren Q, Liu X, et al. Anomalous left coronary artery from the pulmonary artery: Outcomes and management of mitral valve. Front Cardiovasc Med. 2022;9:953420. Gao Y, Zhang J, Huang GY, et al. Surgical outcomes of anomalous origin of the left coronary artery from the pulmonary artery in children: an echocardiography follow-up. Chin Med J-Peking. 2017;130(19):2333–8. 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Twenty-year outcome of anomalous origin of left coronary artery from pulmonary artery: Management of mitral regurgitation. Ann Thorac Surg. 2014;97(3):938–44. Hu RJ, Zhang W, Yu XF. Midterm Surgical Outcomes for ALCAPA Repair in Infants and Children. Thorac cardiov suger. 2022;70(1):2–9. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. 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1","display":"","copyAsset":false,"role":"figure","size":144640,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend\u003c/p\u003e","description":"","filename":"figure141.png","url":"https://assets-eu.researchsquare.com/files/rs-7360768/v1/0c8fac756e07fc3b6370bbcd.png"},{"id":94223632,"identity":"c60a7abf-d44e-4edc-a634-7f86fbcb57a1","added_by":"auto","created_at":"2025-10-23 19:09:03","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":449377,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend\u003c/p\u003e","description":"","filename":"figure143.png","url":"https://assets-eu.researchsquare.com/files/rs-7360768/v1/af4c09a14a6c0f9a27726686.png"},{"id":94223625,"identity":"3d55795c-7c2a-4e86-bb1f-b7ddb33f8ce2","added_by":"auto","created_at":"2025-10-23 19:09:03","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":67886,"visible":true,"origin":"","legend":"\u003cp\u003eSee image above for figure legend\u003c/p\u003e","description":"","filename":"figure144.png","url":"https://assets-eu.researchsquare.com/files/rs-7360768/v1/427da7a51b90198282403830.png"},{"id":94474916,"identity":"77ce30e5-6d16-4351-9e43-d39c7d075597","added_by":"auto","created_at":"2025-10-27 15:50:50","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1198234,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7360768/v1/a7960091-2a83-4e42-8904-6944db233b77.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Analysis of relevant factors associated with post-operative recovery after anomalous origin of the left coronary artery from the pulmonary artery surgery in children","fulltext":[{"header":"Introduction","content":"\u003cp\u003eAnomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly of coronary artery anatomy, with an incidence rate of 1 in 300,000\u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]\u003c/sup\u003e. As pulmonary vascular resistance decreases during the first few weeks after birth, myocardial ischemia and/or infarction may occur. ALCAPA causes severe myocardial ischemia, overall left ventricular dysfunction, and dilation of the mitral valve annulus, resulting in varying degrees of mitral regurgitation (MR)\u003csup\u003e[\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]\u003c/sup\u003e. ALCAPA is usually diagnosed in infancy, but adults may also be affected by this deformity, although it is extremely rare. Most infants who do not undergo surgical treatment die within one year, with a mortality rate varies from 35\u0026ndash;85%\u003csup\u003e[\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e]\u003c/sup\u003e. Some patients may show no symptoms, while others may present with manifestations such as left ventricular dysfunction, malignant arrhythmias, or sudden cardiac death\u003csup\u003e[\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]\u003c/sup\u003e. Therefore, early diagnosis and surgical can significantly reduce the mortality rate.\u003c/p\u003e\u003cp\u003ePrevious studies have reported that a low pre-operative left ventricular ejection fraction (LVEF), age at surgery, and severe pre-operative MR are risk factors for post-operative mortality after ALCAPA surgery\u003csup\u003e[\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]\u003c/sup\u003e. This study conducted long-term follow-up on patients with ALCAPA, aiming to analyze the factors influencing the prognosis in children and to provide a strong basis for clinical treatment decisions and prognosis assessment.\u003c/p\u003e"},{"header":"Method","content":"\u003cp\u003eStudy population\u003c/p\u003e\u003cp\u003e Patients with ALCAPA admitted to Guangzhou Women and Children\u0026rsquo;s Medical Center, Guangzhou Medical University from January 2015 to December 2024 were retrospectively reviewed. The inclusion criteria were: age\u0026thinsp;\u0026lt;\u0026thinsp;18 years, diagnosed with ALCAPA through echocardiography and enhanced cardiac CT, and exclusion of cardiomyopathy, complex congenital heart disease, and other coronary artery origin abnormalities.\u003c/p\u003e\u003cp\u003eClinical data collection\u003c/p\u003e\u003cp\u003eClinical data of the patients were retrieved from the record, including sex, weight Z-score, symptoms and signs, age of surgery, perioperative conditions, whether it was a second surgery, prognosis, and follow-up data after discharge. The follow-up data include mortality rate and complications. The echocardiographic parameters include the LVEF, the degree of MR and left ventricular end diastolic diameter (LVEDD). Z-scores were extrapolated based on age and body surface area\u003csup\u003e[\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]\u003c/sup\u003e. Ethical approval for the present study was granted by the Ethics Committee of Guangzhou Women and Children\u0026rsquo;s Medical Center, Guangzhou Medical University (No.212A01), and the parents of the patients all signed the informed consent form.\u003c/p\u003e\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\u003ch2\u003eStatistical analysis\u003c/h2\u003e\u003cp\u003eAll statistical analyses were performed using SPSS software, version 29.0 for windows. Graphpad Prism 10 was used for plotting. Continuous variables are reported as mean\u0026thinsp;\u0026plusmn;\u0026thinsp;SD or median and interquartile range, according to normal or nonnormal distribution as per the Shapiro-Wilk normality test. Groups were compared by use of the unpaired Student \u003cem\u003et\u003c/em\u003e test when normally distribution, whereas the Mann-Whitney \u003cem\u003eU\u003c/em\u003e test was applied to those with non-normal distribution. The Wilcoxon matched-paired signed-rank test was used to analyze paired data at different time points. Categorical variables were compared via the Fisher exact test. The Kaplan-Meier survival curve was used to analyze the occurrence of death and reoperation during the follow-up of the patients with ALCAPA, and the Log-rank test was employed for comparison between groups. The Cox regression model was used to analyze the risk factors for adverse events in patients with unfavourable prognosis. A two-tailed P-value of less than 0.05 was considered statistically significant.\u003c/p\u003e\u003c/div\u003e"},{"header":"Results","content":"\u003cp\u003ePatients baseline data\u003c/p\u003e\u003cp\u003eOf 48 patients meeting the study criteria, the median age at surgery was 5 months (range 0\u0026ndash;13 years old), median weight Z-score was \u0026minus;\u0026thinsp;1.7\u0026thinsp;\u0026plusmn;\u0026thinsp;1.2. Among all patients, 4 patients were accompanied by other cardiac malformations, including ventricular septal defect, tetralogy of Fallot, transitional atrioventricular septal defect, and aortic coarctation. All the patients underwent left coronary artery reimplantation, and among them, 24 patients underwent mitral valvuloplasty simultaneously. The post-operative death or reoperation of the patients are defined as unfavourable prognosis. Patients are divided into two groups: the favourable prognosis (n\u0026thinsp;=\u0026thinsp;41) and the unfavourable prognosis (n\u0026thinsp;=\u0026thinsp;7). The baseline characteristics are described in Table\u0026nbsp;\u003cspan refid=\"Tab1\" class=\"InternalRef\"\u003e1\u003c/span\u003e. Patients in the unfavourable prognosis group were more likely to have feeding difficulties (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.008). Their pre-operative LVEF (\u003cem\u003ep\u0026thinsp;\u0026lt;\u003c/em\u003e\u0026thinsp;0.001) and pre-operative LVEDD Z-score (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.011) were also significantly lower than those in the favourable prognosis group. The pre-operative mechanical ventilation and vasoactive drugs in the unfavourable prognosis group was significantly more commonly used than that in the favourable prognosis group (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.018).\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab1\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eDemographic characteristics and perioperative data with ALCAPA\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"4\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\"\u003e\u0026nbsp;\u003c/th\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eFavourable prognosis group (n\u0026thinsp;=\u0026thinsp;41)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003eUnfavourable prognosis group (n\u0026thinsp;=\u0026thinsp;7)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003e\u003cem\u003eP\u003c/em\u003e\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDemographic data, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eSex\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.412\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMale\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e15 (36.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e4 (57.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eFemale\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e26(63.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3(42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eWeight Z-score\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e-1.7\u0026thinsp;\u0026plusmn;\u0026thinsp;1.0\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e-1.54\u0026thinsp;\u0026plusmn;\u0026thinsp;1.55\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.707\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAge at surgery, months\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e5(3.5, 33)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e5(2, 13)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.509\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePresenting symptoms, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eShortness of breath\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e24(58.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3(42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.683\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eFeeding difficulties\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e1(2.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e1(6.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.008*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eGrowth redardation\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e11(26.8)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e1(14.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.662\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative NT-proBNP, pg/mL\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e4979 (353.5, 21303.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e33739 (26355, 35000)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.013*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eElectrocardiograph, at admission, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAbnormal Q wave\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e7(17.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e1(14.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1.0\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eST-T change\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e19(46.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3(42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1.0\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT-wave change\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e13(31.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3(42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.672\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative echocardiogram, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLVEF, %\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e39 (30.5, 59.5)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e26 (20, 28)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e\u0026lt;\u0026thinsp;0.001*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eModerate-severe MR\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e23 (56.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e6 (85.7)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.219\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLVEDD Z-score\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e4.22\u0026thinsp;\u0026plusmn;\u0026thinsp;2.28\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e6.58\u0026thinsp;\u0026plusmn;\u0026thinsp;1.18\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.011*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative mechanical ventilation, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2 (4.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3 (42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.018*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative vasoactive drugs, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e2 (4.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3 (42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.018*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eCPB time, min\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e140 (119.5, 169)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e157 (121, 205)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.267\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAortic cross-clamp time, min\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e68 (59.5, 86)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e70 (60, 90)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.599\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eMR repair, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e19 (46.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e4 (57.1)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.696\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDelayed sternal closure, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e3 (7.3)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e3 (42.9)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.033*\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eECMO, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e1 (2.4)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e2 (28.6)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.052\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLength of stay, days\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c2\"\u003e\u003cp\u003e17(13, 21)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c3\"\u003e\u003cp\u003e18(9, 49)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.988\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003ctfoot\u003e\u003ctr\u003e\u003ctd colspan=\"4\"\u003eNT-proBNP: N-terminal pro-B-type natriuretic peptide; LVEF: Left ventricular ejection fraction; MR: Mitral regurgitation; LVEDD: Left ventricular end-diastolic diameter; CPB: Cardiopulmonary bypass; ECMO:Extracorporeal membrane oxygenation\u003c/td\u003e\u003c/tr\u003e\u003c/tfoot\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e\u003cp\u003eMortality and complications\u003c/p\u003e\u003cp\u003eMortality of patients was 10.42% (5/48). 2 patients died of heart failure (respectively on the 9th and 49th days after surgery, among which there was one patient received repetitive mitral valvuloplasty due to severe MR). 2 died of malignant arrhythmia (respectively on the 7th and 18th days after surgery) and 1 died of respiratory failure due to a severe pulmonary infection (49 days after surgery). All of them were post-operative death.\u003c/p\u003e\u003cp\u003eSurvival analysis\u003c/p\u003e\u003cp\u003eFollow-up was available for 43 patients, median follow-up was 687 days (range 1-2961 days), and there were no patients who died after discharge. Reoperation was required in 2 patients. Reasons for reoperation included MR re-repair for recurrent MR, and pulmonary artery balloon dilation for pulmonary artery trunk and right pulmonary artery stenosis. The estimated rates of freedom from unfavourable prognosis at 1, 3 and 5 years were 89.6%, 87.5%, and 87.5%, respectively (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). Echocardiograms during the follow-up period showed that LVEF of the surviving patients with pre-operative low LVEF was completely restored. The median time to normalization of left ventricular function was 118 days (range 7-604 days) (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). The differences among Kaplan-Meier survival curves for different subgroups were statistically significant (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003eA-F). Patients with feeding difficulties (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001), pre-operative LVEDD\u0026thinsp;\u0026gt;\u0026thinsp;4 (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.05), pre-operative mechanical ventilation (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001), pre-operative vasoactive drugs (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001), ECMO (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001) and delayed sternal closure (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001) are more likely to have an unfavoural prognosis.\u003c/p\u003e\u003cp\u003eCox proportional hazard analyses\u003c/p\u003e\u003cp\u003eUnivariate and multivariate Cox proportional hazard analyses for independent risk factors for the prognosis are shown in Table\u0026nbsp;\u003cspan refid=\"Tab2\" class=\"InternalRef\"\u003e2\u003c/span\u003e. On multivariate Cox proportional hazard analysis, the surgical age\u0026thinsp;\u0026lt;\u0026thinsp;1 year (HR 16.521, 95%CI:1.084, 251.718, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.044), pre-operative LVEF (HR: 0.708, 95%CI: 0.536, 0.937, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.016), CPB time (HR: 1.024, 95%CI:1.006, 1.042, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.01) and length of stay (HR: 1.074, 95%CI: 1.02, 1.132, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.007) may have a higher risk of unfavourable prognosis for patients. Figure\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e shows the influence of variable on the unfavourable prognosis.\u003c/p\u003e\u003cp\u003e\u003cdiv class=\"gridtable\"\u003e\u003ctable float=\"Yes\" id=\"Tab2\" border=\"1\"\u003e\u003ccaption language=\"En\"\u003e\u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e\u003cdiv class=\"CaptionContent\"\u003e\u003cp\u003eCox proportional hazard analysis for factors for the prognosis among ALCAPA patients\u003c/p\u003e\u003c/div\u003e\u003c/caption\u003e\u003ccolgroup cols=\"5\"\u003e\u003cdiv align=\"left\" class=\"colspec\" colname=\"c1\" colnum=\"1\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c2\" colnum=\"2\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c3\" colnum=\"3\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c4\" colnum=\"4\"\u003e\u003c/div\u003e\u003cdiv align=\"char\" char=\".\" class=\"colspec\" colname=\"c5\" colnum=\"5\"\u003e\u003c/div\u003e\u003cthead\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c1\" morerows=\"1\" rowspan=\"2\"\u003e\u003cp\u003eRisk factor\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colspan=\"2\" nameend=\"c3\" namest=\"c2\"\u003e\u003cp\u003eUnivariate Analysis\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colspan=\"2\" nameend=\"c5\" namest=\"c4\"\u003e\u003cp\u003eMultivariate Analysis\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003ctr\u003e\u003cth align=\"left\" colname=\"c2\"\u003e\u003cp\u003eHazzard Ratio\u003c/p\u003e\u003cp\u003e(95% CI)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c3\"\u003e\u003cp\u003e\u003cem\u003eP\u003c/em\u003e\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c4\"\u003e\u003cp\u003eHazzard Ratio\u003c/p\u003e\u003cp\u003e(95% CI)\u003c/p\u003e\u003c/th\u003e\u003cth align=\"left\" colname=\"c5\"\u003e\u003cp\u003e\u003cem\u003eP\u003c/em\u003e\u003c/p\u003e\u003c/th\u003e\u003c/tr\u003e\u003c/thead\u003e\u003ctbody\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eSex\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.467 (0.104, 2.091)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.32\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eWeight, Z-score\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.334 (0.632, 2.814)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.45\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAge at surgery\u0026thinsp;\u0026lt;\u0026thinsp;1y, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.892 (0.172, 4.625)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.892\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e16.521(1.084, 251.718)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e-0.044\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eShortness of breath, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.563 (0.126, 2.518)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.452\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eGrowth redardation, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.565 (0.066, 4.841)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.603\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative NT-proBNP\u0026thinsp;\u0026gt;\u0026thinsp;5000 pg/ml, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e6.064 (0.726, 50.637)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.096\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative mechanical ventilation, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e16.252 (3.043, 86.789)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.001\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative vasoactive drugs, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e16.252 (3.043, 86.789)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.001\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative LVEDD Z-score\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.813 (1.105, 2.975)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.019\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative LVEF, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.82 (0.721, 0.933)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.003\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e0.708 (0.536, 0.937)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.016\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003ePre-operative moderat-severe MR, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e3.656 (0.436, 30.670)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.232\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAbnormal Q wave, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.767 (0.092, 6.374)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.806\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eST-T change, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e0.72 (0.153, 3.392)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.678\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eT-wave change, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.326 (0.295, 5.954)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.712\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eCPB time, min\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.009 (1, 1.017)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.038\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1.024 (1.006. 1.042)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.01\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eAortic cross-clamp time, min\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.008 (0.982, 1.035)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.548\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eDelayed sternal closure, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e12.056 (2.34, 62.113)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.003\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eECMO, n (%)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e15.849 (2.529, 99.31)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.003\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"left\" colname=\"c4\"\u003e\u0026nbsp;\u003c/td\u003e\u003ctd align=\"left\" colname=\"c5\"\u003e\u0026nbsp;\u003c/td\u003e\u003c/tr\u003e\u003ctr\u003e\u003ctd align=\"left\" colname=\"c1\"\u003e\u003cp\u003eLength of stay, days\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c2\"\u003e\u003cp\u003e1.031 (1.003, 1.059)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c3\"\u003e\u003cp\u003e0.027\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c4\"\u003e\u003cp\u003e1.074 (1.02., 1.132)\u003c/p\u003e\u003c/td\u003e\u003ctd align=\"char\" char=\".\" colname=\"c5\"\u003e\u003cp\u003e0.007\u003c/p\u003e\u003c/td\u003e\u003c/tr\u003e\u003c/tbody\u003e\u003c/colgroup\u003e\u003c/table\u003e\u003c/div\u003e\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eFetuses with ALCAPA usually have no symptoms because the diastolic pressures of the pulmonary artery and aorta are similar during the prenatal circulation. However, after birth, when pulmonary vascular resistance begins to decline, most infants start to show clinical symptoms due to the retrograde flow in the left coronary artery, such as poor development, excessive sweating, breathing difficulties, pallor and feeding difficulties\u003csup\u003e[\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e. At the same time, coronary artery retrograde flow can lead to coronary steal, which further aggravate myocardial ischemia and thereby worsens symptoms. Prompt surgical correction of ALCAPA is required as soon as clinical diagnosis is confirmed, regardless of the patient's age and cardiac function. The anomalous coronary artery is reimplanted into the aorta to reconstruct the dual coronary artery\u003csup\u003e[\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]\u003c/sup\u003e. This operation usually has a good prognosis\u003csup\u003e[\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]\u003c/sup\u003e. The post-operative mortality rate ranges from 0\u0026ndash;16%\u003csup\u003e[\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]\u003c/sup\u003e, and 3% of patients require long-term re-interventions\u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]\u003c/sup\u003e. MR and pulmonary artery stenosis are the main reasons for reoperation surgery\u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]\u003c/sup\u003e.In this study, the post-operative mortality rate was 10.4% (5/48), and the rate of reoperation during the follow-up period was 4.7% (2/43), which are consistent with previous reports.\u003c/p\u003e\u003cp\u003eSince the clinical manifestations of this disease are not specific, our study found that most patients presented with symptoms such as shortness of breath, feeding difficulties, and developmental delays. However, in the group with unfavourable prognosis, feeding difficulties was more common. It has been reported in the literature that younger patients are more likely to experience respiratory distress (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.04), while older patients are more likely to have chest pain as the initial symptom (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.004)\u003csup\u003e[\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e]\u003c/sup\u003e. In some centers, younger surgical age may also be associated with death, possibly due to insufficient development of coronary artery collateral vessels, or more severe ventricular ischemia or dysfunction, or both\u003csup\u003e[\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]\u003c/sup\u003e. In the multivariate Cox regression analysis, it was found that age at surgical\u0026thinsp;\u0026lt;\u0026thinsp;1 year was statistically significant for unfavourable prognosis of the patients..\u003c/p\u003e\u003cp\u003eDuring ALCAPA surgery, there has always been controversy over whether to simultaneously repair the mitral valve. Brown et al.\u003csup\u003e[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]\u003c/sup\u003ebelieve that patients with ALCAPA do not need to undergo mitral valve intervention simultaneously. However, Bicer et al.\u003csup\u003e[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e]\u003c/sup\u003epointed out that although the rate of mitral valve re-intervention is relatively low, it is still necessary to pay attention if MR exceeds moderate severity before the operation. For moderate and severe MR, the degree of damage to the mitral valve is very high. Simply correcting the abnormal coronary arteries and restoring the blood flow of the dual coronary arteries is not sufficient to restore the function of the mitral valve; mitral valve repair needs to be carried out simultaneously\u003csup\u003e[\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]\u003c/sup\u003e. Early implementation of mitral valve repair can improve early post-operative cardiac output and facilitate the early recovery of cardiac function\u003csup\u003e[\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e, \u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]\u003c/sup\u003e. Although some literature has reported that MR is a risk factor for post-operative death\u003csup\u003e[\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e, \u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]\u003c/sup\u003e, the degree of MR did not predict any of our post-operative outcomes. In this study, 29 patients had moderate or severe MR before the operation. Among them, 24 patients underwent mitral valve repair simultaneously. Only 1 patient had severe MR due to gradually worsening mitral regurgitation 6 years after the operation and thus underwent MR repair again. 5 patients did not undergo mitral valve repair, 2 of them died, and 3 survived. During the follow-up period, MR recovered to a mild state in these 3 patients.\u003c/p\u003e\u003cp\u003eIn this studies, longer CBP time (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.01) and length of stay (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.007) were risk factors for unfavourable prognosis. This is the first report indicating that the longer CPB time and the length of stay may be related to the post-operative prognosis.\u003c/p\u003e\u003cp\u003eEarly mortality is significantly associated with left ventricular dysfunction. While advancements in surgical techniques and pharmacological therapies have mitigated the adverse effects on left ventricular function, numerous studies have demonstrated that pre-operative left ventricular dysfunction remains an independent predictor of early mortality\u003csup\u003e[\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e, \u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e]\u003c/sup\u003e. This study corroborates these findings, showing that lower pre-operative LVEF values adversely affect patient prognosis. If there is no left ventricular dysfunction, the prognosis after the surgery will be favourable\u003csup\u003e[\u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]\u003c/sup\u003e. Kudumula's\u003csup\u003e[\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]\u003c/sup\u003ereported in a retrospective study of infants and children with ALCAPA hat 88% of patients achieved left ventricular function recovery during long-term follow-up of 20 years. Existing literature reports a median left ventricular recovery duration of 4 months, with some cases requiring nearly one year for complete functional restoration\u003csup\u003e[\u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e]\u003c/sup\u003e. Occasional prolonged recovery may indicate the gradual reversal of these adaptive cellular changes. Post-operative myocardial depression may sharply exacerbate cardiac dysfunction, that is, the persistent transient mechanical dysfunction that persists after reperfusion and restoration of normal coronary artery blood flow\u003csup\u003e[\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e]\u003c/sup\u003e. In this study, 5 patients died, all of whom presented with significantly reduced pre-operative LVEF. During the post-operative follow-up period, echocardiography showed that the left ventricular function of all the surviving patients, especially those with a low LVEF before the operation, had fully recovered. The median time to LVEF recovery was 181 days, and the longest was 604 days.\u003c/p\u003e\n\u003ch3\u003eLimitations\u003c/h3\u003e\n\u003cp\u003eThe main limitations of this study include its retrospective design, single-center nature, and relatively small sample size. Additionally, the exclusive reliance on echocardiography for cardiac function assessment may not provide comprehensive evaluation of left ventricular contractility.\u003c/p\u003e"},{"header":"Conclusions","content":"\u003cp\u003eALCAPA is a rare congenital anomaly of coronary artery anatomy. Early surgical intervention achieves satisfactory outcomes and improves the left ventricular function in survivors. Younger surgical age, reduced pre-operative LVEF, longer CPB time and length of stay are the key risk factors that affect the unfavourable prognosis of patients.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eALCAPA\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eAnomalous origin of the left coronary artery from the pulmonary artery\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eMR\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eMitral regurgitation\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eLVEF\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eLeft ventricular ejection fraction\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eLVEDD\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eLeft ventricular end diastolic diameter\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eNT-proBNP\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eN-terminal pro-B-type natriuretic peptide\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eCPB\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eCardiopulmonary bypass\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003cdiv class=\"DefinitionListEntry\"\u003e\u003cdiv class=\"Term\"\u003eECMO\u003c/div\u003e\u003cdiv class=\"Description\"\u003e\u003cp\u003eExtracorporeal membrane oxygenation\u003c/p\u003e\u003c/div\u003e\u003c/div\u003e\u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003ch2\u003eCompeting interests\u003c/h2\u003e\u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u003c/strong\u003e\u003cp\u003e This study was performed with the ethics approval from the Institutional\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003ch2\u003eConsent for publication\u003c/h2\u003e\u003cp\u003eNot applicable.\u003c/p\u003e\u003c/p\u003e\u003ch2\u003eFunding\u003c/h2\u003e\u003cp\u003eGuangzhou Major clinical technology project (2023C-ZD10). Guanghzou Science and Technology Bureau Project (2024A03J1165). The Basic and Applied Basic Research Project of Guangzhou Municiple Sccience and Technology Bureau (2025A03J4265)\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eJ Y was responsible for the writing of the article. Yanxing Lv responsible for the analyzed data. Xinyuan Ding performed the collected pre-operative data. L M responsible for the surgery. Hang Yang and Lin Jiang responsible for registering the post-operative follow-up data of patients. N Z designed the research study. ALL authors have read and approved the final version of this manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgements\u003c/h2\u003e\u003cp\u003eWe would like to thank the Guangzhou Women and Children\u0026rsquo;s Medical Center, Guangzhou Medical University for proving support and guidance. The above opinions are those of the authors alone.\u003c/p\u003e\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e\u003ch2\u003eData availability\u003c/h2\u003e\u003cp\u003eThe data used in this manuscript are available upon reasonable request by contacting the corresponding author via email.\u003c/p\u003e\u003c/div\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eYu J, Ren Q, Liu X, et al. Anomalous left coronary artery from the pulmonary artery: Outcomes and management of mitral valve. Front Cardiovasc Med. 2022;9:953420.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eGao Y, Zhang J, Huang GY, et al. Surgical outcomes of anomalous origin of the left coronary artery from the pulmonary artery in children: an echocardiography follow-up. Chin Med J-Peking. 2017;130(19):2333\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLee AC, Foster E, Yeghiazarians Y, et al. Anomalous origin of the left coronary artery from the pulmonary artery: a case series and brief review. Congenit Heart Dis. 2006;1(3):111\u0026ndash;5.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLotman EM, Karu K, Mikkel M, et al. Late adult presentation of ALCAPA syndrome: need for a new clinical classification? A case report and literature overview. Eur Heart J Case Rep. 2020;4(6):1\u0026ndash;5.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKazmierczak PA, Ostrowska K, Dryzek P, et al. Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants. Interact Cardiov Th. 2013;16(6):797\u0026ndash;801.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003ePettersen MD, Du W, Skeens M. Regression equations for calculation of z scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr. 2008;21(8):922\u0026ndash;34.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eHumes E, Dehaki RA, Al-Dairy MG, Rezaei A. Mid\u0026ndash;term outcomes of surgical repair for anomalous origin of the left coronary artery from the pulmonary artery: In infants, children and adults. Ann pediat cardiol. 2017;10(2):137\u0026ndash;43.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eBacker CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg. 1992;103(6):1049\u0026ndash;57.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg. 2002;74(3):946\u0026ndash;55.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eWeigand J, Marshall CD, Bacha EA, et al. Repair of anomalous left coronary artery from the pulmonary artery in the modern era: preoperative predictors of immediate postoperative outcomes and long term cardiac follow-up. Pediatr Cardiol. 2015;36(3):489\u0026ndash;97.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMuzaffar T, Ahmad Ganie F, Gpoal Swamy S, et al. The surgical outcome of anomalous origin of the left coronary artery from the pulmonary artery. Int Cardiovasc Res J. 2014;8(2):57\u0026ndash;60.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eStraka N, Gauvreau K, Huang Y. Analysis of Perioperative and Long\u0026ndash;Term Outcomes Among Presentations of Anomalous Left Coronary Artery from the Pulmonary Artery Diagnosed Beyond Infancy Versus During Infancy.Pediatric cardiology. 2025, 46(1): 139\u0026ndash;47.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAzakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: Early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg. 2003;75(5):1535\u0026ndash;41.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eBrown JW, Ruzmetov M, Parent JJ, et al. Does the degree of preoperative mitral regurgitation predict survival or the need for mitral valve repair or replacement in patients with anomalous origin of the left coronary artery from the pulmonary artery? J Thorac Cardiovasc Surg. 2008;136(3):743\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eBi\u0026ccedil;er M, Korun O, Yurdak\u0026ouml;k O, et al. Anomalous left coronary artery from the pulmonary artery repair outcomes: preoperative mitral regurgitation persists in the follow-up. J Card Surg. 2021;36(2):530\u0026ndash;5.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMong\u0026eacute;MC, Eltayeb O, Costello JM, et al. Aortic implantation of anomalous origin of the left coronary artery from the pulmonary artery: long-term outcomes. Ann Thorac Surg. 2015;100(1):154\u0026ndash;60.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eZhang C, Luo Q, Li Y, et al. Predictors of short-term outcomes following repair of anomalous origin of the left coronary artery from the pulmonary artery in Chinese children: a case-control study. J Cardiothorac Vasc Anesth. 2018;32(6):2644\u0026ndash;51.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSchwartz ML, Jonas RA, Colan SD. Anomalous Origin of Left Coronary Artery From Pulmonary Artery: Recovery of Left Ventricular Function After Dual Coronary Repair. JACC. 1997;3(2):547\u0026ndash;53.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eZhang C, Zhang H, Yan J, et al. Mid-term outcome for anomalous origin of the left coronary artery from the pulmonary artery. Heart Lung Circ. 2020;29(5):766\u0026ndash;71.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eHarky A, Noshirwani A, Karadakhy O, et al. Comprehensive literature review of anomalies of the coronary arteries. J Card Surg. 2019;34(11):1328\u0026ndash;43.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eKudumula V, Mehta C, Stumper O, et al. Twenty-year outcome of anomalous origin of left coronary artery from pulmonary artery: Management of mitral regurgitation. Ann Thorac Surg. 2014;97(3):938\u0026ndash;44.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eHu RJ, Zhang W, Yu XF. Midterm Surgical Outcomes for ALCAPA Repair in Infants and Children. Thorac cardiov suger. 2022;70(1):2\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"ALCAPA, children, the unfavourable prognosis","lastPublishedDoi":"10.21203/rs.3.rs-7360768/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7360768/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eObjectives\u003c/h2\u003e\u003cp\u003eThis study aims to examine long-term outcomes on patients with ALCAPA and analyze the relevant factors influencing the post-operative after surgery.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e\u003cp\u003ePatients with ALCAPA admitted from January 2015 to December 2024 were retrospectively reviewed. Clinical data of the patients were retrieved from the record. The follow-up data include mortality rate and complications. The Kaplan-Meier survival curve was used to analyze the occurrence of death and reoperation during the follow-up of the patients with ALCAPA, and the Log-rank test was employed for comparison between groups. The Cox regression model was used to analyze the risk factors for adverse events in patients with unfavourable prognosis.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e\u003cp\u003eOf 48 patients meeting the study criteria. The post-operative death or reoperation of the patients are defined as unfavourable prognosis. Patients are divided into two groups: the favourable prognosis (n\u0026thinsp;=\u0026thinsp;41) and the unfavourable prognosis (n\u0026thinsp;=\u0026thinsp;7). The differences among Kaplan-Meier survival curves for different subgroups were statistically significant. Patients with feeding difficulties (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001), pre-operative LVEDD\u0026thinsp;\u0026gt;\u0026thinsp;4 (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.05), pre-operative mechanical ventilation (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001), pre-operative vasoactive drugs (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001), ECMO (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001) and delayed sternal closure (\u003cem\u003ep\u003c/em\u003e\u0026thinsp;\u0026lt;\u0026thinsp;0.001) are more likely to have an unfavorable prognosis. On multivariate Cox proportional hazard analysis, the age at surgical\u0026thinsp;\u0026lt;\u0026thinsp;1y (HR 16.521, 95%CI: 1.084, 251.718, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.044), pre-operative LVEF (HR: 0.708, 95%CI: 0.536, 0.937, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.016), CPB time (HR: 1.024, 95%CI: 1.006, 1.042, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.01) and length of stay (HR: 1.074, 95%CI: 1.02, 1.132, \u003cem\u003ep\u003c/em\u003e\u0026thinsp;=\u0026thinsp;0.007) may have a higher risk of unfavourable prognosis for patients.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e\u003cp\u003eYounger surgical age, reduced pre-operative LVEF, longer CPB time and length of stay are the key risk factors that affect the unfavourable prognosis of patients.\u003c/p\u003e","manuscriptTitle":"Analysis of relevant factors associated with post-operative recovery after anomalous origin of the left coronary artery from the pulmonary artery surgery in children","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-10-23 19:08:58","doi":"10.21203/rs.3.rs-7360768/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"7476ede6-3fa9-4102-993a-e2cb63a041ac","owner":[],"postedDate":"October 23rd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-10-27T14:41:14+00:00","versionOfRecord":[],"versionCreatedAt":"2025-10-23 19:08:58","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7360768","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7360768","identity":"rs-7360768","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}