Mutation spectrum of FLT3 and significance of non-canonical FLT3 mutations in haematological malignancy.

Fms-like tyrosine kinase 3 (FLT3) is frequently mutated in haematological malignancies. Although canonical FLT3 mutations including internal tandem duplications (ITDs) and tyrosine kinase domains (TKDs) have been extensively studied, little is known about the clinical significance of non-canonical FLT3 mutations. Here, we first profiled the spectrum of FLT3 mutations in 869 consecutively newly diagnosed acute myeloid leukaemia (AML), myelodysplastic syndrome and acute lymphoblastic leukaemia patients. Our results showed four types of non-canonical FLT3 mutations depending on the affected protein structure: namely non-canonical point mutations (NCPMs) (19.2%), deletion (0.7%), frameshift (0.8%) and ITD outside the juxtamembrane domain (JMD) and TKD1 regions (0.5%). Furthermore, we found that the survival of patients with high-frequency (>1%) FLT3-NCPM in AML was comparable to those with canonical TKD. In vitro studies using seven representative FLT3-deletion or frameshift mutant constructs showed that the deletion mutants of TKD1 and the FLT3-ITD mutant of TKD2 had significantly higher kinase activity than wild-type FLT3, whereas the deletion mutants of JMD had phosphorylation levels comparable with wild-type FLT3. All tested deletion mutations and ITD were sensitive to AC220 and sorafenib. Collectively, these data enrich our understanding of FLT3 non-canonical mutations in haematological malignancies. Our results may also facilitate prognostic stratification and targeted therapy of AML with FLT3 non-canonical mutations.

Investigations of the prognostic value of RUNX1 mutation in acute myeloid leukemia patients: Data from a real-world study.

RUNX1 is one of the recurrent mutated genes in newly diagnosed acute myeloid leukemia (AML). Although historically recognized as a provisional distinct entity, the AML subtype with RUNX1 mutations (AML-RUNX1mut) was eliminated from the 2022 WHO classification system. To gain more insight into the characteristics of AML-RUNX1mut, we retrospectively analyzed 1065 newly diagnosed adult AML patients from the First Affiliated Hospital of Soochow University between January 2017 and December 2021. RUNX1 mutations were identified in 112 patients (10.5%). The presence of RUNX1 mutation (RUNX1mut) conferred a lower composite complete remission (CRc) rate (40.2% vs. 58.4%, P＜0.001), but no significant difference was observed in the 5-year overall survival (OS) rate (50.2% vs. 53.9%; HR=1.293; P=0.115) and event-free survival (EFS) rate (51.5% vs. 49.4%; HR=1.487, P=0.089), even within the same risk stratification. Multivariate analysis showed that RUNX1mut was not an independent prognostic factor for OS (HR=1.352, P=0.068) or EFS (HR=1.129, P=0.513). When patients were stratified according to induction regimen, RUNX1mut was an unfavorable factor for CRc both on univariate and multivariate analysis in patients receiving conventional chemotherapy, and higher risk stratification predicted worse OS. In those who received venetoclax plus hypomethylating agents, RUNX1mut was not predictive of CRc and comparable OS and EFS were seen between intermediate-risk and adverse-risk groups. The results of this study revealed that the impact of RUNX1mut is limited. Its prognostic value depended more on treatment and co-occurrent abnormalities. VEN-HMA may abrogate the prognostic impact of RUNX1, which merits a larger prospective cohort to illustrate.

The Relationship between Corporate Social Responsibility and Firm Value of Chinese Firms: Exploring from Degree of Internationalization.

The purpose of this paper is to explore the relationship between corporate social responsibility (CSR) and firm value in the Chinese market and identify factors that may influence it. We discuss the relationship between CSR engagement in Chinese firms and firm value from a nonlinear perspective. In addition, we examine how the degree of internationalization in a firm may affect the relationship between CSR and firm value. We employ the Hausman test to compare a random-effects and a fixed-effects model, and after testing and comparison, the fixed-effects model was chosen in our paper. Using data from 314 firms listed in China's A-shares market from 2010 to 2017, we verify the U-shaped relationship between CSR and firm value. Meanwhile, the degree of internationalization will affect firm value but cannot positively regulate the relationship between corporate social responsibility and firm value.

Flumatinib plus venetoclax as an effective therapy for Philadelphia chromosome-positive acute myeloid leukemia: A case report.

Philadelphia chromosome-positive acute myeloid leukemia (Ph + AML) is a rare type of AML with a low survival rate and poor prognosis. We first report a Ph + AML patient who remained in long-term remission after the combination of flumatinib and venetoclax, which could provide corresponding treatment ideas for clinical practice.

Metagenomic next-generation sequencing performed on blood samples for the early recognition of severe Pneumocystis pneumonia in critical hematological patients.

Severe Pneumocystis pneumonia (PCP) has a poor prognosis, and its early and precise diagnosis is difficult in immunocompromised individuals. Therefore, this study explored the diagnostic value of metagenomic next-generation sequencing (mNGS) of peripheral blood in diagnosing severe PCP in patients with hematological diseases. This prospective study analyzed the clinical manifestations, mNGS results (from the peripheral blood), traditional pathogen detection results, laboratory test results, chest computed tomography (CT) images, treatments, and outcomes of severe PCP in hematological patients who were hospitalized in the 2 centers of the Affiliated Hospital of Soochow University between September 2019 and October 2021. A total of 31 cases of hematological diseases complicated with pulmonary infections, including 7 cases of severe PCP diagnosed by mNGS performed on peripheral blood samples, were analyzed. Traditional pathogen detection methods for PCP cannot be used. In contrast, the laboratory readings for Pneumocystis jirovecii (Pj) detected within 48 hours of symptom onset by mNGS on the 7 blood samples ranged from 12 to 5873, with a median value of 43. Under the guidance of the mNGS results, preemptive antimicrobial therapy with trimethoprim/sulfamethoxazole alone or in combination with caspofungin was administered to treat Pj. After treatment, 4 patients recovered, and 3 patients died of acute respiratory failure and acute respiratory distress syndrome (ARDS). MNGS performed on peripheral blood samples is optional but can provide early recognition of severe PCP and help guide empirical treatment in critical hematological patients.

Mid-term results after correction of type I and type II persistent truncus arteriosus in older patients.

OBJECTIVE: This study aims to analyze long-term results after correction of type I and type II truncus arteriosus in older patients operated in one institution over five years. METHODS: Between 2006 and 2010, 12 patients, median age 4 years, underwent repair of truncus arteriosus. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 12 patients. RESULTS: There was no early mortality. All patients are alive with their original conduit 0.6 to 5 years after correction. No patients required reoperations for conduit dysfunction. Recent clinical examination was undertaken in all patients and they are in good condition. CONCLUSIONS: Though mean age at operation was higher in this study than published results, the operation should be performed if the pulmonary vascular resistance is under 8 units.m(2) before operation.

Study on the influence mechanism of students' behavior of participation in industrial colleges-Analysis framework based on theory of planned behavior.

As a new organizational form for the coordinated development of education and industry, industrial college is an important carrier for application-oriented colleges and universities to implement application-oriented talent training. Based on Theory of Planned Behavior (TPB) as a theoretical framework, this research constructs a model of the influencing factors of college students' participating in industrial colleges from five criteria: behavioral attitudes, subjective norms, perceived behavioral control, behavioral intentions and situational factors. In this study, participants (N = 541) have completed a questionnaire to assess their behavior of participation in the industrial colleges by using the structural equation model (SEM). It turns out that perceived needs, mandatory norms, exemplary norms, and self-efficacy significantly positively affect college students' intention to participate in industrial colleges. Behavior intention and self-efficacy significantly positively affect college students' participation in industrial colleges. Behavior intentions act as a complete mediator between perceived needs and behavior, as well as between exemplary norms and behavior. Behavioral intentions partially mediate between mandatory norms and behavior, as well as between self-efficacy and behavior. In addition, school support plays a significant positive moderating role in college students' intention and behavior of participating in industrial colleges. These findings not only expand the relevant literature on college students' learning behavior, but also provide useful enlightenment for college education managers on how to stimulate college students' endogenous motivation to participate in industrial colleges.

Research on the Influence Mechanism of Enterprises' Participation in School Enterprise Cooperation Based on the Analysis Framework of Theory of Planned Behavior.

School enterprise cooperation, as the basic school running form of applied undergraduate education, is an important way to cultivate applied talents. However, at present, the lack of motivation for enterprises to participate in school enterprise cooperation and the resulting problem of "school hot and enterprise cold" seriously limit the talent training quality in China's application-oriented universities. There is an urgent need to explore the influencing factors and mechanisms of enterprises' participation in school enterprise cooperation to improve the training quality of applied talents. Taking Ajzen (2002) Theory of Planned Behavior as the theoretical framework, this study constructs the influencing factor model of enterprise participation in school enterprise cooperation from four aspects, namely, behavior attitude, subjective norms, perceived behavior control, and behavior intention. In this study, participants (N = 250) completed a questionnaire assessing their participation in school enterprise cooperation, which was analyzed by a structural equation model. The results show that the behavior attitude and perceived behavior control of enterprises have a significant positive impact on their intention to participate in school enterprise cooperation and then have a significant positive impact on the school enterprise cooperation behavior of enterprises. The behavior intention and perceived behavior control of enterprises have a significant positive impact on their participation in school enterprise cooperation. The policy environment has a significant regulatory effect on the relationship between the intention and behavior of enterprises' participation in school enterprise cooperation. Therefore, from the perspective of enhancing the intention of enterprises to cooperate, colleges and universities should establish the awareness of win-win cooperation and meet the interest demands of enterprises in school enterprise cooperation in order to improve the behavior attitude and intention of enterprises. From the perspective of the formation conditions of school enterprise cooperation, with the help of industry associations, an information service platform for school enterprise cooperation should be built in order to eliminate the information islands between enterprises and universities. From the perspective of the needs of school enterprise cooperation environment, government departments should strengthen the policy support for school enterprise cooperation in order to eliminate the worries of enterprises' participation in school enterprise cooperation.

A Data Envelopment Analysis Evaluation Study of Urban Crowd Sourcing Competitiveness Based on Evidence From 21 Chinese Cities.

In the era of the global village, crowd sourcing as a new model of service outsourcing is increasingly being valued by all walks of life. This study uses the data envelopment analysis (DEA) method to explain the crowd sourcing competitiveness of service outsourcing base cities by using input-output efficiency. The crowd sourcing competitiveness among crowd sourcing base cities is organized and analyzed by collating and analyzing the data of 21 service outsourcing base cities in China from 2016 to 2019. The results show that there is no significant difference in the competitiveness of 21 service outsourcing, the overall trend is that the competitiveness of crowd sourcing is not strong, the match between input and output is not high. Comparatively speaking, Beijing, Shanghai, Nanjing, Hangzhou, Suzhou and Wuxi are more competitive in crowd sourcing. Combining with the reality of China's economic development, taking the road of crowd sourcing with Chinese characteristics is a good choice for the development of China's crowd sourcing industry.

The Effect of Transformational Change on Performance: An Employee's Stress Appraisals Perspective.

This study aims to determine the specific impact of employees' perceptions of transformational change on in-role performance and how stress assessment can mediate the relationship between transformational change and in-role performance. According to the cognitive appraisal theory, the same individual has different appraisals of the same stressors, including challenge, and hindrance appraisal. As an important stressor, transformational change also affects individuals differently depending on their assessments. This study integrates employees' challenge or hindrance appraisal of transformational change into a conceptual model to distinguish between the roles of the two appraisals. It examines it as a mediating mechanism between transformational change and in-role performance. Additionally, 313 employees who recently experienced transformational change were used as samples to test the hypothesis. The results show that transformational change negatively affects employees' in-role performance; hindrance appraisal negatively mediates the relationship between transformational change and in-role performance, and challenge appraisal positively mediates the relationship between transformational change and in-role performance. The originality and value of this research extend the application of stress appraisals in organizational change management. Research shows that, in the context of major change, employees' in-role performance is reduced by the impact of transformational change. However, when employees positively appraise organizational change, the negative effects of change are weakened.

Efficiency of anti-VEGF therapy in central nervous system AML relapse: A case report and literature review.

There have been few reports on the treatment of central nervous system (CNS) acute myeloid leukemia (AML) relapse. This case study demonstrates that bevacizumab may be a viable treatment option when combined with IT chemotherapy as maintenance therapy for those with CNS leukemia.

Combination of Venetoclax and Midostaurin Efficiently Suppressed Relapsed t(8;21)Acute Myeloid Leukemia With Mutant KIT After Failure of Venetoclax Plus Azacitidine Treatment.

Acute myeloid leukemia (AML) with t(8;21) is categorized as favorable-risk AML, but KIT mutations show a significantly poor prognostic impact in such patients. Persistent vulnerability to relapse is a major challenge in the treatment of this subtype of patients. Venetoclax is a BCL-2 selective inhibitor. The venetoclax+HMA strategy is also a notable salvage regimen that achieves good clinical outcomes in the treatment of relapsed or refractory (R/R) AML. However, in our clinical practice, we found that disease progressed rapidly even after venetoclax+azacitidine (AZA) therapy in two relapsed t(8;21) AML patients with KIT mutations. We report for the first time the therapeutic potential of venetoclax+midostaurin as a new combination therapy for relapsed t(8;21) AMLs with KIT mutations showing resistance to venetoclax+AZA therapy. Our ex vivo study also showed that midostaurin alone could inhibit proliferation and induce apoptosis of Kasumi-1 cells (e.g. Midostaurin induced G2 phase cell arrest, down-regulated p-KIT and BCL-2, while Bax protein levels were up-regulated) and observed a synergistic anti effect when the two drugs were combined. Our study shows that the venetoclax+midostaurin regimen may be a promising treatment option for R/R t(8;21) AML with KIT mutations.

The Effect of Baby Food E-Store Image (for Ages 0-3) on Consumers' Purchase Intention.

This research aims to investigate the influence of baby food e-store image (for ages 0-3) on consumers' purchase intention, mainly based on the stimulus-organism-response (S-O-R) model. Perceived value is additionally tested as a mediator of the relationship between baby food e-store image and consumers' purchase intention. An online questionnaire survey was conducted among consumers of baby food e-stores that specialize in ages 0-3. The final sample comprised 584 valid responses. To test the hypotheses in the relationships among latent variables, structural equation model analysis was used in this study. The findings showed that design image, order fulfillment image, communication service image, and security image of baby food e-stores have positive effects on consumers' perceived value, while perceived value mediates the relationship between baby food e-stores and consumers' purchase intention. As a result, more perceived value would be created between online stores and their consumers with a higher level of value. Two managerial implications derived from this study explain how to manage baby food e-stores and how to promote online baby food undertakings. Particularly, consumers' perceived emotional and functional value can be shaped by four dimensions of baby food e-store image, e-store design image, order fulfillment image, communication service image, and security image, which finally determines their purchase intentions. The originality and value of this study enriches the importance of consumers' perceived value in the field of online store image. Moreover, this study demonstrates that baby food e-store image increases consumers' perceived value and purchase intention.

Understanding the Dark Side of Online Reviews on Consumers' Purchase Intentions in E-Commerce: Evidence From a Consumer Experiment in China.

Background: Online review, as an important way of electronic word-of-mouth (eWOM) communication, plays an important role in e-commerce. However, few studies have examined the dark side of online reviews and their effect on consumers' purchase intentions. Information inconsistency is one of the dark sides that plays a critical role in influencing consumers' purchase intentions through online reviews. Methods: Using a 2*2 between-subject design that explores the main effects of the type of information inconsistency (vertical- vs. horizontal-attribute inconsistency) on purchase intention and the moderating effect of product type (search vs. experience product). Results: This study examines whether and how the type of information inconsistency between online recommendations and reviews influences consumer purchase decision-making. Conclusions: The findings show that vertical-attribute inconsistency leads to a lower purchase intention for search products; moreover, both vertical- and horizontal-attribute inconsistencies have no significant effect on purchase intention for experience products.

Influencing Factors of the Continuous Usage Intention of Consumers of Online Food Delivery Platform Based on an Information System Success Model.

Online food delivery (OFD) has gradually become a development trend in the catering industry. However, the homogenization of the principal OFD platforms is becoming increasingly obvious, and there is an urgent need to explore the continuous usage intentions of consumers of the OFD platform. Unlike previous studies, this study employs DeLone and McLean's information system (IS) success model as the theoretical framework and constructs the influencing factors model of the continued usage intentions of users of the OFD platform from three aspects, namely, service, system, and information quality. Participants (N = 361) completed a questionnaire assessing their continuous usage intentions of the OFD platform, and the results were analyzed using a structural equation model (SEM). Service worker, platform quality, and content consistency have significant positive effects on the trust in the platform and satisfaction of consumers. Both the trust in the platform and satisfaction of consumers have a positive impact on their continuous usage intentions. We also explored the mediating effect of the trust in the platform and satisfaction of consumers. This study adds to the academic literature and practical implications for the continuous usage intention of consumers. This study discusses the influencing factors and formation mechanism of the continuous usage intention of consumers and proposes a model of the continuous usage intention of consumers of OFD platforms. Moreover, OFD platforms can make differentiation strategies more targeted and eliminate the dilemma of homogenization competition of OFD platforms.

A Combined Histone Deacetylases Targeting Strategy to Overcome Venetoclax Plus Azacitidine Regimen Resistance in Acute Myeloid Leukaemia: Three Case Reports.

The management of patients with relapsed or refractory (R/R) acute myeloid leukaemia (AML) remains a challenge with few reliably effective treatments. Chidamide, a new selective HDAC inhibitor, has demonstrated some effectiveness in AML patients. Herein, we reported three patients with R/R AML who were unresponsive to venetoclax plus azacitidine (VA) but were successfully treated with VA when chidamide was added to the regimen. MCL1 is one of the anti-apoptotic proteins. Chidamide targets the MCL1 protein, which may permit venetoclax resistance when upregulated. We determined MCL1 protein expression in different AML cell lines, and chidamide could downregulate MCL1 expression in venetoclax resistance AML cells. In general, our experience showed that the chidamide/VA combination could improve the condition of R/R AML patients who are resistant to VA. Formally evaluating this regimen in R/R AML patients may be meaningful.

Safety and efficacy of arterial switch operation in previously inoperable patients.

BACKGROUND: Patients older than six months with complete transposition of the great arteries and ventricular septal defect or Taussig-Bing anomaly and severe pulmonary arterial hypertension (PAH), previously thought to be inoperable, usually are not indicated for arterial switch operation (ASO) or even repair. This study aimed to evaluate the safety and efficacy of ASO in these selected subset patients. METHODS: The records of 86 patients older than six months with complete transposition of the great arteries and ventricular septal defect or Taussig-Bing anomaly and severe PAH who underwent ASO at our institution from May 2000 to October 2008 were reviewed retrospectively. Eighty survivors were followed-up. RESULTS: There were six hospital deaths (7.0%, 95% confidence limit 1.6 to 12.4%). From January 2006 to October 2008, 46 consecutive ASOs were performed with no death. Operative mortality and mobility decreased significantly (p = 0.008 and p = 0.046, respectively). The median duration of follow-up was 42.1 +/- 28.8 months (range, 2.0 to 99.5). Two late deaths occurred. Latest follow-up data showed that 2.8% of survivors were in New York Heart Association (NYHA) class II and 97.2% were in NYHA class I. CONCLUSIONS: Excellent early and mid-term results of ASO are obtained from patients older than six months with complete transposition of the great arteries and ventricular septal defect or Taussig-Bing anomaly and severe PAH in current era, and ASO is safe and effective in these selected subset patients.

[Influence of coronary artery variation on the outcome of arterial switch operation].

OBJECTIVE: To investigate the influence of coronary artery variation on the outcome of arterial switch operation for transposition of great arteries. METHODS: Among 280 patients undergoing arterial switch operations at our hospital from 2001 to 2008, 73 (26.1%) had concurrent coronary arteries variation (54 males and 19 females; median age: 0.6 ± 1.1 years old; mean body weight: 5.8 ± 2.6 kg). Of these 73 patients (variant group), 21 cases had transposition of great arteries with a ventricular septal defect and 30 cases with an intact ventricular septum. The other 22 cases were of Taussig-Bing anomalies. Another 207 cases had usual coronary arteries (usual group). Coronary artery transfer was achieved by implantation of buttons to the previously anastomosed neo-aorta. RESULTS: There were 29 early death (10.4%) including 12 cases (16.4%) in variant group and 17 cases (8.21%) in usual group (P < 0.05). Mean cardiopulmonary bypass and cross-clamp durations were 229 ± 84 and 146 ± 48 min in variant group while 206 ± 59 and 137 ± 40 min in usual group (P < 0.05). Six cases were confirmed intra-operatively as coronary compression or obstruction. Complications included infection (n = 11), low output syndrome (n = 7), diaphragm paralysis (n = 3), pericardial effusion (n = 2) and atrioventricular block (n = 2). CONCLUSION: Coronary artery variation increases the operative difficulty and influences the outcome. The operative proficiency may decrease the mortality.

[One-stage repair of the interrupted aortic arch associated with cardiac anomaly].

OBJECTIVE: To evaluate the clinical experiences of one-stage repair of the interrupted aortic arch (IAA) associated with cardiac anomaly in neonates and infants. METHODS: From July 1996 to October 2008, 48 neonates or infants with IAA associated with cardiac anomaly underwent one-stage repair. There are 30 males and 18 females ranging from 0.08 to 7.00 years old (1.97 +/- 2.05) with body weight 3 - 20 (9 +/- 5) kg. 42 patients had the middle-severe pulmonary hypertension (PH), the pulmonary pressure was 61 - 106 (82 +/- 14) mm Hg and the pulmonary arterial resistance was 66 - 762 (315 +/- 259) dynxsxcm(-5). There are 40 patients with type A IAA and 8 patients with type B IAA, all patients had an associated patent ductus arteriosus, other combined malformations included ventricular septal defect (VSD) in 33 cases, atrial septal defect (ASD) in 8 cases, mitral valve insufficiency in 6 cases, tricuspid valve insufficiency in 5 cases, aortopulmonary window in 4 cases, double outlet of the right ventricule in 3 cases, mitral valve stenosis in 2 cases, and et al. the operation was performed through median sternotomy under the general anesthesia and cardiopulmonary bypass (CPB, the deep hypothermia with circulatory arrest, the deep hypothermia with low flow rate, or the deep hypothermia with low flow rate and circulatory arrest), the interrupted aortic arch repair was achieved with the arch anastomosis in 30 patients and with an interposition graft in 18 patients, the concomitant operations included VSD repair in 33 cases, ASD repair in 8 cases, mitral valve plasty in 7 cases, tricuspid valve plasty in 4c ases, aortopulmonary window repair in 4 cases, intraventricular baffle tube repair in 2 cases, arterial switch operation in 1 case, and et al. RESULTS: The time of the CPB and the aortic occlusion were 112 to 375 min (182 +/- 52) and 24 to 287 min (99 +/- 45) respectively. The endotracheal intubation time were 8 to 936 hours (179 +/- 133) and the ICU stay were 1 - 57 days (14 +/- 14). By echocardiograph examination after operation, the pressure gradient across the arch was obvious relieved and the combined cardiac malformations was satisfied corrected. 3 patients (3/48, 6.25%) died during the perioperative period. The cause of death included severe pulmonary hypertension crisis, dysfunction of the pulmonary or the serious low cardiac output syndrome. the main complications included reopen for bleeding in 2 cases, tracheostomy in 2 cases, the right diaphragma paralysis in 1 case, delayed sternal closure in 1 case, hydrothorax in 1 case, the severe pulmonary infection in 1 case. Physical activities of the survivals increased obviously and discharged from hospital uneventfully, all surviving patients had no late complications and death during the follow-up 3 months to 12 years. CONCLUSION: One-staged primary repair of IAA with other intracardiac anomalies can be the preferred surgical approach and safely applied with good results as to lead a better life.

[The analysis of the recent effectiveness of the total anomalous pulmonary venous connection repairs on infants].

OBJECTIVE: To review the efficacy of total anomalous pulmonary venous connection (TAPVC) repair and to conclude the factors impacting the peri-operative death rate. METHODS: The clinical data of 145 infants under 1 year old who underwent the TAPVC repair from January 2001 to July 2008 was analyzed. There were 94 male and 51 female patients. The mean age when the repair was performed was (7 ± 3) months, and the average weight was (6.3 ± 1.6) kg. As to the pulmonary connection type, 77 patients were supracardiac (53.1%), 47 patients were cardiac (32.4%), 9 patients were intracardiac (6.2%), and the remaining 12 patients were mixed (8.3%). Pre-surgery echocardiography showed that 21 patients had pulmonary venous obstruction (12 patients were supracardiac type, 3 patients were cardiac type, 3 patients were intracardiac type, and 3 patients were mixed type). RESULTS: All patients underwent two-ventricle anatomy correction (the cases of complex malformations had been excluded). Peri-operative mortality was 11.7% (17/145). Because of the significant improvement in the surgical techniques, anesthesiology, cardiopulmonary bypass and the management of ICU in January 2006, the population was divided into two groups: A (before January 2006) and B (after January 2006). Peri-operative mortality decreased from 19.0% in group A to 6.2% in group B(P = 0.020). After analysis, it was determined that the factors impacting mortality were which group the patient belongs to, whether he/she had preoperative pulmonary vein obstruction and how big the atril septel connection was. The operative technique to keep the anastomotic aperture adequate and prophylaxis pulmonary hypertensive episodes contributed to the improvement on the mortality rate. There had been no case of repeating the surgery because of pulmonary venous obstruction during peri-operative care period. CONCLUSIONS: Improvements of the surgical technique as well as the treatment in preoperative and postoperative have led to the reduction of the mortality. Preoperative pulmonary vein obstruction is still an important factor that contributes to early mortality.