Objective    To compare and investigate the efficacy and differences of modified B-T shunt, central shunt and right ventricle-pulmonary artery (RV-PA) connection in the treatment of pulmonary atresia with ventricular septal defect (PA/VSD). Methods    A total of 124 children with PA/VSD underwent initial palliative repair in Shanghai Children's Medical Center from September 2014 to August 2019, including 63 males and 61 females, aged 7 days to 15 years. They were divided into in a modified B-T shunt group (55 patients), a central shunt group (22 patients) and a RV-PA connection group (47 patients). The clinical data of these children were retrospectively analyzed. Results    There were 9 early deaths after palliation, with an early mortality rate of 7.3%. The mean follow-up time was 26.5±20.3 months, with 5 patients lost to follow-up, 5 deaths during the follow-up period, and 105 survivors. The 1-year and 5-year survival rates were both 89.7%. The monthly increased Nakata index was 5.2 (–0.2, 12.3) mm2/m2, 9.2 (0.1, 23.6) mm2/m2, 6.3 (1.8, 23.3) mm2/m2 in the modified B-T shunt group, the central shunt group, and the RV-PA connection group, respectively, with no statistical difference among the three groups. The 1-year survival rate was 85.3%, 78.4%, 95.2%, and the 5-year (4-year in the central shunt group) survival rate was 85.3%, 58.8%, 95.2% in the three groups, respectively, with a statistical difference among them (P<0.05). The complete repair rate was 36.5%, 19.0% and 67.4% in the three groups, respectively, with a statistical difference among the three groups (P<0.001). Conclusion    All these three palliative surgical approaches can effectively promote pulmonary vascular development. But compared with systemic-pulmonary shunt, RV-PA connection has a lower perioperative mortality rate and can achieve a higher complete repair rate at a later stage, which is beneficial for long-term prognosis.

Objective:To optimize the strategy of Nuss procedure with open approach via median sternotomy for pectus excavatum(PE) with congenital heart disease(CHD).Methods:25 PE patients with CHD treated in our center from January 2017 to November 2021 were reviewed and divided into two groups. The CHD surgery and open Nuss procedure with median sternotomy were performed concomitantly in 9 cases(group A), whose height of(121.7±28.0)cm, weight of(22.2±14.0)kg, age of(7.65±4.08) years old, and Haller index of 3.99±1.37. 16 cases underwent open Nuss procedure via median re-sternotomy after congenital heart surgery(group B), whose height of(130.9±27.2)cm, weight of(26.5±14.3)kg, age of(8.82±4.09) years old, and Haller index of 4.18 ± 0.97. The cardiac anatomy, cardiac function and the severity of PE of all patients were evaluated by echocardiography and computed tomography preoperatively. The preoperative gender, appearance of PE, types of associated CHD, retrosternal adhesion and the interval between open Nuss procedure and CHD surgery were collected. The details of operation, hospital stay, intraoperative adverse events, postoperative complications and follow-up were collected.Results:All the operations were accomplished successfully in 25 children. No intraoperative complications occurred. One had a postoperative wound infection in group A. The operation time of group A was significantly longer than that of group B[(3.78±1.54) h vs.(2.19±0.94) h, P<0.05]. There was no significant difference in the mechanical ventilation time between the two groups[(22.50±45.64) h vs.(4.18±1.41) h, P=0.263]. The ICU stay of group A was significantly longer than that of group B[(4.00±6.42) days vs.(1.13±0.34) days, P<0.05]. There was no significant difference in the length of hospital stay between the two groups[(16.00±15.18) days vs.(9.19±2.31) days, P=0.419]. The Nuss bar was removed in 14 cases of the two groups, with a interval of(27.9±11.25) months after open Nuss procedure, the postoperative Haller Index was 2.48±0.49. There was no difference of LVEF in postoperative and preoperative echocardiography(0.663±0.028 vs. 0.659±0.038, P=0.533). The FVC and FEV1 were significantly improved compared with before operation[(87.2±3.9)% vs.(84.1±8.2)%]. The outcome was good. Conclusion:For PE patients with CHD, one-stage or staged individualized Nuss procedure with open approach via median sternotomy can be performed after careful preoperative evaluation. A skillful median re-sternotomy and widely dissecting retrosternal adhesion between sternum and anterior wall of heart are necessary to avoid serious intraoperative complications such as massive heart bleeding and ensure good sternal elevation.

Pectus excavatum is a common congenital chest wall deformities in children, presented with abnormal collapse of anterior chest wall. Thoracoscopic Nuss procedure is the present widely utilized clinical approach. Due to implanting a steel bar into the substernal area, operative organ injuries, postoperative pain, bar malposition, incomplete correction as well as risks of bar removal may occur. With the advancement of digital precision medicine and the interdisciplinary research of various disciplines and fields, there are more and more clinical applications and translations of these emerging applications represented by computational biomechanical simulations, three-dimensional printing and the latest absorbable biomaterial. They bring new opportunities and prospects for solving problems in the clinical treatment of PE as well as the improvement and development of patient-specific Nuss procedure planning.

Objective:To investigate hemodynamic characteristics of the modified Blalock-Taussig shunt (MBTS) with the preservation or ligation of ductus arteriosus (DA) based on computational fluid dynamics (CFD), thus to help preoperative surgery design and postoperative prediction.Methods:A patient with pulmonary atresia and patent ductus arteriosus was included. Patient-specific three-dimensional model was reconstructed and virtual surgeries of shunt insertion and ductus ligation or preservation were performed via computer-aided design (CAD). CFD was utilized to analyze hemodynamic parameters preoperatively and postoperatively based on patient-specific anatomy and physiologic data.Results:The preservation of DA competitively reduced the shunt flow but increased total pulmonary perfusion. The shunt flow and ductal flow collided with each other, causing large and complicated turbulence in pulmonary artery where lower wall shear stress and higher oscillatory shear index were distributed, as well as higher energy loss.Conclusion:The preservation of DA is riskier in hemodynamics which may lead to pulmonary over-perfusion, inadequate systemic perfusion and heavier cardiac burden, thus increasing the risk of heart failure and it seems to bring no benefit in terms of reducing risks of thrombosis.

Objective    To investigate the prognosis and risk factors of mild to moderate or moderate atrioventricular valve regurgitation (AVVR) after Fontan operation. Methods    A total of 34 patients with mild to moderate or moderate AVVR who accepted Fontan operation and atrioventricular valve (AVV) repair between 2004 and 2018 in our center were selected as an AVV repair group. The patients in the same period were matched as a control group by the ratio of 1 : 1-2. Finally 99 patients were included into this study, including 64 males and 35 females, with an average age of 63.4±36.3 months and weight of 17.3±6.7 kg. Grades of AVVR decreased more than 1 was defined as significant improvement. Endpoints of the study were death, Fontan takedown, AVV replacement. Risk factors including Fontan procedures, AVV repair procedures, cardiac anatomy were analyzed. Results    Patients were followed up for 1.5 (0.3-4.0) years. Overall mortality was 15.2%. Most (82.4%) of AVV repair group accepted single AVV repair procedure while partial annuloplasty was the most common (52.9%). With the extension of follow-up, the degree of AVVR in the whole group showed a gradually increasing trend (r=0.352, P=0.000). Mild to moderate AVVR improved spontaneously after Fontan operation, while moderate AVVR did not. AVV repair could improve the degree of AVVR after moderate regurgitation, without increasing the surgical mortality, and regurgitation significantly decreased in 8.8% patients. AVV repair was not effective for mild to moderate AVVR and would increase surgical mortality. Conclusion    AVV function shows a gradual downward trend after Fontan operation. AVV repair is effective for moderate AVVR, does not increase mortality, but the degree of improvement is limited. AVV repair is not effective for mild to moderate AVVR and increases surgical mortality.

To evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center. Methods    From January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed. Results    All patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ. Conclusion    According to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.

Objective    To evaluate the effects of anatomic correction for congenitally corrected transposition of the great arteries (ccTGA) and 10-year follow-up. Methods    From January 2008 to December 2018, 48 patients with ccTGA who underwent anatomic correction were reviewed. There were 29 males and 19 females with age of 39.2 (3-91) months. The cohort was divided into two groups: a biventricular anatomic correction group (39 patients) and a 1.5 ventricular anatomic correction group (9 patients). They were followed for in-hospital mortality, late mortality, long-term survival, freedom from reoperation, and heart function. Results    There were 3 early deaths and 2 early re-intervention in the biventricular anatomic correction group, but no death and only one re-intervention in the 1.5 ventricular anatomic correction group. Compared with the biventricular anatomic correction group, the operation time, tracheal intubation and ICU time were significantly reduced or shortened in the 1.5 ventricular anatomic correction group (P<0.05). The patients were followed up for 0.5-10.4 years. Four patients were lost. Two patients died in the biventricular anatomic correction group, and two patients received re-intervention. The 1-year, 5-year and 10-year survival rate was 88.2%, 84.0%, and 84.0%, respectively. There was no death or intervention in the 1.5 ventricular anatomic correction group. The quality of life of the other patients in the medium-term follow-up was satisfactory. Only 2 patients were classified as grade Ⅲ in cardiac function, and the other patients were classified as grade Ⅰ-Ⅱ. Conclusion    According to the different anatomic characteristics of ccTGA, the individualized strategy of anatomic correction can achieve satisfactory surgical results, and the medium-term quality of life was good. Especially, 1.5 ventricular anatomic correction may obtain better therapeutic effects because of its lower operative mortality and less postoperative complications.

Objective    To evaluate the effect of off-pump pulmonary valvulotomy for the patients with pulmonary atresia with intact ventricular septum (PA/IVS). Methods    The clinical data of 61 PA/IVS patients who underwent off-pump right ventricular decompression surgery in our hospital from January 2013 to September 2019 were retrospectively analyzed, including 37 males and 24 females, with an average age of 29.7 (2.0-86.0) d and weight of 4.1 (2.5-6.9) kg. Thirty-nine patients received off-pump pulmonary valvulotomy (an open-view valvulotomy group) and 22 patients received balloon valvuloplasty through the right ventricle pulmonary valve (a hybrid therapy group). The postoperative mortality, early re-intervention, and completion of final operation of the two groups were compared. Results    There were 2 deaths in the study with a mortality rate of 3.3% (2/61), and the mortality rate of the two groups was not significantly different (2.6% vs.4.5%, P=0.68). The rate of early re-intervention in the two groups was 5.3% and 19.0%, respectively (P=0.09). There was no statistical difference in intubation time (56.0±25.9 h vs. 62.0±28.9 h, P=0.41), ICU retention time (4.7±2.9 d vs. 5.5±2.2 d, P=0.23) and postoperative hospital stay time (3.9±0.9 d vs. 4.3±1.1 d, P=0.38) between the two groups. The follow-up time was 45.3 (4.0-84.0) months. There were 5 patients lost to follow-up. During the follow-up period, in the open-view valvulotomy group, 17 patients did not need further operation, 13 patients completed the final operation. In the hybrid therapy group, 7 patients did not need further operation, 8 patients completed the final operation. Heart function classification of all patients was in New York Heart Association class Ⅰ-Ⅱ. Conclusion    Compared with the hybrid therapy, off-pump pulmonary valvulotomy for PA/IVS also has the advantages of simple operation, short operation time and high survival rate, and it may be easier to be promoted in clinical application because of its more economic benefits and relatively lower re-intervention rate.

Objective:To investigate the diagnosis and treatment of congenital leukemia and the main factors affecting its prognosis.Methods:Clinical data of children with congenital leukemia admitted to Zhengzhou Children′s Hospital from July 2007 to December 2017 were retrospectively collected.Their clinical characteristics, chemotherapy and factors affecting their long-term survival were analyzed.Results:A total of 23 cases of congenital leukemia were collected, who mostly complained of " increased white blood cells, skin nodules and skin hemorrhage" . Among them, 10 cases abandoned the treatment, who all died according to the follow-up results.Among the 13 treated cases, 7 cases were male, and 6 were female.One of the 13 cases was a twin.Eight cases were acute myeloid leukemia, and 5 cases were acute lymphoblastic leukemia.One case did not perform the fusion gene test. MLL gene was positive in 2 cases and negative in 10 cases.No other fusion genes were detected.Karyotype analysis showed that 1 case was 21-trisomy and 12 cases had a normal karyotype.All 13 patients received symptomatic and/or reduced chemotherapy.The median follow-up time was 4 years and 11 months.Six cases recovered and 7 cases died during the follow-up.One case was diagnosed with acute lymphoblastic leukemia on the 6 th day after the birth in another hospital.He was not treated timely and came to Zhengzhou Children′s Hospital with general contract blocks at the age of 32 days.After checking, he was diagnosed with center leukemia and testicular leukemia.He developed a severe infection in the lung and brain after strong chemotherapy, and died even after the remote consultation with Lurie Children′s Hospital of Chicago.The other 6 cases died of severe infection and multiple organ injuries after chemotherapy, and no one died of leukemia relapse. Conclusion:Reduction chemotherapy may be an important treatment to children with congenital leukemia.

Objective:To investigate the pathology characteristics, so as to provide treatment experience of primary cardiac tumors for pediatric patients.Methods:A retrospective study was conducted for 135 patients with primary cardiac tumor between January 2004 and December 2017 in Shanghai Children′s Medical Center.The median age was 0.54 years (range, 0-14.36 years). Single tumor was discovered in 61 cases and multiple rumors were found in 74 cases.Forty-five patients presented obvious clinical symptoms, 4 patients with mild symptoms and 86 patients without symptoms.Forty-one patients underwent surgical treatment, including complete resection of the tumor in 24 cases, partial resection in 16 cases and heart transplantation in 1 case.Concomitant valvuloplasty was required in 13 patients.Ninety-four patients did not receive surgical treatment.Results:A total of 40 patients underwent surgical treatment in Shanghai Children′s Medical Center.Three patients died of low cardiac output (in-hospital mortality: 7.5%), and 2 patients recovered from postoperative low cardiac output.The hemodynamic status was stable in the remaining 35 cases.One patient who received heart transplantation in another hospital survived and had good cardiac function during 24 months follow-ups.There were no significant differences in the survival rate between partial resection and complete resection of benign tumors.One late death was observed in patients with malignant tumors for 2 years after operation.Surveillance was kept in 94 non-surgical patients, among whom 1 case died for the abandon of surgery and 1 case died when waiting for heart transplantation.Other 2 patients were waiting for heart transplantation and other 90 patients had no hemodynamic disorder.Conclusion:Most of primary cardiac tumors are benign and long time follow-up should be able to get on for pediatric patients.When patients develop hemodynamical obstruction, arrhythmia or malignant tumor, surgical treatment is necessary.The principle of surgical therapy in these patients is to restore normal hemodynamic status, instead of completely removing tumor.Heart transplantation is potentially the only way for patients whose tumor cannot be resected.