Objective To review and summarize the experiences of modified extracardiac conduit Fontan operation for heterotaxia syndrome with complex congenital heart disease. Methods There were 11 patients with cynosis complex congenital heart disease, 9 were aspleenia syndrome (right atrium isomerism, including 6 single ventricle with common atrioventricular valve, 2 double outlet right ventricle with atrioventricular discordance, and 1 corrected transposition of the great arteries), and 2 were polyspleenia (left atrium isomerism, double outlet right ventricle with common atrioventricular valve). The mean age was (6.3?3.7)year-old, the mean body weight was (21.0?5.5)kg. 3 patients underwent one-stage modified extracardiac conduit Fontan procedure, 8 patients after bi-directional Glenn operation underwent two-stage procedure. Results During early postoperative period, two patients had low cardiac output syndrome, 1 renal dysfunction; and 1 supraventricle tachycardiac. The time of chest drainage (120*!ml/d) was more than 10 days in 2 patients. 2D-echo showed that superior vena cava blood flow rate was 0.6 to 0.8 m/s, inferior vena cava flow rate was 0.3 to 0.4 m/s. Oxygen saturation were from 0.92 to 0.95 in room air in 9 patients, 2 patients were under 0.86. The exercise capacity was significantly improved. All patients were survived and no early death. At follow-up ranging from 6 months to 2 years, supra vena cava blood flow rate was 0.8*!m/s, inferior vena cava flow rate is 0.4 to 0.7*!m/s. There was no thrombus formation in the conduit. The diameter of fenestration was 0.34*!cm and blood flow from right to left. No pulmonary vein drainage obstruction. Heart function was normal. No atrial arrhythemia were detected. The oxygen saturation in the room air was more than 0.90. No chronic effusion and protein-losing enteropathy, no mid-term death. Conclusion Modified extracardiac conduit Fontan operation is suited for heterotaxia syndrome with complex congenital heart disease. The incidence of arrhythemia is low.

Purpose:To investigate the telomerase activity in human digestive tract cancer and its significance and evaluate usefulness of the TRAP ELISA method .Methods:Telomerase activity was examined in 112 tumor specimens, including the following cancers: esophageal(12), gastric(36), hepatic(15), pancreatic (11), colorectal(38) and paracancerous tissues(94) by TRAP ELISA method.Results:The telomerase activity in tumor tissues was significantly higher than in paracancerous and normal tissues ( P

Objective To evaluate accurately the cardiac output, an early post-operative indicator for the cardiac function, after cardiac surgery in the neonates with congenital heart diseases and assess the risk factors for surgery, with an aim at exploring the early strategy for decressing the mortality. Methods From January 2007 to November 2007, forty-seven consec-utive neonates with complex congenital heart diseases underwent surgical correction at Shanghai Children's medical center.There were 38 boys and 9 girls with age from 3 days to 29 days [mean age (21.98 +8.15) days] and weight from 2.6 kg to 4.2 kg [ mean (3.49±0.51 ) kg]. 15 patients had delayed sternal clousure. The operations were performed with hypothermia, deep hypothermia low flow, or deep hypothermia circulatory arrest techniques. Cardiac index (CI) was derived from pulse contour analysis and calculated with the PiCCO plus system. Meanwhile, serum cardiac troponin I ( cTnl), mixed venous oxygen saturation ( SvO_2 ) and cardiopulmonary bypass (CPB) time were measured. Finally, the risk factors for surgical treatment in survivors and that associated with an increased mortality were analyzed. The association between post-operative cardiac output and the death after surgery was examined. Results Four neonates died after surgery, with a surgical mortality of 8.5%. CIvalue in the neonates [ (2.0±0.3 ) liters per minute per square meter of body surface was less than normal 2.5±0. 3. CI was associated inversely with CPB time but had a positive correlation with SvO_2. No significant difference association between CI and cTnI was observed. The value of cTnl was associated with the type of surgical procedures. Cases for which DHCA and low flow cerebral pefusion technique were used were free from neurological complications, ischemia in the lower extremities and oli-guria. The duration of cardiopulmonary bypass, urgent state of the operation and the abnormal coronary artery were associated with high mortality in the neonates after cardiac surgery. Conclusion The cardiac output of the neonates in whom cardiac pro-cedures were performed is adequate for the the oxygen supply required by the whole body though it is slightly lower than nomad after cardiac surgery. Higher mortality in the neonates during early postoperative period may be due to the complexity of the primary diseases, prolonged cardiopulmonary bypass time, residual abnormalities and severe acidosis before operation. Various techniques for CPB and that for the myocardial protection are safe and can provide an appropriate operative field.

Objective To study the characteristics of neonatal sepsis caused by gram positive (G +) bacteria,gram negative (G+) bacteria and fungi.Method Clinical data of 202 neonates with sepsis hospitalized from Jan.2012 to May.2015 were studied.According to the different pathogens,202 neonates were divided into gram positive bacteria group,gram negative bacteria group and fungi group.The general information,clinical manifestation,laboratory examination and treatment outcome of the three groups were analysed with Chi square analysis,LSD,Fisher exact probability tests.Result A total of 202 cases of neonatal sepsis were recruited.The detection rate of gram positive bacteria,gram negative bacteria and fungi was 35.2％ (71 cases),56.4％ (114 cases) and 8.4％ (17 cases),respectively.Comparing with gram negative group and fungi group,gram positive group had older gestational age (36.0 ± 3.8 w,compared with gram negative 33.0 ± 3.9 w,fungi group 31.2 ± 3.2 w,P ＜ 0.05),larger birth weight (2 620 ± 925 g compared with gram negative group 1 999 ± 849 g,fungi group 1 595 ± 666 g,P ＜ 0.05),lower nosocomial infection rate (29.6％ compared with gram negative group 70.2％,fungi group 94.1％,P ＜0.05),lower rate of shock,blood glucose disturbance and thrombocytopenia (P ＜ 0.05).There was no statistics difference between the gram negative group and fungi group.Comparing with the other two groups,fungi group was older [20.0 (11.5,39.5) d compared with gram positive group 7.0 (2.0,17.0) d,and gram negative 10.0 (6.0,18.2) d,P ＜ 0.05].The rate of deep venous catheterization in fungi group was higher than that in gram positive group and gram negative group (88.2％ compared with gram positive group 25.4％,gram negative group 40.4％,P ＜0.05).The treatment course of fungi group was longer than that of the gram positive group and gram negative group [22.0 (12.0,37.5) d compared with the gram positive group 14.0 (10.0,17.0) d,gram negative group 14.0 (11.0,18.0) d,P ＜0.05].The incidence of apnea in the gram negative group was higher than that in gram positive group and fungi group (P ＜ 0.05).The rate of leukocytosis,leukocytopenia and elevated CRP were higher in gram negative and gram positive group (P ＜ 0.05).Conclusion The clinical manifestations and laboratory examinations in neonatal sepsis caused by different pathogens were different,which can help to early identification of different pathogenic infections.However,there is no specific indicators to differentiate neonatal sepsis caused by different pathogens.Early identification of the pathogen needs clinical acumen.

Objective To compare the clinical efficacy of covered and uncovered metal stents in treatment of malignant biliary obstruction. Methods 123 cases of malignant biliary obstruction from May 2003 to May 2014. The survival time, the stent patency rates, the effective and biochemical indexes between the two groups were analyze and compared, follow-up period ended in March 2015. Results The level of bilirubin, transaminase, alkaline phosphatase (ALP) was declined after the covered and uncovered metal stents placed by ERCP, and the results of the two groups had no statistical significance (P > 0.05). Also there was no significant difference of the incidence of postoperative cholangitis and the cumulative survival rate between the two groups (P > 0.05). One year survival rates was related to tumor types (r = -1.55, P < 0.05). Conclusions Covered and uncovered metal stents for malignant biliary obstruction have no statistically significant about remission of liver function, stent occusion and the cumulative survival rate.

Objective To review and summarize the experiences of mitral valve replacement in the children aged less than five years.Methods Twelve patients with moderate to severe mitral valve regurgitation (MR) or/and severe mitral valve stenosis (MS) were retrospectively study from January 2008 to December 2011,all of them suffered from severe heart failure and underwent mechanical mitral valve replacement.There were 9 males and 3 females.They aged from 4 to 58 months [mean (26.2 ± 18.1) months] and weighted from 5.6 to 13.0 kg [mean (9.6 ± 3.8) kg].Three patients underwent unsuccessful mitral valve repair before mitral valve replacement.17 to 23 mm aortic bileaflet mechanical prosthesis were reverse directional implanted in nine patients and 25 to 27 mm mitral bileaflet mechanical prosthesis were implanted in three patients.Results One patient died,the operative mortality rate was 8.3％.2 patients were complicated by cardiac arrhythmia and 2 patients complicated by mild hemolysis,all recovered after symptomatic treatment.The heart function of servived 11 patients improved significantly,none of them were complicated by haemorrhage or thromboembolic.Conclusion Severe mitral valve lesion injure heart function significantly for the young children and timely surgical intervention is the only option.Mitral valve replacement is the ultimate option for the patients failed in mitral valve annuloplasty.The introduction of aortic bileaflet mechanical prosthesis which smaller size is available and reverse directional implantation resolve the difficulty of prosthesis-patient mismatch essentially,most children with a small prosthesis has to redo mitral valve replacement with a larger size prosthesis.The compliance of permanent anticoagulation of young children is favourable and more intensively follow-up is essential to avoid the incidence of bleeding and thromboembolic.

Objective: To explore the surgical strategy for Ebstein anomaly in children. Methods: From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm. The tricuspid valve regurgitation (TR) were mild in 26 cases, moderate in 46 cases and severe in 69 cases. Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction, 37 valve hoist), tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases. Surgical strategy were divided into biventricular heart function in 77 cases, one and a half ventricular heart function in 56 cases, and single ventricular heart function in 8 cases. Results: Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period. One case died in biventricular group. The complete atrioventricular block were occurred in 3 patients and pacemaker were applied. One hundred and forty cases discharged from hospital. There were mild TR in 118 cases, moderate in 14 cases and closed in 8 cases. One hundred and thirty-seven cases were followed up regularly in 18 to 172 months. Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases, moderate in 15 cases and severe in 1 case). Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases, moderate in 12 cases and severe in 3 cases). In the patients with severe TR (4 cases), 3 cases were reoperated by cone reconstruction. One case′s valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function, the oxygen saturation was increased. Two patients underwent tricuspid valve replacement, 1 died and the other′s mechanical valve was removed, and changed to single ventricular function repair. Conclusions Although tricuspid cone reconstruction can achieve good results, the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair. To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia, transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.

Objective To study the seropositive ratio of the antibody to aquporin 4 (AQP4-IgG) and myelin oligodendrocytes glycoprotein antibody(MOG-IgG)in patients with autoimmune-associated central nervous system (CNS) diseases. Meanwhile, epidemiology and clinical manifestation and diagnosis,laboratory examination and magnetic resonance imaging(MRI)of AQP4-IgG seropositive and MOG-IgG seropositive patients are described. Methods 2068 patients serum samples were collected and enrolled in the multi-center research. The methodology of cell-mediated immunofluorescence staining was used to detect serum AQP4-IgG and MOG-IgG. Clinic medical records were collected and characteristics of epidemiology and manifestation were compared. Results 681 patients were AQP4-IgG seropositive and 110 patients were MOG-IgG seropositive. The female/male ratio and age of onset of patients with AQP4-IgG seropositive(616 female and 65 male,female:male=9.50:1.00;Age of onset=41.7±14.9)were significantly higher than that of patients with MOG-IgG (57 female and 53 male, female:male=1.08:1.00, P<0.0001; Age of onset=27.0 ±17.7, P<0.0001). The optic neuritis was significantly higher in patients with AQP4-IgG seropositive and patients with MOG-IgG seropositive (38.4% vs.53.5%, P<0.05).Among patients with AQP4-IgG seropositive, 42.14% conformed the diagnostic criteria of neuromyelitis optica (NMO),which was higher than that of patients with MOG-IgG seropositive (13.64%, P<0.0001). Laboratory examination showed that there was no significant difference in cerebrospinal fluid protein levels between patients with AQP4-IgG seropositive and those with MOG-IgG seropositive.MRI imaging suggested that AQP4-IgG positive patients were more common in cervical thoracic spinal cord lesions, while MOG-IgG positive patients were more involved in thoracolumbar spinal cord. The study also found that these two groups of patients could be comorbid with other autoimmune antibodies. Conclusions This multi-center research has revealed that patients with AQP4-IgG seropositive and those with MOG-IgG seropositive display differences in epidemiology,clinic manifestations and diagnosis,laboratory examination and MRI imaging. AQP4-IgG and MOG IgG auto-antibody detection are necessary for clinic diagnosis and differential diagnosis.

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.