Objective To summarize perioperative care and effect of surgical treatment of total anomalous pulmonary venous connection (TAPVC) in newborns and babies within 6 months.Methods From September 2001 to May 2013,ninetytwo consecutive patients with TAPVC received surgical correction under cardiopulmonary bypass (CPB) with moderate or deep hypothermia.The anatomic subtype included supracardiac type 48,intracardiac type 36,infracardiac type 6 and mixed type 2.There were 36 newboms.Pathological diagnosis was made by echocardiogram,computerized tomography and operative findings during operation.The option of procedure was determined by findings.Respirator assisted breathing after operation and cardiac function was maintained by a variety of positive inotropic drugs,to prevent the happening of the pulmonary hypertension crisis.Results Severely low cardiac output syndrome occurred in 8 patients and they died within 24 hours after operation.The remaining cases postoperative recover smoothly and receive postoperative follow-up about 1 month to 12 years,3 cases of postoperative pulmonary vein stenosis (PPVS) occurred.Conclusion Total anomalous pulmonary venous connection should be surgical treatment as soon as possible with satisfied effect.The key of perioperative care is maintenance of left heart function,negative balance of liquid,prevention and treatment of pulmonary hypertension.

Objective This study was designed to investigate the correlationship between plasma metastin and pathogenesis of adolescent women with polycystic ovary syndrome(PCOS).MethodsFrom Jan 2006 to Jun.2006.42 PCOS patients including 19 adolescent women and 23 adults with syndrome were treated in Second Affiliated Hospital of Sun Yat-Sen University.According to the range of age,those patients were divided into 19 cases in adolascent group(≤19 years)and 23 cases in aduh group(＞19 years).Meanwhile,20 adolescent women were matched as controls.Blood samples were collected between day 1 and day 5 of a spontaneous bleeding episode in the PCOS groups and a menstrual cycle of the controls.The Jevels of luteinizing hormone(LH)，follicle-stimulating hormone(FSH)，testosterone(T)，free T(FT)，dehydroepiandrosterone sulfate(DHEAS)，sex hormone-binding globulin(SHBG)，insulin，glucose，and metastin were detected from day 1 to day 5 of spontaneous bleeding or withdrawal bleeding by progesterone.On the next day,oral glucose tolerance test(75 g)and insulin release test were performed on those above patients and controls.The area under carve(AUC),the ratio of fasting blood glucose to insulin(GIR)and homeostasis model assessment insulin resistance jndex ( HOMA-IR)were calculated.Results(1)The level of hormone:the level of LH was in(12±7)U/L in adult group and(12±8)U/L in adolescent PCOS group，which were significantly higher than(6±4)U/L in controls(P＜0.05).The level of FT was(2.3±1.2)pmol/L in adult group，which was significantly higher than(1.3±0.8)pmol/L in adolescent group and(1.1±0.5)pmol/L in control roup(P＜0.05).It was observed that the level of(3.1±2.7)μmol/L in adolescent group was significantly lower than(6.3±2.7)μmol/L in control group(P＜0.05).Meanwhile,the level of FAI of 5.6±4.1 in adult group was significantly higher than 3.0±1.3 in control group(P＜0.05).No significant difference in FSH，T and SHBG levels among three groups were observed (P＞0.05).(2)Metastin and metabolism:Both the levels of fasting blood insulin,2-hour insulin and AUC of insulin were(13±7)mU/L，(88±59)mU/L and(133±80)mU·L-1·min-1 in adolescent group，which were significantly higher than(7±3)mU/L，(57±29)mU/L and(82±34)mU·L-1·min-1 in control group.The fasting blood insulin of(13±7)mU/L in adolescent group was significantly higher than (9±5)mU/L in adult group.The level of fasting blood glucose and 2-hour glucose were(5.01±0.44)mmol/L and(6.48±1.16)mmol/L in adult group，which were significantly higher than(4.68±0.29)mmol/L and(5.44±0.83)mmol/L in control group and(4.67±0.30)mmol/L and(5.93±1.44)mmol/L in adolescent group.The glucose AUC of(9.99±1.85)mmol·L-1·min-1 in adult group was significantly higher than(8.42±1.53)mmol·L-1·min-1 in control group(P＜0.05).HOMA-IR of 2.6±2.0 in adolescent group was significantly higher than 1.4±0.7 in control group.GIR of 10±8 in adolescent group was significantly lower than 16±10 in control group(P＜0.05).The metastin level of (0.25±0.19)pmol/L in adolescent group and(0.29±0.29)pmol/L in adult group were all significantly higher than(0.18±0.23)pmol/L in control group(PPh glucose were observed(r=0.256，0.286 and 0.267.P=0.044.0.025 and 0.043).Conclusions The expression of metastin in adolescent PCOS women was significantly higher that of normal adolescent women The increased level of metastin might be associated with pathogenesis of adolescent women with PCOS.

Objective To investigate the effect of anti-Mlllerian hormone (AMH) on hormone secretion and P450 aromatase mRNA expression from cultured human luteinized granulosa cells. Methods Human luteinized granulose cells were derived from 10 patients treated by in vitro fertilization-embryo transplantation (IVF-ET) in the Second Affiliated Hospital of Sun Yat-sen University from June to December 2006. Granulose cells were divided into group A, B, C, D, E depending on different concentration of AMH,testosterone group and blank group. 1×10-7moL/L testosterone and 1,5,10,20,50 μg/L AMH were added into the culture medium of group A,B,C,D and E. 1×10-7mol/L testosterone was added into the culture medium of testosterone group while no other ingredient was added into the medium of blank group. Estrogen levels in supernates were measured at 24,48,72 hours after cell incubation. RT-PCR was performed to detect the P450 aromatase mRNA expression in group B, C, D, E and testosterone group at 72 hours after cell incubation. Results (1) Estrogen levels in supernates of granulose cell culture at 24,48,72 hours were (8.529±0.381)×104, (10.977±0.436)×104, (13.309±0.506)×104 pmol/L in group A, (7.027±0.276)×104, (9.167±0.300)×104, (10.794±0.555)×104 pmol/L in group B, (6.039±0.226)×104,(7.585±0.548)×104, (8.797±0.518)×104 pmol/L in group C, (5.118±0.460)×104, (5.716±0.496)×104, (6.205±0.667)×104 pmol/L in group D, (4.932±0.148)×104, (5.323±0.184)×104,(5.629±0.212)×104 pmol/L in group E. When compared with blank group [(0.001±0.001)×104,(0.006±0.003)×104, (0.029±0.011)×104 pmol/L], the statistical differences were observed in group A,B,C,D,E(P<0.01) ; when compared with testosterone group [ (8.418±0.569)×104, (10.841±0.689)×104, (13.301±0.637)×104 pmol/L], the statistical differences were observed in group B,C,D and E(P<0.01) ; statistical differences were also observed in group C, D and E when compared with group B, and also group D and E when compared with group C(P<0.01). No significant difference was observed between group D and E (P>0.05). In group A, B, C, D, E and testosterone group, the estrogen levels at 24 hours after cell culture were significantly lower than those at 48 and 72 hours (P<0.01) ; statistical difference was observed between estrogen levels at 48 and 72 hours(P<0.01). No significant difference was observed among 24,48 and 72 hours in blank group (P>0.05). (2) Relative ratios of intensity of P450 aromatase/β-actin at72 hours of cell culture in group B,C,D and E were 0.6148±0.0046, 0.5156±0.0012, 0.4698±0.0027 and 0.4282±0.0017, respectively, which were statistically lower than that in testosterone group (0.8224±0.0021, P<0.01) ;statistical differences were also observed in group C, D and E when compared with group B, and also group D and E when compared with group C(P<0.01). No significant difference was observed between group D and E (P>0.05). Conclusion It is suggested that AMH might affect estrogen synthesis by inhibiting P450 aromatose activity so that lead to hyperandrogenism microenvironment in local ovary.

Objective To sum up the clinical information and outcomes of one-stage surgical repairs for interrupted aortic arch (IAA) associated with cardiac defects through median stemotomy in infant.Methods From August 2005 to January 2012,23 IAA patients,aged 18 days to 3 years [mean age (8.61 ± 11.81) months],body weight 3.3-13.0 kg [mean (6.61 ± 3.26) kg] were reviewed underwent one-stage repair.There were 12 male and 11 female.The anatomic subtype ineluded type A 20 (87.0％),type B 3 (13.0％),and no type C in the records.All cases included ventricular septal defect and patent ductus artefious,some also with artirical-pulmonary window,aterical septal defect,bicuspid or unicuspid aortic valve,and subvalvular aortic stenosis.Diagnosis was determined in of the patients and suspected in by echocardiography.Also,64 layers helico-CT was employed to make a definite diagnosis for 18 patients and cardiac catheterization was used for 4 patients.All patients with cardiac anomalies underwent one-stage repair through median sternotomy.The aortic continuity was reestablished by anastomosis between the descending aortic segment and aortic arch.Results In all 23 cases,21 were successful.There was 2 (8.69％) postoperative death:one was due to surgical hemorrhage and severe low cardiac output during perioperative stage and the other was 2-month old due to crisis of pulmonary hypertension.CPB time was ranged from 53-215 min [(129.76 ± 38.98) min],and aortic crossclamp time was 34-125 min[(74.47 ± 24.30) min].The length of stay in ICU postoperatively was 96h averagely.The postoperative complications included severe low cardiac output syndrome in 3 patients,hypoxemia in 13,pneumonia in 7,and supraventricular tachycardia in 12.21 patients were followed up from 2 months to 6 years and were in good condition without recoactation.Conclusion The outcomes of early and medium term for one-stage repair of IAA and associated cardiac anomalies through median stemotomy is excellent.Technique of extended anastomosis between the descending aortic segment and aortic arch may reduce the incidence of recoarctation.It is simplified the procedure and improved life quality of patients.

Objective To analyze the operation of long-term effect of edge to edge mitral repair for mitral vavle moderateto-severe insufficiency in children retrospectively.Methods Clinical dates of 102 patients who underwent edge to edge mitrel valve repair in Nanjing Children's Hospital from March 2003 to March 2015 were collected.Postoperative thoracic echocardiography was adopted to observe the effect of mitral valve forming and evaluate the follow-up results.Results The whole group of 2 patients died in hospital,Followed up for 1 months to 12 years,during the follow-up of 10 patients were lost.The early fol low-up,medium-term follow-up and long-term follow-up were completed 93,55 and 26 cases,respectively.In the early followup,6 patients had recurrent moderate mitral regurgitation(MR),and 5 patients had recurrent moderate-to-severe MR and reoperated during the follow-up period,a total of 2 cases died.In the medium-term follow up,moderate and severe MR were found in 6 and 4 cases,respectively.Three patients accepted reoperation,two patients died.In the long-term follow-up,1 case had recurrent severe MR,2 cases had re-operation and had no death.Early survival rate was 97.8 ％ (91/93),avoid reoperation rate was 94.6％ (88/93).The incidence of moderate or severe mitral regurgitation was 11.8％ (11/93).Midtcrm survival rate was 96.4％ (53/55),avoid reoperation rate was 94.6％ (52/55),moderate or severe mitral regurgitation rate was 18.2％ (10/55).Long-term follow-up have no death,avoid reoperation rate was 92.3 ％ (24/26),mitral valve moderate and above reflux rate was 7.7 ％ (2/26).The actuarial survival and freedom from MV reoperation rate was (92.4 ± 2.4) ％ and (77.7 ± 6.4) ％ at 10 years,respectively.Conclusion Edge to edge mitral repair in the treatment of children with moderate to severe mitral insufficiency had relatively stable curative effect,relatively fewer complications,and high long-term clinical effect.

Objective To investigate the effect and feasibility of early enteral nutrition support on postoperative nutrition assessments and clinical outcomes in children patients with congenital heart disease. Methods From October 2013 to October 2014, a number of 100 cases congenital heart disease aged six months to two years old were treated with early enteral nutrition support after operation. According to gender, fifty patients were randomly divided into the intervened group, who were treated with the early enteral nutrition support program. The other fifty patients were divided into control group with no nutrition sup-port. The detailed early enteral nutrition support project were( a) withdraw breathing machine at the same day, giving enteral nutrition 6 hours later after postoperation;( b) patients assist with long-term breathing machine, giving enteral nutrition 12-24 hours later after postoperation. The age, heigth, weight, serum C- reactive protein(CRP), serum retinoic binding protein ( RBP) , serum prealbumin( Pre-ALB) were recorded before operation. Seven days after operation, above indicators were ob-served again. In addition, the first time of excrete, the number of feeding interruption, the time of feeding, the time of ventila-tor, and the related complications were also recorded at hospitalization period. Results No difference of basic information and accompanying complications were observed between control and nutritional intervention group. As to nutritional status, weight-for-age z-score(WAZ) were significant higher in the nutritional intervention group than the control group( -0. 22 ± 1. 16 vs. 0.73 ±1.29, P=0.019) after operation. However length/height-for-age z-score(LAZ/HAZ) and weight-for-length/height z-score( WLZ/WHZ) were similar between control and nutritional intervention group whatever pre-operation and postoperation. Preoperative CRP, RBP, and Pre-ALB were no significant difference between early enteral nutrition and control group. After operation CRP levels in the early enteral nutrition group were significantly lower than that of control group[(45.2 ±16.2)mg/L vs.(67.3±35.5)mg/L,P<0.001],whileRBP[(0.3±0.1)mg/Lvs.(0.2±0.1)mg/L]andPre-ALB[(35.2±12.2)g/Lvs.(25.2±14.2)g/L] weresignificantlyhigherthanthoseofcontrolgroup(Pvaluewere0.031and0.029,respective-ly) . In the early nutritional intervention group and control group, the first time of excrete were remarkable in advance in nutri-tionalinterventiongroupcompredtocontrolgroup[(36±12)hvs.(65±15)h,P=0.008],whilethedifferenceoffeeding interruption times, intensive care unit( ICU) time and mechanical ventilation time in the two groups were not statistically signif-icant(P>0. 05). Conclusion Early nutritional intervention can help gastrointestinal function, enhance nutritional status, lower serum CRP levels and increased serum RBP and Pre-ALB concentrations. It did not add ICU time and ventilation time.

OBJECTIVETo compare the efficacy of thoracoscopic surgery versus thoracotomy in repairing esophageal atresia type Ⅲ with tracheoesophageal fistula (EA/TEF) in neonates.

METHODSA retrospective analysis was conducted in 97 neonates who underwent EA/TEF repair between January 2012 and December 2017 in the Affiliated Children's Hospital of Nanjing Medical University, including 75 patients receiving thoracotomy and 22 patients receiving thoracoscopic surgery. The perioperative data and the incidence of early postoperative complications were compared between two groups.

RESULTSThe operations were completed in all patients. One child (4.5%) in thoracoscopic surgery group was converted to thoracotomy with modified Livaditis procedure due to the long distance of two blind ends (>4 cm) and thinner distal end. The operation time was longer in thoracoscopic surgery group[(143±48) min vs. (120±40) min, <0.05], but the postoperative ventilation time was shorter[(55±22) h vs. (65±19) h, <0.05] and the first oral feeding was earlier in thoracoscopic surgery group[(3.2±1.1) d vs (3.9±1.3) d, <0.05]. No statistical difference was observed in the ratio of red blood cell transfusion, length of hospital stay and drainage tube indwelling time between two groups (all >0.05). The incidence of lung complications in thoracotomy group was higher than that in thoracoscopic surgery group (20.0% vs. 9.1%, <0.01), while there were no significant differences in the incidence of other postoperative complications between two groups. There was no death in thoracoscopic surgery group, while 2 patients died in thoracotomy group.

CONCLUSIONSThoracoscopic repair is a preferred surgical procedure for EA/TEF in neonates.