Objective To observe the change of lipid metabolism during adaptive heart failure.Method The transverse aorta constriction mouse models established by minimal invasive surgery technique were evaluated for early small changes of body composition by EchoMRITM whole body composition analysis,and the different gene expression was analyzed by whole genome expression microarray.Results Three weeks after the surgery,the fat composition,in the mouse models were (0.49 ± 0.13) g,which was significantly lower than that in the sham surgery group [(0.81-±0.14) g] (P =0.002).The expression of the natriuretic peptide type A significantly increased over time (R2 =0.939,P =0.005).In addition,the levels of fatty acid desaturase,long-chain fatty acid elongase 5,and N-acylsphingosine amidohydrolase increased,and the levels of acyl-coenzyme A dehydrogenase,muscle glycogen phosphorylase,and pyruvate dehydrogenase kinase decreased,showing increased fat mobilization and decreased carbohydrate utilization.Conclusions Surgery for transverse aorta constriction can cause complex metablic changes in heart.The activated lipid mobilization maybe lead to the low body fat composition.

Objective Evaluate the outcome of total anomalous pulmonary venous connection (TAPVC) repair in newborn,controlling for anatomic subtypes and surgical technique.Methods Between 1999 and 2011,68 patients (median age 16 days) underwent repair for supracardiac (21),cardiac ( 8 ),infracardiac ( 36 ) or mixed ( 3 ) TAPVC.All patients were emergencies,due to obstructed drainage.Supracardiac and infracardiac TAPVC repair included the side-to-side anastomosis between the pulmonary venous (PV) confluence and the left atrium.Coronary sinus unroofing was preferred for cardiac TAPVC repair.Results Early mortality was 2.9％ (2/68).The echo showed no obstruction in the pulmonary vein anastomosis and flow rate was 1.1 m/s ～ 1.42 m/s in the follow-up of 3 years.Side-to-side anastomosis provides excellent results for TAPVC repair while left atrial enlargement procedures appear to be associated with higher risk of late arrhythmias.Although early and aggressive reintervention for recurrent PV obstruction is mandatory,intrinsic PV stenosis remains a predictor of adverse outcome.The incidence of pulmonary vena is gradually reduced to 6％ ～ 11％.This often occurred in the infracardiac or mixed TAPVC.Conclusion The nicety of preoperative diagnose,the improvement of protection of heart function,using of pulmonary vena tissue for anastomose and avoiding of distortion of pulmonary venues and delayed closure of stemum can reduce the mortality.The preoperative degree of pulmonary veno obstruction and the time of emergency operation and the infracardiac or mixed TAPVC can affect prognosis.Along with the surgical technique,the mortality of TAPVC is gradually reduced and the result is amazing,but it is important to attach importance to the patient with re-stenosis of pulmonary veno,the time and method for reoperation.

Objective Reviewed and evulated the prognosis of multiple levels of left ventricular outflow tract obstruction (LVOTO) with Konno operation.Methods Between May 2006 and May 2012,we performed Konno operation in 16 children with LVOTO.They aged from 1.9 to 13.6 years,averaged(5.1 ± 0.7)years,and weighted from 12 to 39 kg,averaged (18.3 ±6.3) kg.There are 13 cases of patients who were multiple levels of LVOTO.The pressure gradient of LVOTO was 56-185 mmHg[(96 ± 31) mm Hg].The operations were performed with middle low temperature in cardiopulmonary bypass (CPB).The time for CPB and Aortic clamp arrest was 70-182 min [(98 ± 21) min] and 34-148 min[(51 ± 11) min] respectively.There were 3 cases,4 cases and 9 cases for Konno,Konno ± Ross and Konno-Rastan respectively.Results All the pacients were alive.The time for making use of respirometer and staying in ICU was 3-9 days [(4.0 ± 1.5) days] and 5-16 days [(8.0 ±2.9) days] respectively.Follow-up with 6 months to 3 years,the ECHO showed no residual obstruction.The pressure gradient of LVOTO was 1.10-2.42 m/s.LVEF was 0.58-0.72 (0.66 ± 0.03).There were 2 cases for mild to middle aortic valve regurgitation,1 case for middle to severe aortic valve regurgitation and 1 case for middle pulmonary valve regurgitation and middle tricuspid valve regurgitation.Conclusion Konno procedure is safty and effective operation for LVOTO in children and is beneficial to the recovery of left ventricular function.It is necessary to follow up the complication in the future.

Objective Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare complicated congenital heart disease.The intracardiac structure is also complicated.We sum up the corporal surgical procedure of PA/IVS.Methods Between May 2004 and May 2010,we performed emergency or sub-emergency operation in 72 newborns,infants and children [age,1 day to 5.2 years; mean age,(1.3 ±0.6) years] with favorable anatomy.The surgical procedures were divided into three kinds:1.16 cases were performed with Blalock-Tassuing (B-T) shunt operation for A group; 2.33 cases were performed with transannular patch from RVOT-MPA with or without B-T shunt operation for B group.3.22 cases were performed a hybrid procedure with or without B-T shunt operation for C group.From all the patients,15 cases had been finished the two staged operation.Results 10 patients has been died in which 6 patients were in B group with B-T shunt.Only 2 patients were for the hybrid procedure.The ECHO showed the gradient across the pulmonary annular was 15-39 mm Hg [(23 ± 5) mm Hg (1 mm Hg =0.133 kPa)] after operation.The tricuspid insufficient had been siginificantly improved.There were 58 cases were preoperatively serious tricuspid insufficient.There were 11 cases and 13 cases were respectively for middle and mild to middle tricuspid insufficient postoperatively.Follow-up with 2-5 years,there were radical operation for 8 cases,one and half ventrical operation for 5 cases and BDG and Fontan operation for 2 cases.Conclusions.Conclusion BT shunt in common procedure and using a hybrid approach is more safe and feasible than traditional procedure for the patients with PA/IVS.Z vale and right ventricular growth were importment for procedure in the future including radical 、one and half ventrical and Fon tan operation.

ObjectiveTo review and summarize the experiences of the modified mitral valve annuloplasty for mitral valve regurgitation (MR) in children.MethodsOne hundred and six patients with moderate to severe MR were retrospectively studied from March 1999 to December 2009.They aged from 0.4 to 8.5 years [ mean (3.7 ± 1.8) years ]and weighted from 6.6 to 52.0 kg [ mean(10.0 ± 3.5) kg].There were 69 males and 37 females.The heart function was evaluated by echocardiography.Patients were divided into three groups according to the age: group 1, ＜6 months , 16 cases; group 2, from 6months to 2 years, 51 cases; and group 3, from 2 years to 8.5 years, 39 cases.Seven cases were MR combined with mitral valve stenosis. Different operations were performed according to preoperative homodynamic, heart function and anatomical structure.ResultsThe operative mortality rate was 2.8％.In 3 died patients, 2 were due to MR and 1 due to severe pulmonary hypertension.104 cases were recovered.All patients were followed up 3 months to 3 years.ConclusionModified mitral valve reconstruction is necessary for children with moderate to severe MR.It should be the first choice for MR patients associated with other cardiac malfomation.This technique could prolong the time for mitral valve replacement when needed.Moreover,this technique not only reduces the valve injury but also decreases the reoperation rate.

Objective Sum up the research of concept of surgical procedure of PA/VSD/MAPCA.Methods Between January 2003 and December 2012,92 children were operated,aged 1 day to 5.2 years ; median age (1.3 ± 0.6) years.There were 26 cases for one stage operation,66 cases were for palliative operation which were divided into 3 stages.The 3 stages of palliative operation included system to pulmonary shunt,conduit from RVOT to MPA or transannular patch from RVOT to MPA,enlargemene of pulmonary artery and ligation of MAPCA.From all the patients,15 cases had been finished the two staged operation.In the end,there were 17 cases who were completed two or three stage radical operation.Results There were 3 cases who died from the first period.There were 9 cases who were completed two stage operation,and 1 case died.There were 8 cases who were completed three stage operation.there was 1 cases who died in this period.Conclusion The surgical treatment for the PA/VSD/MAPCA could be that the connection between RVOT and pulmonary artery should be performed as early as possible which could provide the blood flow to pulmonary artery and promote the growth.Uniforcalization could also be set up as early as possible if it is needed.It could be ligated in different period.The PA/VSD/MAPCA could be repaired by stagesand have satisfied results.

Objective To compare the results of surgical repair for congenitally corrected transposition of the great arter-ies(ccTGA) with pulmonary stenosis(PS), and to analyze the risk factors that may affect early results and surgical technique. Methods From Aug. 2001 to Dec. 2008, 21 patients with ccTGA and PS were treated in our hospital. They aged 3.5 months to 6.3 years [(31 ± 18) months], weighted 6.28 kg [(13.1± 6.5) kg]. Fifteen cases had paramembranous ventricular sep-tal defect (VSD), 1 noncommitted VSD and 5 subpulmonary VSD. The repairs comprised of Senning and Rastelli operation. RV-PA reconstruction was done by xenopericardial conduit in 13 patients. RV-PA direct anastomosis plus pericardial patch in 6 patients and homograft patch with autopulmonary valve in 2 patients. Results All the patients were alive. During 2 - 5 years follow-up, blood flow rate in superior vena cava increased to 1.8 - 2.2 m/s in 3 cases. One of them needed reoperation to re-lease the stenosis and the rest 2 were in follow-up. Tow patients had right ventricular outlet obstruction with a pressure gradient of 30 -45 mmHg were in follow-up. There was no other stenosis and valve insufficiency. Conclusion Double-Switch opera-tion is practical and impressive in treating of CCTGA with PS. It is important to evaluate the size of VSD and the reconstruction of RV-PA. But it still need more cases and longer time to evaluate the long-term effects.

Objective Evaluating a new continuity between the right ventricle and the pulmonary artery for repair of persistent truncus arteriosus(PTA).Methods Between January 2000 and December 2012,we performed operation of 53 children with without a conduit:sleeve and direct right ventricle-pulmonary artery anastomosis [age,40 day to 2.3 years; median age,(0.9 ± 0.5) years] in 86 cases of PTA.The other 33 cases were performed with valved conduit.we used different methods to form the posterior floor of the new pulmonary arterial trunk.The edge of the floor was attached directly to the superior margin of an oblique incision made in the left-anterior wall of the right ventricle.A Gortex monocusp was attached to the lower half margin of the right ventricular incision in 26 cases.Results There were high pulmonary hypertension (PH) in 6 cases with the treatment of anti-PH.There were 2 death postoperatively because of pulmonary hypertension and right heart disfunction.The other patients were discharged from hospital.Cardiac ultrasound at hospital discharge showed pulmonary regurgitation that was trivial in three cases,mild in twentyseven cases,and moderate in twentyone case.During follow-up(36-60 months,median 54 months),there were no death.Conclusion This simple modification for surgical correction of persistent truncus arteriosus may be an effective alternative that overcomes conduit-related problems from the mid-followup period.

Objective To analyze the influencing factors of surgical management for neonatal complicated congenital heart disease(CCHD),sumarize the clinical experience and improve the surgical effect.Methods The clinical data of 154 neonates with complicated congenital heart disease who were undergone surgical management from August 2008 to August 2013 were retrospectively analysed.Results There were 14 cases of death with a mortality rate of 9.1％ (14/154),including 4 cases of preoperative death.The operative motality is 6.7％ (10/150),including 3 cases of single malformations(D-TGA/VSD,PA/VSD,IAA),and 7 cases of multiple malformation(HRHS/PS in 2,D-TGA/COA in 1,D-TAG/PS in 1,COA/SV in 1,SV/PA/IVS in 1 and D-TGA/COA/SV in 1).140 cases accepted regular follow-up after hospital discharge from 3 months to 5 years,8 of them underwent secondary surgery and all survived.Conclusion Timely diagnosis,actively improvement for preoperative internal environment,fine operative technique,individual cardiopulmonary bypass and intensive postoperative care are all important influencing factors of surgical effect for neonatal severe complicated congenital heart disease.

Objective To review and compare various types of operations for congenitally corrected transposition of the great arteries (ccTGA),to provide more suitable surgical procedure and improve surgical results.Methods Analysing 203 patients with ccTGA between June 1999 and June 2014,there were thirten patients who had undergone palliative procedure.Thirty-nine patients had received conventional repair operation.Eighty-eight patients had received double switch operation in which there were three patients of Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure.Sixty-three patients had received other surgical treatments including functional univentricular repair operation.Results There were 13 hospital deaths in all patients,consisted of 2 by conventional repair operation,9 by double switch operation and 2 by other procedure.Conclusion The operative procedure depend on the anatomy and physiology in ccTGA.The double switch operation have relatively higher mortality,more complication,the indication of Double Switch With Hemi-Mustard and Bidirectional Glenn Procedure should be more acceptable,but the long-term outcomes will be followed-up.