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.

Objective To studiy the surgical outcomes of children with congenital aortic stenosis (AS),and summarized the experience of surgical procedures.Methods From February 2006 to November 2011,a total of 49 consecutive children with AS underwent surgical treatments.Twenty-nine patients were male and 20 patients were female.The median age was 17 months (1 month to 14 years),and median weight was 15.6 kg (3.2-47.0 kg).Peak AS gradients was (74.9 ±20.4)mmHg (45-123 mm Hg) before surgery.Fourteen patients had isolated congenital AS,4 had combined moderate-to-severe aortic insufficiency (AI).Thirty-Three patients had associated cardiac anomalies,including ventricular septal defect,atrial septal defect,patent ductus ateriosus,coarctation of aorta,subaortic stenosis,and supravalvular mitral stenosis.Thirty-one patients underwent aortic valvotomy,9 valvuloplasty,2 Aortic valve replacement (AVR),1 Ross procedure,2 Ross-Konno procedure,and 4 Konno/AVR.Median follow-up was 20 months (2-55 months).Clinical and echocardiographic follow-up data were analyzed.Results There was 1 postoperative death who died of heart failure due to severe mitral valve insuffciency.Latest follow-up data showed that the survivals performed in satisfactory cardiac function.Left ventricular ejection fraction (LVEF)was 0.69 ±0.10,and fraction shortening (FS) was 0.38 ±0.09.Peak AS gradients decreased significantly after surgery to (38.6 ± 15.8) mm Hg (20-73 mm Hg),P ＜ 0.001.One patient needed reintervention because of severe recurrence of adhesion in the commissure 3 months after the previous surgery.Totally 31 patients underwent simple valvotomy procedure.Postoperative AI was none or trace in 5,mild in 20,and moderate in 6.Two surgical techniques were used to repair the aortic valve according to the anatomy of the valve and the cause of insufficiency,including commissuroplasty in 6 and pericarial casp extension in 3.Nine patients underwent valvuloplasty procedure,post-operative AI was none or trace in 3,mild in 5,and moderate in 1.We put more attention to the 7 patients who had moderate AI valvotomy or valvuloplasty,and the latest follow-up showed that these patients were in good condition and there was no deterioration the degree of AI.Conclusion Long-term outcomes show that aortic valvotomy and valvuloplasty are reliable and effective approaches for congenital AS in children.When moderate-to-severe AI exists,other procedures such as Ross,Ross-Konno,AVR,or Konno/AVR are preferred.

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 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 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 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 A retrospective review of the use of delayed sterna closure (DSC) after pediatric cardiac operations.The purpose of this study is to access the morbidity of DSC and to analyze the risk factors that may predict the need for DSC.Methods Between January 2008 and December 2011,5 546 infants (2 843 males,2 703 females) underwent cardiac operations through midterm sternotomy in Shanghai Children' s Medical Center.Median age was 5 months(1 day to 11 months) and mean weight was 6.1 kg(2.0-12.5 kg).The pathologies included transposition of great artery(TGA),corctation of aorta (CoA),interruption of aortic arch (IAA),pulmonary atresia (PA),truncus arteriosus (Truncus),complete atrioventricular septal defect(CAVSD),total anormalous pulmonary vein connection (TAPVC),double outlet of right ventricle(DORV),tetralogy of Fallot(TOF),and ventricular septal defect(VSD),et al.All hospital records were reviewed and clinical data were studied and analyzed.Results A total of 313 patients had DSC (5.6％).There were 191 males and 122 females.The reasons for DSC are hemodynamic instability in 296,bleeding in 11,and ECMO(or VAD) in 6 patients.Two hundred and fiftyone (80.2％) patients were less than 5 kg,84 (26.8 ％) patients were neonates,and 125 (39.9％) patients aged between one and three months old.The incidences of DSC for these patients were 13.9％,34.4％,and 18.4％ respectively.The diagnosis of complex congenital heart defects had a high risk of DSC.The incidences of DSC for TGA,CoA,IAA,PA,Truncus,CAVSD,TAPVC,and DORV were 28.8％,17.8％,43.1％,9.0％,30.4％,6.5％,and 10.8％ respectively.The DSC patients had longer duration of CPB time(147 min versus 72 min) and clamping time(81 min versus 40 min).In 282 patients the sternums were closed 1-11 days after the initial operation.In 3 cases the trial of closure failed for the first time and succeeded a second time until several days later.Fouty-six patients died ultimately including 15 patients died after the closure of sternum with a total mortality of 14.7 ％.Surgical site infection occurred in 17 patients (5.4％),and surgical intervention were needed in 4 severe cases(1.3％).Conclusion Neonates,an age less than 3 months,weight less than 5 kg,long CPB time and clamping time,and the diagnosis of complex congenital heart defects were risk factors of the need for delayed sterna closure after pediatric cardiac operations.