Tetralogy of Fallot(TOF) with absent pulmonary valve syndrome is a rare pathologic condition which is characterized by absence of pulmonary valve, dilatation of the pal-monary artery involving its left and right branches in addition to the basic pathoanatomies of TOF. In some cases,the enlarged left pulmonary artery may cause compression of the left bronchus. Its complexity deseves some special attention during corrective surgary. Betwleen 1985 and 1998, six patients with this syndrome underwent surgical correction under CPB combined with moderate hypothermic in our hospi-tal. The surgical procedures consisted of resection of the muscular bands present in RVOT causing obstruction and partial excision of the anterior wall of the markedly dilated left and right pulmonary arteries in 2 cases and transannular pericardial patching in 4 cases. One patiant with absence of left pulmonary artery died on the first postoperative day. The other 5 survivors all recovered. Regarding the proper time for surgical treatment on this particular group of patients, it is advisable that in sick children with no respiratory symptoms, corrective surgery be deiyed to the age of 3 or 4 years old. However, when the sick children developed severe respiratory symptoms, corrective surgery should be done as early as possible. In this special situation, a valved transannular patch is preferred.

Objective To summarize experiences of surgical correction of aortopulmanary septal defect (APSD) in children. Methods Fifteen children with APSD,aged 5 months to 11 years,weighed 4.5 to 21.0kg,underwent surgical correction. Based on Richardson's classification,type I in 7 cases,type II in 3,and type III in 5. Eight cases were associated with other cardiac defects (53.5%),including 4 cases with complicated cardiac defects (26.7%). Operative technique included patch repair of defect in 8 cases with type I and II,an intraaotic synthetic baffle directed pulmonary blood from the APSD to the right pulmonary artery (RPA) in 3 cases with type III,an artificial conduit was used to connect the RPA with main pulmonary artery (MPA) and a flap of aortic wall was excised along with the anomalous RPA to extend the anastomosis in each case with type III,direct suture was used in 2 cases. Other associated cardiac defects were repaired simultaneously. Results The post-operative mortality rate was 6.7% (1/15). Eleven cases were followed-up from 3 months to 13 years in good condition. Conclusion APSD associated with complicated cardiac defects is apt to be misdiagnosed. Correct diagnosis can be made by 2-D echocardiography, cardiac catheterization angiography,and MRI. The operation should be done as early as possible once definite diagnosis is made. Operation should be done infancy to prevent development of pulmonary vascular disease. In type III APSD and APSD associated with complicated cardiac defects,operative mortalith is high. Preoperative accurate diagnosis and full understanding of the pathophysiology are the keys to an optimal surgical correction.

Objective To review and sum up experience of surgical repair of single ventricle. Methods Since January 1973 to December 1999, 47 patients with single ventricle have been repaired. Right ventricle type was 17 patients, left ventricle type was 14, and unrecorded type was 16. Surgical procedure consisted of pulmonary banding 1, Fontan operation 23, and bidirection superior cavopulmonary anastomosis 23. Results Seven patients died. The motality was 15%. There was no death in bidirection superior cavopulmonary anastomosis patients. Conclusion For the single ventricle, early surgical intervention is required to prevent pulmonary congestion and severe cyanosis. The bidirection superior cavopulmonary anastomosis can improve single ventricle function secondary to pressure and volume load to decrease surgical mortality and to minimize the risk factors for subsequent Fontan operation.

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.

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 To summarize the experiences with minimally invasive pectus repair (Nuss procedure) for recurrent and acquired pectus excavatum after open thoracic surgery.Methods From Jun 2004 to Sep 2011,eighteen patients with recurrent or acquired pectus excavatum underwent Nuss procedure,including 12 males and 6 females The age ranged from 3.1to 14.8 years with mean age of (8.8 ±4.0) years.The body weight was 11 to 55kg with mean weight of (30.2 ±14.8 ) kg.Ten cases were recurrent pectus excavatum with previously failed open surgery repair,eight were acquired pectus excavatum after other open thoracic surgery.Sixteen cases had symmetrical and 2 had asymmetrical pectus excavatum.Haller' s index was 5.4 ± 3.4.The operation was performed with thoracoscopic assistance.Results All patients had successful operation with one bar insertion in each patient,one stabilizer was put on right side in seventeen and double stabilizers were put in one case.Therapeutic results evaluation was excellent in 16 cases and good in 2.Percentage of excellent and good was the same with that in our primary Nuss procedure ( P ＞ 0.05 ).Chest drainage duration was 1 to 4 days.One case had bar displacement revision 5 months later.Heart perforation occurred in one on whom a sternotomy and perforation repair were immediately performed.The echocardiography exam shows normal cardiac function after operation,and no nerve system complications were detected.One developed pneumothorax on operative day and one had pleural effusion three days later,both were treated by chest tube drainage.Twelve patients' bars were removed after 24 - 45 months of stagnation period.Anatomic results at bar removal were 10 excellent and 2 good,there were no recurrent cases.Conclusion Nuss procedure is an effective method and has good results on recurrent and acquired pectus excavatum.Safety of patients and complications minimization is always the first to be considered.

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 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 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.