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 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.

15 mm) should be used in patients over the age of 4 and later reoperation might be avoided. For D-TGA without long segment of LVOTO, we can choose arterial switch plus pulmonary valvotomy or Konno procedure to relief the LVOTO. To prevent postoperative functional mitral regurgitation, in C-TGA-VSD-PS patients the best surgical procedure is double switch operation.

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 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 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 study the relationship between MRI features and pathological characteristics of the renal clear cell carcinoma,and to conduct a comparative analysis.Methods 23 patients renal clear cell carcinoma who were proved by postoperative pathology were retrospectively analyzed. The preoperative MRI findings and the postoperative pathological characteristics were compared. Results MRI plain scan showed uniform signal was seen in 2 cases, and mixed signal was found in 21 cases with cystic and necrosis. Blood was seen in 11 cases and false envelope was present in 12 cases. Dynamic enhanced scanning revealed that solid part was enhanced obviously, it was lower than the renal cortex but higher than the renal medulla in cortical phase and continued to be enhanced in medullary phase and delay phase.Postoperative pathological characteristics showed that tumor profiles were yellow-white and cystic and necrotic structures were present within tumors, blood was found in 17 cases. Endoscopic tumor cells were present in solid nests or acinar structure, and mesenchyma was rich in thin-walled vessels involved in reticular interval.14 cases were found to have false envelope.Conclusion Renal clear carcinoma MRI imaging manifestion is related to organixational srructure. MR plain scan combined with dynamic enhanced scan has very high value in the diangnodis of renal cell carcoinoma.

ObjectiveTo discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.MethodsBetween May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.ResultsAll patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.ConclusionConclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.

Objective Infracardiac total anomalous pulmonary venous connection, a rare congenital cardiac defect, is associated with high mortality. A modification was designed for the procedure to reduce the post-operative obstruction in the pulmonary venous. Methods From September 2005 to December 2007, seven patients with infracardiac total anomalous pulmonary venous connection were treated with repair surgery through right side approach. The patients' age at operation was (70.57 ±44.67) days , the weight was (4.07 ±0.87) kg. Three patients had pulmonary venous obstruction, and 2 with small PFO. A modified right - side approach for repairing this defect was used. Results No death occurred after the operation. The postoperative complications included low cardiac output in 5 patients (71.43%), pulmonary hypertension crisis in 3patients ( 42.86% ) , mechanical ventilation for more than 7 days, which happened in 2 patients (28.58%) and pulmonary infection. All of the patients received follow-up. No residual shunt and pulmonary venous return obstruction were identified on echocardiogram(with a velocity from 1.2 m/s to 1.47 m/s). The heart function of patients was within the normal range(EF 0.70 -0.79, FS 0.32 -0.44). Conclusion The modified surgical procedure for the correction of infracardiac total anomalous pulmonary venous connection by right side approach was associated with favorite clinical outcomes, The post-operative outcomes depended on the size of anastomosis between the common vein and left atrium and the patency of the pulmonary venous return. Adequate size of anastomosis and maintenance of the spatial structures in adjacent regions were helpful in decreasing the adverse effect of postoperative obstruction.