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.

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.

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 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.

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 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 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 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.