Objective To review the experience of surgical repair of aortic origin of pulmonary artery.Methods Fifteen patients underwent surgical correction for aortic origin of pulmonary artery.All of the patients had anomalous origin of right pulmonary artery,and 11 were associated with patent ductus arteriosus,6 with patent foramen ovale,3 with atrial septal defect,2with aortic pulmonic window,1 with ventricular septal defect,1 with interruption of aortic arch,1 with coarctation of aorta and 1 with pulmonary vein stenosis.All patients were associated with severe pulmonary hypertension.All patients were operated on with the implantation of the AORPA to the main pulmonary artery derectly.Results All 15 patients were cured and no died.1 lost follow-up and the other 14 were survivable during a follow-up 3 months to 81 months.one patient recepted pacemaker implantation 2 years after operation because of sinus arrest,no other patients required reoperation.Conclusion Echocardiography combined with spiral ct angiography is a good method for AOPA diagnosis.Good surgical outcome and low reoperation rate of aortic origin of pulmonary artery can be achieved by direct anastomosis.

Objective To review the experience of anomalous origin of the left coronary artery from the pulmonary artery.Methods 36 patients were admitted,17 males and 19 females,the median age was 9.5 months.12 were associated with severe mitral regurgitation,10 with left ventricular aneurysm,1 with patent ductus arteriosus,2 with patent foramen ovale.33 pa tients underwent surgical repair,of which 3 underwent left coronary artery (LCA) ligation,1 underwent mitral valve replacement,29 underwent reestablishment of a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta,incidental mitral valvoplasty in 7 patients,mitral valve replacement in 1,patent ductus arteriosus ligation in 1 and patent foramen ovale closure in 2.Results Two patients died in the hospital,one postoperative patient lost follow-up,one underwent LCA reimplantation suddenly died 3 days after discharged,one received coronary artery bypass graft 2 years after LCA ligation,the remainder were asymptomatic during a follow-up of 1 to 73 months.one of the patients who did not underwent surgical repair lost follow-up,when one suddenly died 2 months after discharged,the remained one was asymptomatic during a follow-up of 5 months.Conclusion Echocardiography combined with spiral CT angiography is a good method for ALCAPA diagnosis.Good surgical outcome of ALCAPA can be achieved by reestablishing a dual coronary system with direct aortic reimplantation of the left coronary artery into the aorta,and left ventricular dysfunction could be significantly improved.Incidental mitral valvoplasty may be helpful in early rehabilitation but poor in long-term prognosis.Left ventricular aneurysm could be improved so a incidental resection of left ventricular aneurysm was not suggested.

Objective The aim of this article is to review and analyze the timing and surgical management of mediate and severe atrioventricular valve regurgitation(AVVR) in single-ventricle patients.Methods Between June 2006 and October 2011,twenty-three cases of single-ventricle patients accompanied with AVVR underwent atrioventricular valve plasty or replacement.There were 17 males and 6 females.Their ages ranged from 2.1 to 22.0 years,and their weight from 12.5 to 59.0 kg.There were 3 cases of A type of single ventricle,17 of B type,2 of C type,and 1 of D type.All cases had one atrioventricular valve except one of D type with 2 groups of atrioventricular valves.There were 18 patients with sever AVVR and 5 with the moderate.Before the management of AVVR,12 patients had undergone the first stage palliation,including B-D Glenn procedure 11 cases and A-P shunt 1 case.The periods between the two stages operations were 7-96 months.Among the all,there were 7 cases of atrioventricular valve replacement ; 3 cases of atrioventricular valve replacement and TCPC ; 5 cases of atrioventricular valve replacement and B-D Glenn procedure ; 2 cases of atrioventricular valve repair and TCPC ; 4 cases of atrioventricula repair and B-D Glenn procedure; 1 case of atrioventricular valve repair,B-D Glenn procedure and TAPVC repair; 1 case of atrioventricular valve repair,B-D Glenn procedure,PA Banding and TAPVC repair.Results In this group,there were 65.2％ patients who underwent atrioventricular valve replacement.The ones with moderate regurgitation underwent atrioventricular valve repair.Only 3 of the 18 cases with severe regurgitation could underwent atrioventricular valve repair(P =0.002).Three cases died.The mortality was 13％.All cases undergone atrioventricular valve repair were alive.The mortality of atrioventricular valve replacement was 20％.All the post-operative alive were followed up.Their follow-up period were between 0.8-6.3 years,withoud a dead case.Conclusion The regurgitation with single ventricle should be managed before the image of myocardium occurred.It is the best time to manage the atrioventricular valve when the regurgitation was moderate.The atrioventricular valve replacement is effective to the cases of single ventricle with severe AVVR.

Objective The aim of this study is to retrospectively analyze surgical management of absent pulmonary valve syndrome(APVS).Methods Between January 2005 and January 2012,totally 11 children with APVS underwent primary surgical correction.There were 7 boys and 4 girls.Age at surgeries range from 1 to 5 years,and wcight from 10.2-17.5 kg,with average (12.3 ± 3.4) kg.Surgical procedures include VSD repair,pulmonary arteries reconstruction and RVOT reconstruction with monocusp valve.5 cases chose Lecompte maneuver as an option to release compression to bronchus,5 cases underwent fibroscopy inspection and airway secretion suction,and 4 cases adapted deep hypothermic circulatory arrest (DHCA)during correction procedures.All survivors are routinely followed-up with echocardiogram.Results All 11 cases survived,2 of them suffered from frequent lungs infections during the first year post surgery.No case exists bronchus or pulmonary arteries compression.Conclusion APVS is a rare congenital heart defect,which may challenge perioperative managements and operations.Ideal surgical correctiou includes RVOT reconstruction,decompression of bilateral bronchus,and airway inspection with fibroscopy.However,compression of intrapulmonary bronchi by abnormally branching pulmonary arteries may expose patients to a relative long time of medication therapy after surgical correction.A large number of patients with long-term follow-up are needed to draw definitive conclusions on this strategy' s effectiveness.

Objective Retrospectively analyze primary experences of surgical correction of complicated aortic arch anomaly with autologous palmonary artery.Methods Between July 2010 and December 2012,13 cases of complicated aortic arch anomaly underwent reconstruction of aortic arch with autologous pulmonary artery.Classifications of aortic arch anomaly were interrupted aortic arch with ventricular septal defect in 4 patients,ventricular septal defect associated with coarctation in nine patients.There were 7 males and 6 females.Their age at surgeries ranged from 1 month to 16 years,and the body weight were from 3.5 kg to 52.0 kg with median weight of 12.6 kg.Cardiopulmonary bapass was estabished with dual arterial cannulations in patients with interrupted aortic arch.During cooling to deep hypothermia(rectal temperature was 18 ℃),intracardiac defects were totally corrected.Arch anomaly was reconstructed under deep hypothermia,including deep hypothermic cardiac arrest(DHCA) in 9 patients,deep hypothermia with regional perfusion in 4 patients.Anterior wall of pulmonary artery was excised in all of 13 patients.In 4 cases,the excised wall of anterior wall of pulmonary artery was sutured to form a conduit with different diameters according to the patient's bady surface area.Two ends of the conduit were anastomosed the aortic arch and desceding aorta respectively.In another 9 patients,aortic arch was augmented with tailored pulmonary artery patch in oval shape.The defect of pulmonary artery was repaired with autologous pericardial patch.Results There was only 1 death due tomutiple organ failure postoperatively.Another 12 patients survived without neurologic complications.Differences of arterial pressures between upper and lower extremeties were not monitored in all cases.During follow-up,routine echocardiogram showed satisfactory results with unobstructive blood flow at the aortic arch.Conclusion Autologous pulmonary artery can be used to relieved complicated aortic arch anomaly completely without any tension of anastomosis site and compression of left main bronchea postoperatively.More patients with long-term follow-up are necessary to draw an accurate conclusion of this technique.

Objective Konno-Rastan procedure is one option to cope with complex multilevel left ventricular outflow tract obstruction (LVOTO),which continues to pose a serious challenge to cardiac surgeons.The aim of this study is to retrospectively analyse indications for Konno-Rastan procedure,and to review the safeguards and pitfalls.Methods Between January 1996 and August 2012,totally 13 children with multilevel LVOTO underwent Konno-Rastan procedure.There were 8 boys and 5 girls.Age at surgeries ranged from 5 to 13 years,and weight from 12 to 51 kg with median of 21 kg.The pathology of this cohort includes:8 cases of congenital aortic valvular stenosis,3 cases of congenital aortic valvular stenosis combined with supravalvular stenosis,1 case of congenital aortic stenosis combined with VSD,coarctation and RVOT obstruction,1 case of aortic stenosis s/p percutaneous balloon aortic valvuloplasty.All patients have secondary diffuse tunnel LVOTO.Diameter of aortic ring ranges from 12.0 to 16.4 mm,and pressure gradient across the stenotic region ranges from 90-151 mm Hg.8 cases were implanted with St.Jude AG19 while 5 cases implanted with St.Jude AG17.Results All 13 cases survived.The 4th patient was implanted permanent epicardial pacemaker for transient Ⅲ AVB.The 4th and 5th patients were found residual ventricular septal repture at the nadir of ventricular incision,one underwent redo procedure while another is being followed up.All cases take cumadine to sustain INR at 1.8-2.5.No death emerges during follow-up period.The motality is 0％,the incidence rate of B is 7.7％,residual VSD 15.4％ and endocarditis 7.7％.Conclusion Konno-Rastan procedure is a promising techi.to relieve LVOTO.However,this complex procedure may lead to several fetal complications.Success of the surgery demands perfect operations.

Objective In order to optimize the Fontan circulation,a technique for direct total cavopulmonary connection was devised.To evaluate its surgical feasibility as well as surgical outcomes,our clinical experience was retrospectively reviewed.Methods From August 2005 to March 2012,23 consecutive patients underwent modified extracardiac Fontan operation with direct total cavopulmonary connection.Clinical profile of the patients,and procedural variables were examined and analyzed.Results All patients had adequately developed main and branch pulmonary arteries.Inferior caval vein was contralateral to the pulmonary trunk main pulmonary artery in 7 cases,ipsilateral in 8,and others in 8.There was 1 hospital death.The other 22 patients remained hemodynamically stable postoperatively.Prolonged effusions (n =13,62％) was a challenging problem.No obvious stenosis was found at the direct cavopulmonary anastomosis.Conclusion we are convinced that a direct total cavopulmonary connection is feasible in select subset of patients.This modified Fontan procedure retains the advantage of extracardiac connections together with the avoidance of prosthetic materials.

Objective Retrospectively analyze surgical correction supracardiac total anomalous pulmonary venous con-nection (TAPVC) using sutureless technique to prevent post-repair pulmonary vein stenosis. Methods Between December 2007 and December 2008, 25 children cases of total anomalous pulmonary venous connection underwent primary surgical cor-rection. The anatomic types of TAPVC were supra cardiac in nine patients, inha cardiac in one, mixed in 3 and intra cardiac in 12. Five of nine supra cardiac TAPVC underwent correction using sutureless technique. There were 2 male and 3 female. Their age at surgeries ranged from 2 months to 13 years, and the body weight were from 4.5 kg to 2.1 kg with an average of (7.9±6.4) kg. After median sternotomy and opening the pericardium, the heart was arrested by delivering cold crystal ear-dioplegia. The heart was then positioned toward the patient' s right and under the right henri sternum. A generous incision across the posterior wall of the left atrium and one on the common pulmonary vein was made. The latter was extended upwards to the midpoint of the vertical vein. The left atrium was subsequently connected to the pulmonary venous confluence by suturing the edge of the atrium to the posterior mediastinal pericardium that surrounding the common pulmonary vein and the vertical vein with 7-0 PDS. The vertical vein was partially ligated after conclusion of CPB, leaving a diameter of 5mm shunt. Routine follow-up with echocardiogram were at diacharging, 3 months, 6 months and 1 year after surgery. Results All 5 cases survived uneventfully excopt 1 baby on ventilation over 7 days after surgery, who had bilateral lung consolidation before the operation. Echocardiogram showed satisfactory results with maxium velocity acrossing the anastomosis of 0.65 -0.85 m/s. Conclusion Sutureleas technique can avoid trauma to the pulmonary venous endothelium and minimize the tension of anastomasis. It may play an important role to prevent post-repair pulmonary vein stenosis. More patients with long-term follow-up are necessary to draw a definite conclusion of this technique.

Objective To compare the two different ways of right ventricular outflow tract(RVOT) reconstruction at repair of pulmonary atresia with ventricular septal defect,the direct RV-PA anastomosis and pericardial conduit to find the better way.Methods From Jun.2002 to Oct.2012,66 patients underwent pulmonary atresia with ventricular septal defect repair in our hospital,age at operation from 14 days to 272 months.Patients were divided into 2 groups according to the way of RVOT reconstruction.Group 1:31 of them,using direct RV-PA anastomasis,Group 2:35 of them,using pericardial conduit.Paired t test was used to evaluate the growth of pulmonary arteries.Chi-square test and Kaplan-Meier were used to calculate the postoperative mortality,reopemtion situation and survival time.Results There are 3 early hospital death in group 1 (9.7 ％),and 5 in group 2(14.3％),P =0.71.There is a significant difference between the two groups in restenosis rate of the RV-PA anastomasis and autologous pericardial conduit with pulmonary branch artery(Group 1:22.2％,Group 2:55.6％,P =0.01).The diameters of RV-PA anastomasis and the pulmonary artery branches in follow-up were significantly lager than the earlier diameters(P ＜ 0.05) in group 1.There is no growth on diameters of the pericardial conduit and pulmonary branches except the right pulmonary artery in follow-up in group 2.There is no significant difference between the two groups in later survival(P =0.30).Conclusion Both the direct anastomasis of RV-PA and pericardial conduit are available for RVOT reconstruction in pulmonary atresia with ventricular setal defect repair.There is lower incidence of RVOT and pulmonary stenosis and anastomosis absolutely has the ability for later growth in the former.