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 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 The study aimed to explore the development direction of congenital heart disease surgery through comparing Europe with Guangdong General Hospital(GDGH) in data of ECHSA Congenital Database.Methods The data between 2009 to 2015 of Europe and GDGH were extracted from ECHSA Congenital Database.The data of Europe and GDGH were compared by basic information,operating difficulty and mortality.Results The results of Europe and GDGH were patient number(71 763 vs 13 119),procedure/patient ratio(126.2％ vs 104.1％),age[(75.91 ± 146.18) months VS(105.80 ± 172.18) months],the proportion of neonate (18.2％ vs 4.4％),Aristotle mean score (7.00 vs 6.67),30 days mortality (2.98％ vs 1.73 ％).The proportion of neonate palliative operation of Europe was more than that of GDGH.In adult group,Europe was more of reoperation and of GDGH was more of primary surgery.Conclusion The surgical treatment of congenital heart disease of GDGH is developing and is close to the mean average of Europe.The proportion of neonate,complex surgery and reoperation is lower than Europe.

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 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 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 evaluate the outcomes of surgical repair of typeⅢ pulmonary atresia with ventricular septal de-fect( PA/VSD) .Methods Retrospectively analyzed the clinical data of 98 patients with type Ⅲ PA/VSD who underwent sur-gical repair in the Department of Cardiovascular Surgery , Guangdong Cardiovascular Institute from September 2005 to December 2014.There were 54 males and 44 females at the mean age of(4.2 ±4.8) years and the mean weight of(13.2 ±10.0) kg. There were 67 patients in palliative repair group and 31 patients in radical repair group .There were 85 patients underwent on-pump operation including 9 beating heart cases.Results The mean bypass time was(121.2 ±49.4)min, the mean aorta cross-clamping time was(75.4 ±31.8) min.The overall postoperative mortality was 6.1%(6/98).For the survival patients, the mean ventilation time was(106.7 ±184.3) h, the mean ICU stay was(8.9 ±10.9) days and the mean hospital stay was (33.4 ±17.0)days.During follow-up period, 28 patients were underwent re-operation, the postoperative mortality was 7.1%(2/28).There were no differences in postoperative status between two groups.Conclusion The outcomes of surgical repair for type Ⅲ PA/VSD was good.Preoperative evaluation of the pulmonary development and MAPCAs were helpful for choosing surgical options.Re-operation was recommended to those appropriate patients.

Objective To summarize the experience and early outcomes of surgical treatment of adult tetralogy of Fallot (ATOF).Methods We retrospectively analyzed the clinical data of 227 patients with ATOF who underwent surgical repair in the Department of Cadiovascular Surgery,Guangdong Cardivascular Institute from January 2004 to December 2014.There were 112 males and 115 females at a median age of 34 years(range,18 to 58 years) and a mean weight of(49.00 ± 8.27) kg.All patients were underwent one stage repair,including 129 cases with transannular patch and 61 cases with MAPCAs in which 4 cases were underwent hybrid occlusion.Results There were 12 cases died in hospital(5.3％),24 cases with Re-thoratomy for hemaostsis,5 cases with poor wound healing,10 cases with postoperative pneumonia.There were 25 cases with residual VSD including 8 cases caused by surgeon in congenital heart disease department and 17 cases caused by surgeon in adult heart disease department(P ＜ 0.05).The repair with transannular patch required significantly longer bypass time [(87.83 ± 26.02) min vs.(78.47 ± 26.00) min,P =0.009].The cases with MAPCAs had higher cost than that with no MAPCAs [(83 137.01 ±69363.05) RMB vs.(66 184.29 ±44219.38) RMB,P=0.03].Conclusion The early outcomes of ATOF is good.The cases with MAPCAs had higher cost than that with no MAPCAs.The CHD surgeon had lower probability of residual VSD.Preoperative evaluation of MAPCAs by CHD surgeon and perioperative maintain of the right ventricular function were helpful.