Objective:To explore the risk factors and follow-up outcomes of pediatric heart transplantation(HT).Methods:Between January 2018 and June 2022, perioperative data are retrospectively reviewed for 41 pediatric HT recipients aged <18 years and donor-recipient weight data for infants aged under 3 years at Guangdong Provincial People's Hospital.Perioperative survivors are followed up until August 31, 2022 through out patient visits and telephone calls.Postoperative survivals are examined by Kaplan-Meier method and possible risk factors for perioperative survival identify with Logistic regression.Results:There are 22 boys and 19 girls with a median age of 120(58~138)months.After preoperative adjuvant therapy of extracorporeal membrane oxygenation(ECMO), 8 cases had a successful transition to HT and 2 children underwent ABO incompatible(ABOi)HT.Six children aged under 3 years had a donor-recipient weight ratio of 2.95.Among 17 children, there are one or more complications, including continuous renal replacement therapy(CRRT, 9 cases, 21.95%), tracheotomy (3 cases, 7.32%), delayed chest closure or redo of sternotomy(6 cases, 14.63%)and acute graft dysfunction(4 cases, 9.76%). Five children died during perioperative period.The possible risk factors for perioperative mortality include preoperative ECMO assistance[ HR: 32.00, 95% CI: (2.83~361.79), P<0.05], preoperative CRRT[ HR: 11.33, 95% CI: (1.15~111.69), P<0.05] and total bilirubin [ HR: 1.02, 95% CI: (1.002~1.040), P<0.05]. During follow-ups, one child died from Epstein-Barr virus (EBV)associated post-transplant lymphoproliferative disease; another case of EBV-associated hepatic leiomyoma underwent transcatheter arterial embolization.With an overall survival rate of 85.37%, the cumulative survival rate is 96.97% for children without preoperative ECMO assistance( P<0.05). Postoperative mortality rate spiked markedly in children with preoperative ECMO assistance ( P=0.0013). However, follow-up results of perioperatively survivors indicate that preoperative usage of ECMO will not affect follow-up survival( P=0.53). In ABOi group or infants aged under 3 years, no mortality occurres postoperatively or during follow-ups. Conclusions:In infant aged under 3 years, the strategies of ABOi HT and large-weight donor HT are both safe and effective and it has no effect upon perioperative and follow-up survivals.Preoperative ECMO assistance, total bilirubin and preoperative use of CRRT are risk factors for perioperative survival.

Objective Norwood StageⅠis the standard procedure to cope with hypoplastic left heart syndrome(HLHS), which continues to be the most challenging congenital heart disease .The aim of this study is to retrospectively analyse the perio-perative management of Norwood StageⅠ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood StageⅠprocedure.They were all boys.Age at surgeries ranged from 29 to 75 days, and weight from 2.57-3.50 kg with median of 3.13 kg.Case 1, 2 and 3 received standard medical regimen after accessing NICU, which included intravenous prostaglandin E1 and mechanical ventilation.The 3 infants underwent emergent operations because of unstable hemodynamics.Case 4 and 5 received no medical intervention before the urgent surgeries.All 5 cases underwent the standard Norwood StageⅠprocedure under deep hypothermic circulatory arrest, including 4 cases of modified Blalock-Taus-sig shunt( MBTS) and 1 case of Sano shunt.Results The case with Sano shunt died from severe hypoxemia and persistent aci-dosis 32 hours after the operation, another case died from low cardiac output syndrome after cardiopulmonary bypass.The first case underwent bidirectional Glenn procedure 12 months after Norwood Stage I, the postoperative heart function was NYHA I and the oxygen saturation was 0.90-0.95 in room air, but he died from accidental brain injury 3 months after stage Ⅱ.The second case was followed up 3 months after stage I procedure with NYHA I and oxygen saturation of 0.78-0.83 in room air, and lost the follow-up after.The fifth case was followed up 3 months after stageⅠprocedure with NYHAⅠ, confluent MBTS and oxygen saturation of 0.84, the patient is being followed up and waiting for further evaluation for stageⅡprocedures.Con-clusion The standard Norwood StageⅠprocedure is a complex procedure, which demands multidisplinary cooperation, to pal-liatively correct HLHS .Sharing expericences on perioperative managements of Norwood Stage I between heart centers in China will be helpful to decrease the mortality and morbidity in relatively short period .

Objective To retrospectively analyse the anaesthetic management of Norwood Stage Ⅰ.Methods Between June 2010 and August 2014, totally 5 small infants with HLHS underwent the standard Norwood Stage I procedure .They were all boys.Age at surgeries ranged from 29 to 75 days with median 36 days, and weight from 2.57 -3.50 kg with median of 3.13kg.The first three cases were received intravenous prostaglandin E1 before they were sent to the operation theatre and were under mechanical ventilation .They were received emergent operations because of unstable hemodynamic situation .The other two cases were relatively stable without mechanical ventilation and were received restrict surgery .All 5 cases received the stand-ard Norwood Stage Ⅰprocedure under intravenous-inhalation balance-general anaesthesia with cardiopulmonary bypass.The technique of deep hypothermia and circulatory arrest were used in all five cases .Results The fourth case died from low cardi-ac output syndrome after cardiopulmonary bypass .The other 4 cases were transferred to the paediatric intensive care unit after withdrawal from bypass.One of the four cases died after 32 h after surgery.Conclusion The standard Norwood Stage Ⅰ pro-cedure is aquite complex procedure, which demands multidisplinary cooperation, to palliatively correct HLHS.We retrospect the experiences of the anesthetic management in our centre and hope it will be helpful to decrease the mortality and morbidity in relatively short period.

Objective The results of repair for TOF with anomalous coronary artery(ACA) were studied to determine the incidence of coronary anomalies and evaluate surgical strategy choicesas well as postoperative outcomes.Methods From January 2008 to August 2014,1142 consecutive patients underwent repair of TOF including 44 patients with TOF and ACA:single coronary artery in 15,dual anterior descending coronary in 15,single left anterior descending coronary arising from the rightcoronary artery in 3 and the other ACA in 5.The median age was 5.7 years (range,1 month-27 years),and the median weight was 16.0 kg(range,4.5-51.0 kg).Surgical procedure was selected according to the extent of right ventricular outflow tract (RVOT) obstruction and distribution of the ACA.Results There was one operative death.No deaths during the follow-up period in the other 37 patients.Single patch techniquewasperformed in 15.RVOT residual obstruction detected in 7 who without transannular patch,and one need reoperation;Two patch technique was performed in 6,and 3 of them required an additional RV-PA(pulmonary artery) tube because of RVOT residual obstruction during the operation;Double oullet technique was in 6.No tube stenosis occurred in follow-up period time;PA translocation technique was in 11.The right PA stenosis was detected in 4;ACA was ligated and divided in 3,then RVOT reconstruction was performed.Conclusion The combination of ACA is not a contraindication to primary repair of TOF.But there are many anatomiacal variations of ACA,and the accuracy of preoperative diagnosis is low.So proper selection of surgical approach should be individualized based on the careful intraoperative identification of the distribution of the ACA as well as the location and degree of the RVOT obstruction.

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 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 To evaluate the role of transesophageal echocardiography (TEE) in perioperative period of pediatric patients with congenital heart disease (CHD).Methods From July 2011 to December 2012,TEE was used in 393 pediatric patients(≤ 14 years) with congenital heart disease in perioperative period.We make a retrospective review with the clinical data of these cases.Results Operative schemes or therapeutic schedules of 60 patients(15.3％) were altered according to TEE.By preoperative TEE,the diagnosis of transthoracic echocardiography (TTE) of 4 cases(1.0％) were amended,4 cases (1.0％) were complemented,and 4 cases (1.0％) got the auxiliary diagnosis,among which operative schemes of 11 cases (2.8％)were altered.During the operate,29 cases(7.4％) found residual problems,11 cases(2.8％) got the auxiliary diagnosis.By postoperative TEE,7 cases(1.8％) found residual problems,1 case(0.3％) got the auxiliary diagnosis.Complications occurred in 9 cases(2.3％) of the 393 patients.Oral and pharyngeal mucous membrane bleeding occurred in 7 cases (1.8％),inadvertent tracheal extubation in 2 cases(0.5％).Conclusion TEE plays an important role in confirming preoperative diagnoses,formulating surgical plans,evaluating immediate operative results,identifying patients with residual defects and guiding the therapeutic schedule in perioperative period of pediatric patients with congenital heart disease.

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.