Objective:To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies.Methods:A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged ( M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results:In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95% CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95% CI: 89.9% to 100%). Conclusions:Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Objective:To examine the early and midterm surgical outcome of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) using revised surgical strategies.Methods:A retrospective analysis of clinical data, surgical methods, and follow-up results was performed of 104 cases of PA/VSD/MAPCA in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center from January 2017 to September 2022. There were 55 males and 49 females, aged ( M(IQR)) 33.9(84.0) months (range: 0.5 to 209.6 months) at the first surgical procedures. The anatomical classification included 89 cases of type B and 15 cases of type C. The number of major aortopulmonary collateral arteries was 4.2 (3.0) (range: 1 to 8). The Kaplan-Meier method was used for survival estimation. Results:In the first stage of surgery, 50 patients underwent a complete primary repair, 12 patients underwent partial repair, 32 patients underwent palliative right ventricular-pulmonary artery connection, and only 10 patients chose the Blalock-Taussig shunt. There were 10 cases of early death. In the second stage, 14 patients underwent complete repair and 4 patients underwent partial repair with no early death. The interval between the two surgeries was 19 (10) months (range: 9 to 48 months). Finally, during the 40 (34) months follow-up period, a total of 64 patients were complete repair and the right/left ventricular pressure ratio after complete repair was 0.63±0.16 (range: 0.36 to 1.00). Survival analysis showed that survival rates at 1 and 5 years after first-stage surgery were both 89.4% (95% CI: 83.5% to 95.3%). At 28 (34) months (range: 1 to 67 months) of follow-up after complete repair, the survival analysis showed that the survival rates at 1 and 5 years were both 95.2% (95% CI: 89.9% to 100%). Conclusions:Using combined approaches tailored to individual patients and optimized unifocalization strategy, the complete repair rate at one stage and the cumulative complete repair rate at 5 years improved significantly with a lower right/left ventricular pressure ratio and satisfactory early and intermediate survival.

Objective:To evaluate the early results of pulmonary autograft mitral valve replacement (Ross Ⅱ procedure) in infants with intractable congenital mitral valve lesions.Methods:Between August 2018 and September 2019, 6 infants underwent mitral valve replacement with a pulmonary autograft in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center. There were 2 males and 4 females.The age at surgery ranged from 50 days to 1 year old.Preoperative diagnoses included severe to critic mitral valve insuffiency in all patients, moderate mitral valve stenosis in 3 patients, and mitral valve prolapse in one. When the pulmonary autograft was harvested, a cuff of bovine or autologous pericardium was sewn onto the proximal (infundibular) end of the autograft ( "top-hat" configuration). The distal (pulmonary) end of the autograft was secured to the mitral annulus.At the level of the left atrial free wall, the pericardial cuff was progressively tailored and sewn onto the atrial wall to remain away from the ostia of the pulmonary veins and to maintain normal morphology of the autograft. The bovine jugular valved conduit was used to reconstruct the right ventricular outflow tract.Results:There was one early death due to sudden cardiac arrest at the night of surgery day. The remaining 5 patients were successfully recovered and discharged. Follow-up of survivors ranged from 3 to 13 months. Echocardiographic follow-up demonstrated the flow velocity across the mitral valve position was 1.5 to 2.3 m/s, with a means gradient of 4 to 6 mmHg (1 mmHg=0.133 kPa). Four patients showed mild mitral insuffiency, normal left atrium and ventricle size and left ventricle ejection fraction.One patient had moderate mitral insuffiency, pulmonary valve endocarditis, and reduced left ventricle ejection fraction. The clinical symptoms of all survivals improved significantly and the weight gain were satisfactory.Conclusion:Pulmonary autograft mitral valve replacement may be a feasible and effective remedial surgical strategy for young infants with intractable congenital mitral valve lesions.

Objective:To evaluate the early results of pulmonary autograft mitral valve replacement (Ross Ⅱ procedure) in infants with intractable congenital mitral valve lesions.Methods:Between August 2018 and September 2019, 6 infants underwent mitral valve replacement with a pulmonary autograft in Department of Cardiovascular Surgery, Guangzhou Women and Children′s Medical Center. There were 2 males and 4 females.The age at surgery ranged from 50 days to 1 year old.Preoperative diagnoses included severe to critic mitral valve insuffiency in all patients, moderate mitral valve stenosis in 3 patients, and mitral valve prolapse in one. When the pulmonary autograft was harvested, a cuff of bovine or autologous pericardium was sewn onto the proximal (infundibular) end of the autograft ( "top-hat" configuration). The distal (pulmonary) end of the autograft was secured to the mitral annulus.At the level of the left atrial free wall, the pericardial cuff was progressively tailored and sewn onto the atrial wall to remain away from the ostia of the pulmonary veins and to maintain normal morphology of the autograft. The bovine jugular valved conduit was used to reconstruct the right ventricular outflow tract.Results:There was one early death due to sudden cardiac arrest at the night of surgery day. The remaining 5 patients were successfully recovered and discharged. Follow-up of survivors ranged from 3 to 13 months. Echocardiographic follow-up demonstrated the flow velocity across the mitral valve position was 1.5 to 2.3 m/s, with a means gradient of 4 to 6 mmHg (1 mmHg=0.133 kPa). Four patients showed mild mitral insuffiency, normal left atrium and ventricle size and left ventricle ejection fraction.One patient had moderate mitral insuffiency, pulmonary valve endocarditis, and reduced left ventricle ejection fraction. The clinical symptoms of all survivals improved significantly and the weight gain were satisfactory.Conclusion:Pulmonary autograft mitral valve replacement may be a feasible and effective remedial surgical strategy for young infants with intractable congenital mitral valve lesions.

Objective To summarize the outcomes and experience of arterial switch operation (ASO) in the past 10 years in our center.Methods From September 2008 to July 2017,238 patients underwent ASO at Guangzhou Women and Children Medical Center for repair of transposition of the great arteries and Taussig-Bing anomaly.There were 193 male and 45 female.Median age at operation was 2 months (2 days to 10 years) and mean body weight was(4.2 ± 1.7) kg (1.8-20.6 kg).There were TGA and VSD 91 cases,TGA/IVS 110 cases,and Taussig-Bing anomaly 37 cases.Among them 24 patients had an aortic arch anomaly.Intramural coronary artery was found in 8 patients.Results All patients successfully completed the operation,one-stage ASO was perfonned in 232 patients.Two-stage ASO was performed in 6 patients.The mortality was 14.2％.The follow-up duration was 1-10 years (median time,46 mouths).There were 3 died.Two suffered sudden death,and another one arrhythmia.10-year survival rate was 92.8％.Conclusion The outcomes of ASO were satisfactory.The long-term reoperation rate was rare.

Objective: To examine the early- and midterm outcomes of pulmonary artery banding as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow. Methods: Between January 2008 and December 2017, 49 patients with single ventricle and unrestricted pulmonary blood flow underwent pulmonary artery banding at Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University. There were 29 males and 20 females. The age at the time of surgery was 5.6 (11.5) months (M(Q_R)), and the weight was 5.2 (3.9) kg. The medical records and results after pulmonary artery banding (death/reoperation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death, transition to the Fontan procedure) were reviewed retrospectively. Actuarial survivals were estimated by the Kaplan-Meier curve. Relative factors for affecting outcomes were analyzed using the Cox regression hazard model. Results: There were 8 early deaths, with a mortality of 16.3%, including 4 cases who received simultaneous arch repair. There were 5 late deaths. During the follow-up of 47(62) (M(Q_R)) months, 11 patients (22.4%) underwent pulmonary artery banding adjustment, 29 patients (59.2%) underwent the Glenn procedure, 21 patients (42.8%) underwent the Fontan procedure. The survival of patients after the initial pulmonary artery banding were 77.4% (95%CI: 65.6% to 89.2%) and 72.6% (95%CI: 59.9% to 85.3%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that systemic ventricular outflow tract obstruction (HR=4.25, 95%CI: 1.50 to 12.03, P=0.006) and total anomalous pulmonary venous connection (HR=6.49, 95%CI: 3.24 to 12.98, P=0.000) were relative factors for death. Conclusions The early and midterm outcomes of pulmonary artery banding as an initial palliative strategy is not satisfactory. Systemic ventricular outflow tract obstruction and total anomalous pulmonary venous connection are associated with high mortality.

Objective: To summarize the surgical results and experience of patients with complete atrioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets. Methods: From April 2013 to June 2017, 10 patients with complete atrioventricular septal defect associated with tetralogy of Fallot or double right ventricular outlets underwent biventricular repair at Guangzhou Women and Children Medical Center. Seven were male, and 3 were female. The age and body weight at surgery was 2 months to 13 years and 3.7-23.6 kg. Repair was performed with modified one-patch technique in 3 patients, modified two-patch technique in 6 patients, two-patch technique in 1 patient. Results: There was no hospital mortality. The ICU stay and hospital stay after operation were 2~5 days and 7~10 days. The follow-up duration was 16 to 65 months. All patients were alive and free from left ventricular outlet obstruction. The left atrioventricular valve function were normal in 2 patients, mild regurgitation in 6 patients, moderate regurgitation in 1 patient and severe regurgitation in 1 patient. Conclusion The outcomes of biventricular repair for patients with complete atrioventricular septal defect associated with Tetralogy of Fallot or double right ventricular outlets were satisfied, and long-term follow-up was demanded.

Objective: To investigate the efficacy of multistage surgery in patients with functional single ventricle (FSV) and risk factors of postoperative death. Methods: The clinical data of all consecutive patients with FSV undergoing multistage single ventricle palliation surgery in Guangzhou women and children's medical center from January 2008 to December 2017 were retrospectively reviewed. The study included 289 patients. The age was 10.0 (6.0, 35.4) months,and there were 198 male and 91 female patients. The patients were followed up at outpatient clinic. Survival rates were calculated with Kaplan-Meier. Multivariate Cox regression analysis was made to determine the risk factors of postoperative death. Results: Seventy-nine patients required the first stage palliation surgery, 232 patients received the Glenn shunt surgery, and 162 patients completed the Fontan procedure. Overall, postoperative death occurred in 39 patients including 21 after the first stage palliation surgery (early stage 13 cases, late stage 8 cases) , 10 after the Glenn shunt surgery (early stage 5 cases, late stage 5 cases) , and 8 following the Fontan procedure (early stage 6 cases, late stage 2 cases) . Kaplan-Meier analysis showed that survival rate of the entire cohort was 90.2% (95%CI 86.7%-93.7%) , 85.9% (95%CI 81.8%-90.0%) ,and 84.6% (95%CI 79.7%-89.5%) at 1 year, 5 years and 10 years post operation. Survival rate was 74.4% (95%CI 64.8%-84.0%) , 73.0% (95%CI 63.2%-82.8%) , and 73.0% (95%CI 63.2%-82.8%) at 1 year, 5 years, and 10 years post the first stage palliation surgery, 97.8% (95%CI 95.8%-99.7%) , 95.2% (95%CI 92.3%-98.1%) , and 95.2% (95%CI 92.3%-98.1%) at 1 year, 5 years, and 10 years post Glenn shunt surgery, 95.6% (95%CI 92.5%-98.7%) and 93.7% (95%CI 88.8%-98.6%) at 1 year and 5 years post Fontan surgery. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (HR=5.47, 95%CI 2.71-11.04, P<0.001), atrioventricular valve regurgitation more than moderate (HR=2.52, 95%CI 1.32-4.79, P=0.005), systemic ventricular outflow tract obstruction (HR=3.47, 95%CI 1.30-9.29, P=0.013), and required the first palliation surgery (HR=3.12, 95%CI 1.59-6.15, P=0.001) were risk factors of postoperative death. Conclusions The multistage surgery can effectively improve the survival of patientswith functional single ventricle and is associated with satisfactory long-term prognosis. Total anomalous pulmonary venous connection, atrioventricular valve regurgitation more than moderate, systemic ventricular outflow tract obstruction, and required the first palliation surgery are risk factors of postoperative death in these patients.

Objective To evaluate the early- and mid-term outcomes of surgical repair of persistent truncus arteriosus in children in a single institution of China. Methods The clinical data of 27 consecutive patients with persistent truncus arteriosus undergoing surgical repair in Guangzhou Women and Children’s Medical Center from November 2009 to May 2018 were retrospectively reviewed. There were 14 males and 13 females. Median age was 3.0 months (range: 13 days -11 years), of whom 10 (37.0%) were older than 6 months. Results There were three early deaths with a mortality of 11.1%. The main complications included VSD partial repair in 2 patients, complete atrioventricular block in one patient. The mean follow-up time was 24.5±19.3 months (range: 1–76 months). There were three late deaths, and two patients lost follow. Echocardiology showed seven patients of right heart outflow tract obstruction, including three in pulmonary artery trunk, and four of pulmonary artery branches. One patient showed moderate aortic valve regurgitation. None required re-intervention during the follow-up. Survival estimates for the entire cohort following surgery were both 76.1% (95%CI 59.2% to 92.9%) at 1 year and 5 years. Conclusion The surgical repair of persistent truncus arteriosus (PTA) remains challenges. The early- and mid-term outcomes of surgical repair of persistent truncus arteriosus are acceptable. For older children with severe pulmonary artery hypertension and/or trunk valve regurgitation, the risk of death is still higher. Some children have the higher risk of late right heart obstructive lesions.

Objective To review the early-and mid-term results of anatomic repair or Fontan pathway for congenitally corrected transposition of the great arteries(ccTGA) in a single institution of China.Methods Hospital records over a 9-year period(2009-2017) were reviewed to identify patients with ccTGA who underwent anatomic repair or Fontan pathway.Pa-tient-and procedure-related variables were reviewed.Results We identified 37 patients.Group 1 consisted of 10 anatomic re-pairs, of which 4 required prior pulmonary artery banding.Median age at anatomic repair was 1.0 years( range: 0.3 -7.8 years).There was one early death, and one patients experienced ECMO support.The mean follow-up was(3.0 ±2.8)years (range:0.7-8.4 years).Five(55.5％) patients showed arrhythmias, and one required permanent pacemaker implantation during follow-up.Group 2(27 patients) underwent Fontan palliation, of which 23(85.2％) underwent prior bidirectional Glenn shunt.Median age at Fontan completion was 3.8years(range:2.2-14.3 years).there was one early death with a mor-tality of 3.7％.The mean follow-up was(2.8 ±1.6) years(range:0.8-8.2 years).There was 4(15.4％) cases of arrhyth-mias, but none required reintervention.The arrhythmias incidence in Fontan group was significantly lower than the anatomic re-pair group.The early-and mid-term survival rate were 90.0％ and 96.3％in the two groups.The difference was not statistical-ly significant(P=0.458).Conclusion Patients with ccTGA do well with both anatomic repair and the Fontan pathway in the medium term.Pulmonary artery banding can be used effectively for morphological left ventricular retraining , and extenuate tri-cuspid regurgitation.Excellent outcomes with reduced early complication and arrhythmias incidence can be achieved for this co-hort of patients when a strategy of avoiding complex anatomic repair in favor of the Fontan pathway is used .