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Objective:To summarize the results of surgical treatment for complete atrioventricular septal defect(CAVSD) in early and middle stages.Methods:147 children with CAVSD in Guangzhou Women and Children’s Medical Center from January 2010 to December 2019 were selected, Males 85, females 62, median age of surgery 5 months(1 months-10 years old), median body mass 5.5 kg(2.4-20.9 kg). Complete atrioventricular septal defect was diagnosed by ultrasonic cardiogram before surgery. All the children underwent atrial ventricular valve formation and underwent simultaneous repair.Outpatient follow-up was planned.Ultrasonic cardiogram and electrocardiogram were performed. SPSS 22 statistical software was used for data analysis.Results:All 147 CAVSD patients underwent one-time surgical correction.Early postoperative death occurred in 7 cases(4.76%). The causes of death were: 3 cases of pulmonary hypertension crisis, 3 cases of severe mitral insufficiency(MI), 1 case of postoperative malignant arrhythmia, and the rest of the children were cured and discharged. Permanent pacemaker was installed in 3 patients due to atrioventricular block(AVB). The follow-up time was 1-10 years old, and 2 patients died in late stage: 1 patient did not seek medical treatment in time due to infection, and 1 patient had unknown cause. Five patients underwent secondary surgery: 4 due to severe mitral/tricuspid insufficiency(MI/TI) and 1 due to delayed AVB. The mid-term follow-up showed 9 cases of severe MI and 4 cases of severe TI. Compared with children with surgical age<3 months and ≥3 months, there were statistically significant differences in postoperative ventilator-assisted ventilation time, severe MI before postoperative discharge and total mortality between the two groups( P<0.05). Mid-term follow-up results showed no difference between the two groups. There were statistically significant differences in surgical age, postoperative CICU stay time and total hospital stay between the children with trisomy 21-syndrome and those without trisomy 21-syndrome( P<0.05), and there was no difference between the two groups in mid-term follow-up results. Residual shunt of 1-3 mm VSD was found in 29 cases, 26 cases were closed during follow-up, and 3 cases had smaller residual shunt. Conclusion:Modified single patch technique treatment of CAVSD has good effect, low mortality and low re-operation rate. But age <3 months group, infant mortality was significantly increased, the duration of postoperative mechanical assisted ventilation was prolonged, and the proportion of early postoperative severe MI was high.Severe MI and TI is easy to occur after CAVSD, which requires long-term follow-up and timely treatment. The children with trisomy 21-syndrome were similar to those with normal chromosome except for longer stay in ICU and total hospital stay.
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@#Objective To compare the early and mid-term results between Fontan operation and anatomic correction for congenitally corrected transposition of the great arteries (ccTGA). Methods The clinical data of 53 patients with ccTGA who underwent anatomic correction and Fontan operation from January 2009 to September 2021 in our hospital were reviewed, including 41 males and 12 females with a mean age of 55.02 (3-168) months. They were divided into an anatomic correction group (16 patients) and a Fontan operation group (37 patients) according to the operation. The hospitalization mortality, survival rate, postoperative complications, and free rate from re-intervention between the two groups were compared. Another 180 healthy children were recruited as a control group, and 14 children were matched with the propensity score matching method as a Fontan control group. The results of cardiopulmonary exercise testing (CPET) between the Fontan operation group and the Fontan control group were compared. Results There were 2 (12.5%) early deaths and 3 (18.8%) early re-intervention in the anatomic correction group, while 1 death and 2 re-intervention in the Fontan operation group. In addition, there were 9 patients (56.3%) in the anatomic correction group and 6 (16.2%) patients in the Fontan operation group suffering from arrhythmia after operation, respectively. Compared with the anatomic correction group, cardiopulmonary bypass time, aortic cross-clamping time, intubation time and ICU stay were significantly shortened in the Fontan operation group (P<0.05). CPET results showed that, percent predicted max VO2 in the Fontan operation group was lower than that in the Fontan control group (0.84±0.11 vs. 0.99±0.12, P<0.05). The patients were followed up for 0.5-126.0 months. Two patients were lost in the Fontan operation group. There was no death and 1 re-intervention in the anatomic correction group, while no death or re-intervention in the Fontan operation group. The 1-year, 5-year and 10-year transplant-free survival rate of the anatomic correction group and the Fontan operation group was 87.5%, 87.5%, 87.5% and 97.3%, 97.3%, 97.3%, respectively (P>0.05). The 48 patients were classified as grade Ⅰ-Ⅱ in cardiac function in the last follow-up. Conclusion There is no statistical difference in the transplant-free survival rate between the anatomic correction and the Fontan operation group. The postoperative complications in the Fontan operation group are decreased than those in the anatomic correction group. The Fontan operation is also a good choice, even though the patients with ccTGA meet the condition of the procedure of anatomic correction.
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Objective:To summarize the early and middle terms of the revascularization of remnant pulmonary artery in unilateral absent intrapericardial pulmonary artery.Methods:We retrospectively analyzed the medical records of 14 patients(7 males and 7 females) with unilateral absent pulmonary artery, in which 10 were right and 4 were left, the median age at surgery was 5 months. The patients received operation from January 2009 to December 2020. 14 patients, 2 cases associated with tetralogy of Fallot, and 1 case with aortopulmonary window. The diagnosis was made by enhanced CT scan or pulmonary vein wedge angiography. The median diameter of the affected hilar pulmonary artery remnants was(3.20±0.94)mm, and the Z value was -3.92±1.64. All the patients received single-stage revascularization: group A: tube graft interposition in 3 patients, autologous pericardial roll in 5; group B: direct anastomosis in 2, unifocalization in one and main pulmonary artery flap angioplasty in the rest 3.Results:No hospital death occurred. There were no difference finds in the age and weight at operation, the cardiopulmonary bypass time, the mechanical ventilation time, and the length of intensive care unit stay between the two groups. All the patients took aspirin for anticoagulation for 6 months after the operation. The follow-up period was 1 month to 68 months. Because the neo-PA stenosis at the anastomosis was found in one patient in group B, transcatheter balloon angioplasty was performed at 41 months after surgery. Nonetheless, the results were encouraging, symptoms have improved in all patients. The diameter and Z value of the latest ipsilateral pulmonary artery was(6.25±0.99)mm and -2.34±1.18 respectively, significantly improved when compared to the preoperative value. Residual pulmonary artery hypertension was not found. The Z value of the affected side of the pulmonary artery in group B was significantly improved than that in group A.Conclusion:Early and aggressive pulmonary artery revascularization is effective at restoring normal antegrade flow to the affected lung, resulting in improved diameter of the PA, and UAPA patient’s symptoms. The use of autologous pulmonary artery tissue angioplasty may reach a more satisfying result. However, transcatheter intervention may diminish the new pulmonary artery stenosis temporarily, reoperation is still needed in the long-term follow-up.
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@#Objective To summarize the results of surgical treatment for 79 patients with coarctation of the aorta (CoA) combined with complex anomalies (CA) in recent years. Methods The data from 79 patients with CoA combined with CA admitted to Guangzhou Women and Children's Medical Center between January 19, 2010 and September 7, 2017 were collected and analyzed. There were 52 males and 27 females. The median age was 71 days, and the median weight was 4.3 kg. There were 26 patients combined with tracheostenosis and 7 patients with preoperative tracheal intubation. Extended end-to-side anastomosis was used to correct the CoA, and the associated cardiac abnormalities were treated simultaneously by an incision through median sternotomy. All operations were performed by the same group of surgeons. Results The median deep hypothermic circulatory arrest was 18 (13-28) minutes, the median aorta cross-clamp time was 62 (15-199) minutes, the median cardiopulmonary bypass time was 145 (71-674) minutes, the median ventilation time was 72 (9-960) hours, the median length of ICU stay was 144 (12-1 944) hours, and the median length of hospital stay was 24 (2-93) days. Early death occurred in 9 patients and late death occurred in 5 patients. Reoperation occurred in 28 patients and recoarctation developed in 10 patients. After operation, transcoarctation gradient was reduced, and the transcutaneous oxygen saturation, C-reactive protein (CRP), lactate and creatinine were increased. Compared to the survival group, both preoperative and postoperative transcutaneous oxygen saturation were worse and the postoperative levels of plasma brain natriuretic peptide (BNP) and CRP were higher in the death group. Conclusion Correcting the CoA and the associated CA simultaneously with extended end-to-side anastomosis by an incision through median sternotomy is effective and safe, and the outcomes of immediate and medial-term are satisfactory. Improving oxygenation and limiting elevation of BNP and CRP levels may reduce the death rate.
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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.
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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(QR)), 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(QR)) 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.
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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.
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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 .
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Objective To analyze the treatment and follow-up of congenital coronary artery fistula (CAF) with giant coronary artery aneurysm (GCAA) in children.Methods The clinical data were analyzed retrospectively in 13 patients who were diagnosed as congenital CAF with GCAA between July 2009 and December 2016 in Guangzhou Women and Children's Medical Center.There were 8 boys and 5 girls.The median age was 18 months,ranging from 40 days to 12 years old.The body weight ranged from 3.8 kg to 29.0 kg with a median of 8.8 kg.Fistulas originated from right coronary artery accounted in 8 patients,with 5 from left coronary artery.Fistulas drained into right atrium in 3 patients,right ventricular in 8 patients and left ventricular in 2 patients.Single fistula occurred in 12 patients and multiple fistulas in 1 patient.The diameter of coronary artery aneurysm ranged from 8 mm to 16 mm with a median of 9.2 mm.Results One patient had tachypnea and growth retardation without heart murmur.The other 12 patients were asymptomatic with heart murmurs occasionally found in routine physical examination.One patient underwent fistula ligation without cardiopulmonary bypass (CPB).The remaining 12 cases received fistula correction with beating heart CPB.Direct suture was used in 10 patients and autologous pericardial patch in other 2 patients.Two patients were associated with atrial septal defect (ASD) and underwent repair of ASD concurrently.The coronary artery aneurysm remained original shape without any intervention during the operation.The mean hospital delay was (11.0 ± 2.5) days.Two patients had decreased ejection fraction as low as 38％ within 3 days after the operation,but went up to over 50％ in follow-up 1 month later.Transient T wave change occurred in 7 patients,and another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.All 13 patients had antiplatelet therapy with 12 taking Aspirin and one taking Dipyridamole.The dosage was 3-5 mg/(kg · d) with duration ranging from 3 days to 13 months with a median of 1 month.During the perioperative period and the follow-up period (3 months to 8 years),all patients were asymptomatically alive.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status,the diameter of dilated coronary arteries was not changed after the operation.There was no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia.Conclusions GCAA can be combined with congenital CAF in children,so it needs early operation.The evidence-based intervention of coronary artery aneurysm and usage of anticoagulant and antiplatelet therapy in pediatric patients was still lacking,which needs long-term follow-up.
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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.
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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 pa-tients 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 nor-mal 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 Tet-ralogy of Fallot or double right ventricular outlets were satisfied, and long-term follow-up was demanded.
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Objective@#To review the early and mid-term results of end-to-side anastomosis technique for interrupted aortic arch in neonates and infants.@*Methods@#Clinic data of 46 patients were diagnosed as interrupted aortic arch in Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center between January 2010 and December 2016 were analyzed retrospectively. Twenty-six cases were neonates. The median age underwent surgery was 23 days (range: 2 days to 8 years). Anatomical subtypes included 36 cases of type A and 10 cases of type B. There was no type C case. The reconstruction of the aortic arch was completed by an extended end-to-side anastomosis technique between the descending aorta and the undersurface of the proximal aortic arch. In 42 patients, all with intracardiac anomalies, had concomitant complete repair of intracardiac anomalies through a median sternotomy. The remaining 4 patients, all without intracardiac anomalies, an end-to-side anastomosis was constructed through a left thoracotomy. During follow-up, aortic arch recurrent obstruction, left ventricular outflow tract obstruction (LVOTO) and tracheal stenosis were focused.@*Results@#There were 3 surgical deaths, with amortality rate of 6.5%. The remaining 43 patients survived after surgery. In 39 of these patients, deep hypothermic cardiac arrest (DHCA) strategy was used for brain protection, and the mean time of DHCA was (16±3) minutes. Eight patients underwent delayed sternal closure. The mean mechanical ventilation time and ICU stay time were (3.4±1.6) days (range: 2 to 12 days) and (6.4±2.7) days (range: 3 to 16 days) respectively. In 16 patients, all with tracheal or bronchial stenosis before surgery, mechanical ventilation was successfully evacuated, and no new airway stenosis occurred. There was no residual pressure difference between upper and lower extremity arterial blood pressure at discharge. Echocardiography showed normal arterial blood flow velocity in aortic arch. At a mean follow-up of (36.2±18.9) months (range: 6 months to 7 years), there were two patients lost and one late date. Four patients developed a recurrent stenosis at the aortic arch, of which two were severe, and the other two were mild. In 2 patients, both with mild LVOTO before surgery, no significant increase in the degree of obstruction was found during the follow-up. Two patients developed new mild to moderate LVOTO without clinical symptoms, and continued to follow up. In all patients, the tracheal or bronchial stenosis were extenuated, and there was no new progressive airway stenosis by regular bronchoscopy.@*Conclusions@#The end-to-side anastomosis technique for the reconstruction of the aortic arch achieved excellent early and mid-term results in neonates and infants suffered from interrupted aortic arch. Reducing the anastomotic tension by extensive mobilization is the key to prevent postoperative early complications and late recurrent arch obstruction.
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Objective@#To review current-era palliation outcomes of patients with heterotaxy and functional single ventricle in a single institution.@*Methods@#The clinical data of 70 consecutive patients with heterotaxy undergoing multistage single ventricle palliation in Guangzhou Women and Children′s Medical Center from January 2008 to December 2017 were retrospectively reviewed, and the prognosis factors for mortality were analyzed. There were 53 male and 17 female patients.The median age was 13.3 months (range: 6 days to 150 months). Single ventricle multistage palliation included 1st stage palliation surgery, 2nd bidirectional Glenn shunt, and 3rd stage modified Fontan.The Kaplan-Meier method was used to estimate the probability of survival. Multivariate analysis was performed by Cox regression model.@*Results@#Sixty-two patients had right atrial isomerism while eight had left atrial isomerism. Eighteen patients (25.7%) required the first stage palliation. Fifty-five patients received the Glenn shunt, and the Fontan procedure was completed in 27 patients. Overall, mortality occurred in 17 patients (24.3%) including 9 after the first stage palliation, 6 after the Glenn shunt, and 2 following the Fontan procedure. Survival estimates for the entire cohort following surgery were 81% (95%CI: 72% to 90%), 74%(95%CI: 64% to 85%), and 74% (95%CI: 64% to 85%) at 1 year, 5 years and 10 years, respectively. Survival estimates following the first stage palliation were 56% (95%CI: 33% to 79%) and 49% (95%CI: 26% to 73%) at 1 year and 5 years, respectively. Multivariate Cox regression analysis revealed that total anomalous pulmonary venous connection (TAPVC) (HR=6.16, 95%CI: 1.65 to 22.95, P=0.007), atrioventricular valve regurgitation more than moderate (HR=3.81, 95%CI: 1.32 to 10.94, P=0.013) and required the first palliation surgery (HR=4.58, 95%CI: 1.34 to 15.72, P=0.016) were prognosis factors for overall mortality.@*Conclusions@#The management of heterotaxy patients with functional single ventricle remains challenging, and the outcomes are continously improving in china. TAPVC, atrioventricular valve regurgitation more than moderate and required the first palliation surgery still are prognosis factors for overall mortality in heterotaxy syndrome.
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Objective@#To evaluate the early- and mid-term outcomes of surgical atrioventricular valve (AVV) intervention in patients with functional single ventricle (FSV).@*Methods@#The clinical data of 40 consecutive FSV patients who underwent surgical AVV intervention between January 2008 and December 2017 at Department of Cardiac Surgery, Guangzhou Women and Children′s Medical Center, Guangzhou Medical University were reviewed retrospectively. There were 25 male and 15 female patients. The median age at AVV intervention was 4.5 to 204.0 months (M(QR): 42.5 (59.7) months), and the median body weight was 6.0 to 55.4 kg (M(QR): 13.8 (8.1) kg). The systemic AVV morphology included common AVV in 21 patients, mitral valve in 7 patients, tricuspid valve in 3 patients, and both mitral and tricuspid valve in 9 patients. At first surgical intervention, 15 patients had severe, 25 patients had moderate AVV regurgitation. The timings of the initial surgical intervention were at the first palliative, Glenn, between Glenn and Fontan, and Fontan stage in 5, 23, 4, and 8 patients, respectively. The methods of the initial surgical intervention were AVV repair in 31 patients, AVV replacement in 9 patients. Actuarial survivals were estimated by the Kaplan-Meier method. Prognosis factors for atrioventricular valve reoperation were analyzed using the Cox regression hazard model.@*Results@#There were 6 early deaths, with a mortality of 15.0%. Thirty-four survival patients received a follow-up of 1 to 117 months (M(QR): 44 (34) months). Survival of patients after the initial surgical intervention were 85.0% (95% CI: 74.0% to 95.9%), 79.3% (95% CI: 66.6% to 92.0%), and 79.3% (95% CI: 66.6% to 92.0%) at 1 year, 5 years and 10 years, respectively. In all, only 13 patients completed the Fontan procedure. Six patients underwent 8 reoperations, including AVV replacement in 5 patients, redo AVV repair in 3 patients. Freedom from AVV reoperation at 1 and 5 years was 89.8% (95% CI: 78.8% to 100%) and 79.4% (95% CI: 64.7% to 94.1%), respectively. Multivariate Cox regression analysis revealed that common AVV (HR=3.53, 95% CI: 1.63-7.67, P=0.020) was the prognosis factors for AVV reoperation.@*Conclusions@#The early- and mid-term outcomes of surgical AVV intervention in FSV patients are not satisfactory. The mortality and reoperation rate are still high, and common AVV predicts the AVV reoperation after the initial surgical intervention.
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Objective To review the surgical outcomes of single-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction in a single institution of china.Methods The clinical data of 26 consecutive patients with Taussig-Bing anomaly or TGA and aortic arch obstruction undergoing single-stage arterial switch with aortic arch reconstruction in Guangzhou Women and Children's Medical Center from November 2009 to December 2015 were retrospectively reviewed,and the risk factors for re-intervention were analyzed.Results There were three in-hospital deaths,and the earlyterm survival rate was 88.5％ (23/26).Follow-up data were available for all patients who survived the operation(range 24-103 months).There were two late deaths.Survival estimates for the entire cohort following surgery were 84％ (95％ CI:70％-98％) and 77％ (95％ CI:58％-96％) at 1 year and 5 years,respectively.Echocardiology showed three cases of aortic regurgitation,two cases of supra-aortic stenosis,one case of recoarctation,seven cases of right heart outflow tract obstruction,and two cases of moderate pulmonary regurgitation.Six patients required a re-intervention during the follow-up period with no mortality.Freedom from re-intervention were 90％ (95％ CI:78％-99％) and 59％ (95％ CI:32％-86％) at 1 year and 5 years,respectively.Freedom from re-intervention for right-sided outflow tract obstruction were 94％ (95％ CI:84％-99％)and 66％ (95％CI:37％-95％) at 1 year and 5 years,respectively.All survivors remained in good condition(New York Heart association functional class Ⅰ or Ⅱ).Conclusion Single-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction had favorable early-and mid-term outcomes.The incidence of right heart outflow tract obstruction is still higher.The optimal operative procedure should be chosen according to the long-term follow-up.
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Objective Analyze the surgical and follow-up outcomes of congenital coronary artery fistula(CAF) in children and to discuss the treatment methods.Methods The clinical data were analyzed retrospectively in 22 patients who were diagnosed as congenital CAF and received surgical treatment between July 2008 and January 2017 in Guangzhou Women and Children's Medical Center.There were 14 boys and 8 girls.The median age was 17 months ranging from 14 days to 12 years old.The median weight was 8.8 kilograms ranging from 3.0 to 31.0 kilograms.Results Two patients had shortness of breath.Three had growth retardation with recurrent respiratory infection or tachypnea.The remaining 17 patients were asymptomatic.Twenty had heart murmurs.Fistulas originated from right coronary artery accounted for 11 patients,with 10 from left coronary artery,1 from both right and left coronary arteries.Fistulas drained into coronary sinus in 1 patient,right atrium in 6 patients,right ventricular in 13 patients and left ventricular in 2 patients.Single fistula occurred in 20 patients and multiple fistulas in 2 patients.The coronary arteries were obviously dilated in all patients with diameter from 4.0 to 12.0 millimeters.There were 8 cases with aneurysmal dilation of coronary arteries.Two patients underwent fistula ligation without cardiopulmonary bypass(CPB).The remaining 20 cases received fistula correction with beating heart CPB.Direct suture was used in 3 patients for closure of fistula inlet and 11 children for outlet.Both inlet and outlet were closed in 6 patients,and in 2 of them autologous pericardial patch was used for outlet.Other associated intra-cardiac anomalies in 7 children were corrected during the operation.The aneurysmal coronary arteries remained original shape without any intervention during the operation.The mean hospital delay was(12.3±3.2) days.Two patients appeared decreased heart function within 3 days after the operation,and recovered in return visit one month later.Another 2 patients showed a residual shunt with size of 1 to 2 millimeters through the fistula without further intervention after the surgical closure.During the perioperative period and the follow-up period(3 months to 8 years),all 22 patients were asymptomatically alive without regular anticoagulation management.Transthoracic echocardiography showed normal cardiac function.Compared with preoperative status,the diameter of dilated coronary arteries was reduced over 6 months follow up after the operation.There were no formation of thrombus in the coronary arteries.Electrocardiography showed no ST-T changes or arrhythmia or myocardial ischemia.Conclusion Clinical symptoms can be appeared in children with congenital CAF due to large shunt.Surgical correction is an effective method for the management of single CAF or CAF with coexisted intra-cardiac anomalies.The outcome was well.
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Objective To summarize the revascularization surgery results of patients with unilateral absence of a pulmonary artery.Methods From November 2014 to July 2016,5 patients with unilateral absence of a pulmonary artery underwent surgical revascularization at Guangzhou Women and Children Medical Center.The age and body weight at surgery was 1-6 months and 3.9-5.8 kg.One patient was diagnosed with unilateral absence of left pulmonary artery.The other 4 patients were diagnosed with unilateral absence of right pulmonary artery.Three patients were diagnosed with isolated unilateral absence of a pulmonary artery.One was associated with Tetralogy of Fallot.One was associated with atrial septal defect.Revascularization was performed by connection of hilar artery and the main pulmonary artery,using direct anastomosis,Gore-Tex conduit or pericardial conduit.Results There was no mortality.The ICU stay after operation were 3-14 days.The follow-up duration was 14-34 months.All patients were alive and free from significant pulmonary stenosis.Conclusion Revascularization surgery for patients with unilateral absence of a pulmonary artery is effective and achieved satisfactory results.
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Objective To summarize the surgical results of patients with quadricuspid aortic valve and aortic regurgitation.Methods From June 2013 to June 2017,4 patients with incompetent quandricuspid aortic valve underwent surgical repair at Guangzhou Women and Children's Medical Center.The age at surgery was 2 months to 5 years,and body weight was 2.7-22.7 kg.3 patients were diagnosed with persistent tmncal arteriosus and underwent complete repair.Another one was diagnosed with tetralogy of Fallot and accepted complete repair 4 years age.All patients were diagnosed with more than moderate quandricuspid aortic valve regurgitation.Repair was performed by tricuspidalization of the native quadricuspid valve,using leaflet and related sinus of Valsalva excision.Results There was no mortality.The ICU stay and hospital stay after operation were 7-12 days and 10-16 days.The follow-up duration was 3 to 51 months.All patients were alive and free from significant aortic valve regurgitation.Conclusion Aortic valve remodeling by leaflet excision and reduction annuloplasty is an effective method for incompetent quadricuspid aortic valve repair.
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Objective We reviewed our experience with modified Fontan in patients with functional single ventricle and apicocaval juxtaposition with a special focus on route selection and outcomes.Methods From January 201 1 to April 2016,31 patients with functional single ventricle and apicocaval juxtaposition underwent modified Fontan procedure at our hospital.Mean age at operation was(58.5 ± 32.4) months(ranged 16 months to 14 year),and the mean body weight was (16.2 ± 6.0) kg (range,9.1-40.3 kg).There were right atrial isomerism 7 cases,complete atrioventricular canal defect 9 cases,≥moderate atrioventricular valve regurgitation 4 cases,total anomalous pulmonary venous connection 8 cases,and anomalous hepatic venous connection 4 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results In 24 patients,the extracardiac conduit was placed between the inferior vena cava(IVC) and the ipsilateral pulmonary artery behind the ventricle.In the 5 other patients,the conduit was placed between the IVC and the contralateral pulmonary artery.In another 2 patients the tube graft was anastomosed with the contralateral pulmonary artery(intraextracardiac Fontan).After operation,the peripheral blood oxygen saturation was 0.90 ±0.06.Five patients suffered pleural effusion,and 1 patient died of infection during hospitalization.The follow-up duration was(17.5 ± 15.4) months(1-65 months).There was no late death and reoperation.Conclusion Modified Fontan procedure in functional single ventricle and apicocaval juxtaposition can be carried out with favorable midterm outcomes.Care must be taken in regard to critical conduit oppression by the ventricle or compression on pulmonary veins.
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Objective We reviewed our experience with modified Fontan in patients with functional single ventricle and apicocaval juxtaposition with a special focus on route selection and outcomes.Methods From January 201 1 to April 2016,31 patients with functional single ventricle and apicocaval juxtaposition underwent modified Fontan procedure at our hospital.Mean age at operation was(58.5 ± 32.4) months(ranged 16 months to 14 year),and the mean body weight was (16.2 ± 6.0) kg (range,9.1-40.3 kg).There were right atrial isomerism 7 cases,complete atrioventricular canal defect 9 cases,≥moderate atrioventricular valve regurgitation 4 cases,total anomalous pulmonary venous connection 8 cases,and anomalous hepatic venous connection 4 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results In 24 patients,the extracardiac conduit was placed between the inferior vena cava(IVC) and the ipsilateral pulmonary artery behind the ventricle.In the 5 other patients,the conduit was placed between the IVC and the contralateral pulmonary artery.In another 2 patients the tube graft was anastomosed with the contralateral pulmonary artery(intraextracardiac Fontan).After operation,the peripheral blood oxygen saturation was 0.90 ±0.06.Five patients suffered pleural effusion,and 1 patient died of infection during hospitalization.The follow-up duration was(17.5 ± 15.4) months(1-65 months).There was no late death and reoperation.Conclusion Modified Fontan procedure in functional single ventricle and apicocaval juxtaposition can be carried out with favorable midterm outcomes.Care must be taken in regard to critical conduit oppression by the ventricle or compression on pulmonary veins.