Objective    To compare the long-term durability of valved homograft conduit (VHC) in patients with Ross and non-Ross right ventricular outflow tract (RVOT) reconstruction. Methods    Patients who underwent RVOT reconstruction using VHC in Fuwai Hospital from January 2008 to October 2020 were retrospectively included. Patients who received Ross RVOT reconstruction were allocated to a Ross group and patients who received non-Ross RVOT reconstruction were allocated to a non-Ross group. The survival and reintervention-free rates of the two groups were evaluated with the Kaplan-Meier survival curve and log-rank test. The propensity score matching analysis was performed on the patients who completed ultrasound follow-up in the two groups, and the VHC dysfunction-free rate was compared between the two groups. Results    A total of 243 patients were enrolled, including 142 males and 101 females, with a median age of 6 years (4 months to 56 years). There were 77 patients in the ROSS group and 166 patients (168 operations) in the non-ROSS group. The cardiopulmonary bypass time in the Ross group was shorter than that in the non-Ross group (175.4±45.6 min vs. 200.1±83.5 min, P=0.003). Five patients in the non-Ross group died early after the operation. The follow-up was available in 231 patients (93.1%), with the average follow-up time of 61.7±44.4 months. During the follow-up, 5 patients in the non-Ross group died. The 12-year survival rate was 100.0% in the Ross group and 93.2% in the non-Ross group (log-rank, P=0.026). In addition, 1 patient in the Ross group and 7 patients in the non-Ross group received VHC reintervention. There was no significant difference in the reintervention-free rate between the two groups (log-rank, P=0.096). Among the 73 patients in the Ross group and 147 patients in non-Ross group who were followed up by ultrasound after discharge, 45 patients (20.5%) developed VHC dysfunction. Before matching, the long-term durability of VHC in the Ross group was better than that in non-Ross group (10-year VHC dysfunction-free rate: 66.6% vs. 37.1%, log-rank, P=0.025). After the propensity score matching, 64 patients included in each group, and there was no statistical difference in the long-term durability of VHC between the two groups (10-year VHC dysfunction-free rate: 76.3% vs. 43.0%, log-rank, P=0.065). In the subgroup analysis, the 10-year VHC dysfunction-free rate in the Ross group was higher than that in the non-Ross group (71.0% vs. 20.0%, log-rank, P=0.032) among patients aged<6 years at surgery. However, there was no significant difference in the 10-year VHC dysfunction-free rate between the two groups (53.7% vs. 56.7%, log-rank, P=0.218) among patients aged ≥6 years at surgery. Conclusion    After the propensity score matching analysis, the long-term durability of VHC has no significant difference between the Ross group and non-Ross group. The long-term durability of VHC after Ross surgery is superior to that of non-Ross surgery in patients aged<6 years at surgery.

Objective    To evaluate the clinical outcome of valved homograft conduits (VHC) used for right ventricular outflow tract (RVOT) reconstruction in Fuwai Hospital in recent 13 years, and explore the factors influencing the long-term durability of VHC. Methods    Clinical data of patients using VHC for RVOT reconstruction in Fuwai Hospital from November 2007 to October 2020 were retrospectively analyzed. The Kaplan-Meier survival curve was used to evaluate survival, VHC reintervention and VHC dysfunction. Cox proportional risk regression model was used to analyze the risk factors for VHC dysfunction. Results    Finally 251 patients were enrolled, including 145 males and 106 females. The median age at surgery was 6.0 (0.3-67.0) years. Early death occurred in 5 (2.0%) patients. The follow-up was available for 239 (95.2%) patients, with the follow-up time of 0.3-160.0 (61.3±45.4) months. Five patients died during the follow-up, and the 1-year, 6-year, and 13-year survival rates were 96.6%, 95.5% and 95.5%, respectively. Eight patients received VHC reintervention during the follow-up, and freedom rates from VHC reintervention were 100.0%, 97.1% and 82.4% at 1 year, 6 years and 13 years, respectively. A total of 226 patients were followed up by echocardiography after discharge, with the follow-up time of 0.2-138.0 (48.5±40.5) months. During the follow-up, 46 (20.4%) patients developed VHC dysfunction, and freedom rates from VHC dysfunction at 1 year, 5 years, and 10 years were 92.6%, 79.6% and 59.3%, respectively. Univariate Cox regression analysis showed that age<6 years and VHC diameter<19 mm were risk factors for VHC dysfunction (P=0.029, 0.026), but multivariate regression analysis only indicated that age<6 years was an independent risk factor for VHC dysfunction (P=0.034). Conclusion    The early and late outcomes of VHC used for RVOT reconstruction are satisfactory, and the long-term durability of VHC is also optimal. In addition, age<6 years is an independent risk factor for VHC dysfunction.

Objective    To summarize the treatment results of double aortic arch (DAA) by minimally invasive surgical technique. Methods    We retrospectively analyzed the clinical data of DAA patients who underwent minimally invasive surgeries in our center between October 2016 and August 2021. Results    There were 11 males and 4 females with a mean age of 3-61 (20.00±18.80) years. There were 8 patients of DAA and 7 patients of DAA complicated with distal left-sided aortic arch atresia and ligamentum connection. All patients received operations through minimal subaxillary incision, 13 patients were through left side and 2 patients were through right side. One patient with ventricular septal defect was performed operations concurrently under the cardiopulmonary bypass through right minimal subaxillary incision. All patients had symptom improvement without surgery related complications or death in postoperative period. The duration of operation was 30-192 (61.93±40.19) min and mechanical ventilation time was 2-9 (5.33±2.53) h. The length of ICU stay was 18-124 (51.00±38.07) h and hospital stay time was 8-21 (12.67±3.42) d. All patients had sympto-matic relief with good growth and exercise tolerance during the follow-up of 6 (3, 9) months. Conclusion    Minimally invasive surgical technique is a safe, effective and cosmetic approach with good results for DAA treatment.

Objective    To analyze the surgical results of patients with supracardiac total anomalous pulmonary venous connection (TAPVC) in a single pediatric cardiac center. Methods    A retrospective study was conducted on 98 pediatric patients with supracardiac TAPVC receiving surgical repair from 2014 to 2019 in our center. There were 64 males and 34 females with a median surgical age of 3.0 (1.5, 7.0) months and a median weight of 5.0 (4.0, 6.0) kg. Twenty-three (23.5%) patients had preoperative pulmonary vein obstruction. Ninety-two (93.9%) patients received conventional surgical repair, while six (6.1%) patients were treated with the sutureless technique. The Cox regression model was used to analyze the data. Results    The median follow-up time was 26.50 (5.75, 44.25) months. There were 9 (9.2%) deaths. Lower weight at the time of repair (P=0.013) and prolonged cardiopulmonary bypass time (P=0.007) were associated with mortality. Postoperative pulmonary vein obstruction was observed in 8 (8.2%) patients. Associated risk factors for postoperative pulmonary vein obstruction included lower weight at the time of repair (P=0.042) and prolonged cardiopulmonary bypass time (P=0.002). Conclusion    Surgical repair of supracardiac TAPVC has achieved satisfactory results in our center. Risk factors such as lower weight at the time of repair and prolonged cardiopulmonary bypass time are associated with a poor prognosis.

Objective    To summarize the surgical experience of infants with transposition of the great arteries (TGA) and intramural coronary artery (IMCA) in our center, and analyze the early and mid-term outcomes. Methods    We retrospectively analyzed the clinical data of 384 infants with TGA undergoing arterial switch operation (ASO) from June 2010 to December 2018 at Fuwai Hospital. According to operative records, 21 (5.5%) infants had IMCA, among whom 20 were males, with a median age of 33 (9-319) d. Coronary transfer using double coronary buttons with unroofed intramural course was performed in all 21 infants. Results    There was no statistical difference in the early mortality after ASO between infants with IMCA and infants with normal coronary anatomy (9.5% vs. 3.0%, P=0.15). In the IMCA group, 2 dead patients presented inadequate coronary artery perfusion after first aortic unclamping. In addition, 1 patient underwent extracorporeal membrane pulmonary support for myocardial dysfunction. The follow-up was available for all 19 survivors, with an average follow-up time of 29.0-120.0 (74.8±27.3) months. During the follow-up, all patients had no obvious symptoms, death, reoperation, or coronary complications. One patient developed moderate pulmonary valve regurgitation and another patient developed distal stenosis of the right pulmonary artery. Conclusion    For infants with TGA and IMCA, coronary transfer using double coronary buttons with unroofed intramural course is a safe and reliable technique, with satisfactory early and mid-term outcomes.

Objective    To introduce a modified sutureless technique and its surgical results in the treatment of total anomalous pulmonary venous connection (TAPVC). Methods    Clinical data of 11 patients with TAPVC who underwent the modified sutureless technique treatment from 2014 to 2019 in our center were retrospectively analyzed, including 4 males and 7 females. The median surgical age was 1.4 (0.3, 27.0) months. The median weight was 4.3 (3.5, 8.5) kg. Six (54.5%) patients were of supracardiac subtype, and five (45.5%) patients were of infracardiac subtype. Five (45.5%) patients had preoperative severe pulmonary hypertension, and three (27.3%) patients had preoperative pulmonary vein obstruction. The surgical results were compared with those of 10 patients treated with conventional surgical technique. Results    The median follow-up was 12 (range, 1-65) months. During the follow-up, no death or postoperative pulmonary vein obstruction occurred in the modified sutureless technique group. The perioperative data and relief of re-obstruction were superior in the modified sutureless technique group, but the difference was not statistically significant (P>0.05). The postoperative survival of the the modified sutureless technique group was better than that of the traditional surgery group (P=0.049). Conclusion    The modified sutureless technique which includes partial suture and then incising, and eversion of pulmonary vein incision, is a safe and reliable method for the treatment of TAPVC with satisfactory short-term results.

Objective    To evaluate the mid- and long-term outcomes of different surgical techniques for subaortic stenosis. Methods    The clinical data of 75 patients with subaortic stenosis who underwent surgery in our hospital from January 2008 to January 2018 were retrospectively analyzed, including 48 males and 27 females, with a median age of 72 (48, 132) months and mean weight of 21.35±15.82 kg. There were 40 (53.3%) patients combined with aortic regurgitation; 38 (50.7%) patients were the first time and 37 patients were the second time to receive the operation. According to the surgical techniques, 75 patients were divided into two groups: a group A (40 patients with simple subaortic membrane resection) and a group B (35 patients with subaortic membrane and muscle resection or modified Konno procedure). Results    Two (2.67%) patients died in hospital. There was one late death in the group B. The average preoperative and postoperative pressure gradient of all patients was 69.96±42.02 mm Hg and 7.44±12.45 mm Hg, respectively. All patients were followed up for 51 (12, 120) months. Pressure gradient at follow-up in the group A and the group B was 8.83±14.52 mm Hg and 5.86±9.53 mm Hg, respectively with no statistical difference (P=0.294). Four patients in the group A and 2 patients in the group B needed reintervention. However, there was no statistical difference in the long-term reintervention rate between the two groups (P=0.480). Conclusion    For the different degree of lesions in the left ventricular outflow tract, our management strategy is feasible. Although there is no statistical difference between two the groups in the long-term reintervention rate after simple valvular membrane resection, prolonged follow-up is necessary to examine the long-term outcomes of different surgical techniques.

Objective    To investigate the best anatomical classification, surgical timing, procedure and clinical outcomes of congenital vascular ring. Methods    The clinical data of 58 patients who underwent congenital vascular ring surgery in Pediatric Surgery Center, Fuwai Hospital between 2014 and 2019 were retrospectively analyzed. There were 32 (55.2%) males and 26 (44.8%) females with a median age of 16.5 (2-73) months. Preoperative symptoms, imaging examinations, anatomical classifications, surgical procedures and postoperative recovery were assessed. Results    There were 20 (34.5%) patients of double aortic arch, 22 (37.9%) patients of right aortic arch with left arterial duct or ligament, 15 (25.9%) patients of left aortic arch with aberrant right subclavian artery, and 1 (1.7%) patient of circumflex aorta with cervical aorta arch. The median ventilator supporting time was 6.0 (0-648) h, and the median hospital stay time was 14.5 (7-104) d. One patient with coarctation of aorta died of severe pulmonary infection during perioperative period, and the others survived without symptoms and reoperation after discharge. The median follow-up time was 7.0 (1-62) months. Conclusion    For children with unexplained dyspnea and dysphagia, or with right aortic arch, preoperative imaging examinations such as computed tomography or magnetic resonance imaging are required to confirm the diagnosis of vascular ring. Surgical correction of congenital vascular ring is safe and reliable, and can effectively relieve symptoms. The mortality rate and reoperation rate are low, and the follow-up results are satisfactory.

We reported a case of a six-year-old boy diagnosed of single ventricle, pulmonary atresia and interrupted inferior vena cava. After modified Blalock-Taussig shunt and bidirectional Glenn procedure, he received the Fontan procedure. The Fontan procedure was done through a unilateral thoracotomy, using an autologous pericardial conduit to connect hepatic vein and azygos vein. The result of short-term follow-up was satisfactory.

Objective    To explore risk factors associated with mortality and restenosis after the surgery for congenital pulmonary venous stenosis (CPVS) combined with congenital heart disease. Methods    From May 2007 to August 2019, 58 patients received surgical relief of CPVS combined with congenital heart disease, including 24 males and 34 females, aged 17.2±26.3 months, weighing 8.8±8.2 kg. Endpoints were death and restenosis, and the risk factors were analyzed. A univariate and multivariate risk analyses were performed. Results    Preoperative pulmonary venous stenosis severity score (PVSSS) was 4.5±2.7. Average pulmonary vein counts with CPVS was 1.9±1.0. There were 2 (3.4%) early deaths. The mean follow-up time was 2-145 (49.8±40.0) months. The 1-, 2-, 3- and 5-year overall survival rates were 86.7%, 81.3%, 78.5% and 73.6%, respectively, and the pulmonary venous restenosis-free rates were 79.6%, 68.5%, 68.5% and 68.5%, respectively. Preterm birth was an independent risk factor for mortality. The pulmonary venous peak flow rate ≥ 1.2 m/s at discharge was an independent risk factor for mortality and restenosis. Conclusion    The prognosis of CPVS is still poor. Postoperative residual stenosis at discharge is an independent risk factor for death and restenosis.