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@#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.
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@#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.
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@#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.
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@#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.
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@#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.
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@#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.
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@#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.
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@#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.
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@#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.
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@#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.
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@#Objective To summarize the clinical characteristics and outcome of tricuspid valve replacement (TVR) in children aged no more than 14 years, and to discuss the selection of prosthesis. Methods From September 2002 to August 2019, 14 patients aged no more than 14 years who received TVR were included in our study. There were 9 males and 5 females, with a mean age of 9.8±4.3 years. Results Mechanical prosthesis was implanted in 8 patients, and bioprosthesis in 6 patients. The mean cardiopulmonary time and aortic-clamp time was 170.3±109.8 min and 95.1±63.1 min, respectively. The mortality within 30 days after surgery was 21.4% (3/14), and all 3 patients died of severe low cardiac output syndrome. Eleven patients were followed up for 34-199 (100.1±57.4) months. During the follow-up, mechanical prosthesis dysfunction occurred in 3 patients, 2 of whom received secondary TVR. One patient died during the follow-up. Conclusion The bioprosthesis is the first choice for TVR in children. Some long-term complications may occur after TVR, and close follow-up and timely intervention are needed.
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@#In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival rate. However, the resulting Fontan physiology is associated with high morbidity. Despite large improvements in outcomes of contemporary Fontan patients, a large burden of disease exists in this patient population. In face of the imbalance of medical resources in China and different understanding of Fontan treatment in various regions, there is no consensus on the current status of Fontan strategy, surgical technique and perioperative treatment. By reviewing large amounts of literature, assessing survival rate and risk factors for mortality and complications of the Fontan circulation, knowledge sharing from tens of experts, we achieved a consensus on many aspects of palliation of Fontan. We hope this consensus will help Chinese colleagues further continue their efforts to improve surgical outcomes of the univentricular heart patients.
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@#Objective To investigate effectiveness and safety of right vertical infra-axillary thoracotomy (RVIAT) in surgical repair for intra-cardiac anomalies combined with patent ductus arteriosus (PDA). Methods We retrospectively analyzed the clinical data of 34 patients who underwent intra-cardiac correction of congenital heart defects and PDA ligation simultaneously via RVIAT in our hospital from August 2014 to August 2019. There were 25 males and 9 females with an age range of 0.5-6.1 years. Results The length of incision was 3.0 (3.0, 3.5) cm. The operation time lasted 110.0 (90.0, 121.0) min. The cardiopulmonary bypass time was 45.5 (38.8, 63.5) min and the aortic cross-clamp time was 22.5 (14.8, 34.8) min. The bleeding volume was 20.0 (13.8, 20.0) mL. ICU stay time was 17.0 (5.5, 22.3) h, post-operative mechanical ventilation time was 4.0 (2.0, 6.0) h, total in-hospital cost was 46 (39, 51) thousand yuan. There was no mortality or reoperation during perioperative and follow-up period. Within the median follow-up of 636.0 days, 1 patient had minimal residual ventricular septal defect shunt while no new-onset scoliosis, funnel chest or pectus carinatum was detected. No bilateral mammary developmental asymmetry was observed in the female patients during the follow-up period. All the patients’ parents or guardians were satisfied with the right vertical infra-axillary aesthetic skin incision. Conclusion The minimally invasive repair for intra-cardiac heart defects combined with PDA via RVIAT is a safe and effective method with minimal invasiveness and excellent cosmesis.
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Objective To summarize the surgical outcomes of neonates with critical and complex congenital heart diseases in the last 15 years to determine the optimal operation time and improve the prognosis.Method From January 2004 to December 2018,data of patients with complex congenital heart disease (CHD) who received surgery in our center were retrospectively analysed.All patients were diagnosed using echocardiography,some patients also received multidetector computed tomography (MDCT) examination.According to the operation date,patients were assigned into three groups:2004-2008,2009-2013 and 2014-2018 groups.Follow-up data were obtained from outpatient check-up and telephone interview.Result A total of 357 (261 males and 96 females) neonates were included.They received the operation at 2-28 days after birth and their body weights were 2 100 ~ 4 900 g.13.4% (48/357) were diagnosed before birth.Percutaneous oxygen saturation (SpO2) < 0.95 were detected in 315 patients (88.2%,315/357),among whom 60 had SpO2 ≤0.60(19.0%,60/315).Cardiac murmur were detected in 181 patients (50.7%,181/357).Among the 357 patients,221 neonates had transposition of the great arteries (TGA),55 had total abnormal pulmonary venous connection (TAPVC),43 had pulmonary atresia and intact ventricular septum (PA/IVS) and severe pulmonary stenosis and intact ventricular septum (SPS/IVS),20 had severe coarctation of the aorta (CoA)/interrupted aortic arch (IAA)/severe aortic stenosis (AS)/hypoplastic left heart syndrome (HLHS) and 18 had other types of complex CHD.The rate of onestage repair was 83.8% (299/357) and 58 neonates underwent palliative surgery.28 patients died in hospital (7.8%,28/357).The rate of prenatal diagnosis in three groups increased [3.0% (2/67),6.3% (7/111),21.8% (39/179)] and in-hospital mortality of all patients,especially patients with TGA,were remarkably decreased in the last 15 years [11.9% (8/67),5.4% (6/111),2.8% (5/179)].329 patients survived after surgery and discharged.The follow-up rate was 97.9% (322/329),7 patients were lost during follow-up,the median duration of follow-up was 63 months (3 ~ 183 months).5 died during follow-up,9 underwent second-stage radical operation,3 had re-operation due to the RVOT/LVOT stenosis.The majority of survivors were asymptomatic and with Ⅰ degree (NYHA classification) during follow-up.Conclusion Cyanosis is the most common manifestation in neonates with complex CHD.The rate of prenatal diagnosis increased gradually in the last 15 years,and in-hospital mortality of all patients,especially patients with TGA,was remarkably decreased.Most symptomatic neonates with complex CHD can survive radical operations with high success rate and good curative effect.Palliative procedures are also important for critical and complex CHD.
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Objective@#To investigate the outcomes of coarctation resection and aortoplasty with autologous pulmonary artery patch for treating coarctation of the aorta combined with hypoplastic aortic arch in infants.@*Methods@#Clinical data of 21 infants with coarctation of the aorta and hypoplastic aortic arch, who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch in Fuwai hospital from January 2009 to June 2016 were retrospectively analyzed. The age of the patients was 4 (2, 5) months,and the body weight of the patients was (5.3±1.6) kg. The patients were followed up to observe the surgery effect.@*Results@#No perioperative death and serious complications occurred. When the patients were discharged,the systolic blood pressure of the right upper limb was lower than the preoperative systolic blood pressure ((85.7±5.9) mmHg(1 mmHg=0.133 kPa) vs. (100.7±16.6) mmHg, P<0.001),and the systolic blood pressure of the right lower limb was higher than the preoperative systolic blood pressure ((98.7±13.3) mmHg vs. (85.6±20.8) mmHg, P<0.001). The pressure gradient of aortic coarctation detected by echocardiography was lower than the preoperative pressure gradient ((13.1±3.8) mmHg vs. (46.2±17.1) mmHg, P<0.001). No restenosis was detected by echocardiography at discharge. Follow-up data were obtained in 19 patients, and the follow-up time was 18 (8, 45) months.The patients grew well, and no death occurred. Restenosis occurred in 3 cases, 1 patient underwent aortic balloon dilatation and the remaining 2 patients were under follow up observation. Computed tomography angiography showed that the morphology of aortic arch was normal without signs of aortic aneurysm.@*Conclusion@#Coarctation resection with autologous pulmonary artery patch aortoplastystrategy is considered as a safe and effective surgical method for management of infant coarctation with hypoplastic aortic arch, and this surgery method is related with satisfactory early and mid-term outcomes in this patient cohort.
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Objective@#To compare the clinical outcome of patients with pulmonary valve stenosis underwent transthoracic and percutaneous balloon pulmonary valvuloplasty.@*Methods@#Clinical data of 806 patients diagnosed as pulmonary valve stenosis underwent transthoracic(171 patients as group A)or percutaneous balloon pulmonary valvuloplasty (635 patients as Group B) in Fuwai Hospital from February 2006 to January 2016 were analyzed retrospectively. There were 72 males in group A (42.1%) and 344 males in group B (54.2%). The average age was (1.6±1.1) years in group A and (21.0±18.5) years in group B. The median weight was 7.65 (7.68) kg (M(QR)) in group A and 43.75 (47.38) kg in group B. There were 732 (90.9%) patients followed up from 3 months to 10 years, with an average interval of (6.3±3.6) years. Sixty cases were ligated patent ductus arteriosus simultaneously, and 20 cases got Blalock-Taussig shunt at the same time of valvuloplasty in group A. There were 47 cases of transcatheter closure of atrial septal defect and 6 cases of transcatheter closure of patent ductus arteriosus in group B. The t test, rank sum test and χ2 test were used to compare data of two groups.@*Results@#There were no hospital death or cardiac tamponade and other serious complications for all patients. The postoperative hospital stayin group A was significantly longer than that in group B (8(5) days vs. 2(2) days, Z=-9.404, P=0.000). In every further consultation, patients were reviewed with transthoracic echocardiography to assess transpulmonary gradient and pulmonary regurgitation. There were significant difference between group A and B of preoperative transpulmonary pressure gradient ((80.6±22.4) mmHg vs.(72.6±20.5) mmHg, t=1.611, P=0.032, 1 mmHg=0.133 kPa) and so as transpulmonary pressure gradient reduction value ((55.9±21.0) mmHg vs. (46.6±23.4) mmHg, t=-1.710, P=0.026). Patients in both groups had good cardiac function during follow-up interval. One patient needed surgical valvuloplasty 10 months after percutaneous balloon pulmonary valvuloplasty and 1 case occurred moderate to severe tricuspid regurgitation in group B. During follow-up period, there was no significant difference between group A and B of transpulmonary pressure gradient ((22.3±6.5) mmHg vs. (25.2±12.6) mmHg, t=1.320, P=0.072), the incidence of pulmonary valve regurgitation in patients of group A was significantly lower than patients of group B (56.1% vs.65.2%, χ2=4.755, P=0.029).@*Conclusions@#The clinical outcome and complications are similar between patients underwent two different routes of balloon pulmonary valvuloplasty. Transthoracic balloon pulmonary valvuloplasty is more suitable for infant and underweight children patients with pulmonary valve stenosis. Percutaneous balloon pulmonary valvuloplasty is more suitable for the treatment of the elder children or adults.
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Objective To analyze clinical characters,associated anomalies,diagnostic approach,and surgical procedures in patients with unroofed coronary sinus syndrome.Methods The clinical,echocardiographic,operative and follow-up data of 109 patients with unroofed coronary sinus syndrome from May 1999 to July 2016 were reviewed retrospectively(type Ⅰ51cases,type Ⅱ 17 cases,type Ⅲ 17 case and type Ⅳ 24 cases).The initial diagnosis of unroofed coronary sinus syndrome was made by echocardiography in 56 cases,while the remains were confirmed by the surgeon at repair of other congenital cardiac anomalies.69 of the 109 cases associated with left superior vena cava(LSVC),and 53 of the 69 directed drained into the left atrium(LA).Among the 109 cases,2 cases associated with complex anomalies underwent palliative operations,and other cardiac malformations were corrected simultaneously by surgical correction in the other cases.LSVC was ligated in 8 cases,and the intracardiac tunnel to drain LSVC to right atrium(RA) was reconstructed in 38 cases,intracardiac baffle in 9 cases,extracardiac repair in 2 cases.The associated cardiac lesions were corrected concomitantly.Results There were 5 hospital deaths.3 patients died of low cardiac output syndrome and 2 patients died of pulmonary infection.We followed up 87 early survivors,and there was no death and severe complications.Conclusion Unroofed coronary sinus syndrome is often missed in the preoperative evaluation of congenitally malformed hearts.Preoperative TTE is still the most important method in the diagnosis of UCSS.Strengthening the understanding of the pathological anatomy and physiological characteristics of the heart malformation may help us to improve the preoperative diagnosis of UCSS.When associated with LSVC,UCSS should be considered as a possible additional finding.We performed different surgical approaches to deal with the different kinds of LSVC with a good result.
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<p><b>OBJECTIVE</b>To explore the feasibility and value of a new categorization of double outlet right ventricular (DORV) on guiding the optimal choices of surgical approaches.</p><p><b>METHODS</b>Five hundred and twenty one DORV patients diagnosed by echocardiography, angiocardiography and CT at Fuwai Hospital from May 2003 to September 2014 were enrolled in this retrospective study. Congenital DORV was categorized according to three basic factors as follows: the positional relationships of great arteries (normal relation or abnormal relation), the relationships of the ventricular septal defect (VSD) to the great arteries (committed VSD or remote VSD), the presence or absence of pulmonary outflow tract obstruction (POTO). Eight types of DORV were established: type I (normal relation, committed VSD, without POTO), type II (normal relation, committed VSD, POTO), type III (normal relation, remote VSD, without POTO), type IV (normal relation, remote VSD, POTO), type V (abnormal relation, committed VSD, without POTO), type VI (abnormal relation, committed VSD, POTO), type VII (abnormal relation, remote VSD, without POTO), type VIII (abnormal relation, remote VSD, POTO). Feasibility of this classification and the value of this classification on guiding the choice of surgical approaches were analyzed.</p><p><b>RESULTS</b>Among the five hundred and twenty one patients, there were 90 patients (17.3%) with type I DORV, 94 patients (18.0%) with type II, 33 patients (6.3%) with type III, 34 patients (6.5%) with type IV, 64 patients (12.3%) with type V, 61 patients (11.7%) with type VI, 33 patients (6.3%) with type VII, 112 patients (21.5%) with type VIII. Thus, all patients could be typed by this classification method. The echocardiography diagnosis was consistent with the intra-operative and or cardiac catheterization/CT findings. Excluding the contraindications of bi-ventricular repair, different surgical approaches were performed in every subtype of DORV according the classification, which indicated that this novel categorization could accurately guide the clinic managements.</p><p><b>CONCLUSION</b>This novel DORV categorization can accurately diagnose DORV lesions, and guide the clinic therapy choice.</p>
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Humains , Ventricule droit à double issue , Échocardiographie , Communications interventriculaires , Ventricules cardiaques , Études rétrospectivesRÉSUMÉ
Objective: To analyze the 10-year experience for placement of permanent epicardial-pacemaker (PM) during peri-operative period in a single center of patients with congenital heart diseases (CHD). Methods: A total of 33 CHD patients who received the placement of epicardial-PM during peri-operative period in our hospital from 2002 to 2013 were retrospectively analyzed. There were 6 patients with congenital atrio-ventricular block (AVB) 27 with iatrogenic AVB. All patients were younger than 8 years and the mean age was (23.2 ± 26.9) months, with the body weight at (9.7 ± 5.6) Kg. 6 patients with congenital AVB received surgical PM placement combined with CHD repair, and the other 27 patients received PM placement at (26 ± 13.1) days after the surgery. Steroid-eluting bipolar epicardial pacing leads were inserted through median sternotomy and connected to various pulse generators within the subrectus pocket. The time, type, acute ventricular stimulation sensing, impedance and electrophysiological information of PM were collected during the operation. The patients were followed-up for (46.8 ± 33.9) months for echocardiography, ECG, programming information of PM, and the major adverse cardiac events (MACE) were recorded. Results: There were 2 congenital AVB patients received dual chamber PM and the rest patients received single chamber PM. Acute ventricular stimulation sensing was (1.34 ± 0.72) V, no signiifcant increase was identiifed in the last follow-up examination as (1.37 ± 0.81) V,P=0.93. Compared with immediate PM implantation, no signiifcant increases were observed for impedance and R wave in the last follow-up examination as (366.7 ± 88) Ω vs (331.9 ± 95.9) Ω,P=0.32 and (12.3 ± 3.5) mV vs (11.4 ± 4.9) mV,P=0.635 respectively. There were 4 patients received PM replacement because of generator dysfunction, 7/33 (21.2%) of patients had MACE as heart failure or sudden death. The age and body weight in MACE patients were similar with the patients with good prognosis,P>0.05. No pocket infection or lead fracture occurred. Conclusion: Iatrogenic high level of AVB has been the primary reason for surgical placement of epicardial PM in CHD patients during peri-operative period. It has better long term outcome, while the type of PM should be optimized.
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<p><b>OBJECTIVE</b>We followed up all the patients underwent pulmonary artery banding (PAB) with or without the second stage double switch (DS) operation to compare the midterm outcomes between the DS and the prolonged palliative PAB.</p><p><b>METHODS</b>From January 2005 to June 2012, 40 consecutive patients diagnosed with congenital corrected transposition of the great arteries (cc-TGA) with morphologically involuted left ventricle were included in this retrospective cohort study. Every patient underwent the first stage PAB. The second stage DS was performed in 15 patients (DS group) and the palliative PAB was prolonged in the other 25 patients (PAB group). The median age at PAB was 3.1 years (range, 3 months to 8 years), weight was 11.5 kg (range, 5.0 kg to 21.5 kg). The patients were followed up after surgery.</p><p><b>RESULTS</b>Follow-up time was (3.4 ± 0.7) years. Overall mortality was 33.3% (5/15) in DS group and 4.0% (1/25) in PAB group (P < 0.05). The ratio of NYHAI-II was 80.0% (8/10) in DS group and 95.9% (23/24) in PAB group (P < 0.05) and the mean left ventricle ejection fraction was (51.4 ± 9.6)% in DS group and (61.0 ± 6.4)% in PAB group(P < 0.01) during follow up. There was no PAB related cyanosis or ventricular dysfunction and the trans-banding pressure gradient was (46.9 ± 21.5)mmHg (1 mmHg = 0.133 kPa) in PAB group. Echocardiography examination revealed moderate to large aortic regurgitation in 3 patients and severe post-double switch operative arrhythmias was found in 2 patients in DS group. The age at PAB (HR = 13.30, 95%CI:6.28-14.02, P < 0.05) and left ventricle dysfunction (HR = 4.77, 95%CI:2.71-8.68, P < 0.05) were identified as the risk factors for mortality after DS.</p><p><b>CONCLUSION</b>Compared to the DS, the prolonged palliative PAB procedure is associated with lower mortality and better cardiac function post operation.</p>