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Comparison of Early Clinical Outcomes between Systemic-Pulmonary Shunts and Right Ventricular to Pulmonary Artery Connection for Patients with Pulmonary Atresia and Ventricular Septal Defect

ObjectiveTo compare early clinical outcomes between systemic-pulmonary shunts (SPS) and right ventricular to pulmonary artery connection (RV-PA connection) for patients with pulmonary atresia and ventricular septal defect (PA/VSD), and investigate early management strategies for these 2 different palliative procedures. MethodsWe retrospectively analyzed clinical data of 89 PA/VSD patients who underwent SPS or RV-PA connection in Fu Wai Hospital from January 2009 to December 2011. According to different surgical procedures, all the 89 patients were divided into 2 groups. In SPS group, there were 59 patients including 35 males and 24 females with their median age of 25 months (4 months to 8 years). In RV-PA connection group, there were 30 patients including 19 males and 11 females with their median age of 24 months (28 days to 7 years and 2 months). Early clinical outcomes including mechanical ventilation time, length of ICU stay, morbidity, reexploration, improvement of oxygen saturation (SO2) and mortality were compared between the 2 groups. ResultsAmong 59 patients in SPS group, 3 patients (5.1%) died postoperatively. There was no in-hospital death among 30 patients in RV-PA connection group. The improvement of percentage of SO2 of RV-PA connection group was significantly higher than that of SPS group (31.7% vs. 22.2%, P < 0.05). There was no statistical difference in length of ICU stay (3.6±2.5 days vs. 4.2±5.1 days, P > 0.05), mechanical ventilation time (34.8±33.5 hours vs. 44.3±39.6 hours, P > 0.05), postoperative morbidity (37.3% vs. 30.0%, P > 0.05) or reexploration rate (15.3% vs. 6.7%, P > 0.05) between SPS group and RV-PA connection group. Incidence of serious postoperative complications of SPS group was signi-ficantly higher than that of RV-PA connection group (25.4% vs. 6.7%, P < 0.05). ConclusionEarly clinical outcomes of RV-PA connection is better than SPS for PA/VSD patients including greater SO2 improvement and lower mortality. Mid-term and long-term clinical results as well as larger study samples are needed for better evaluation.

新生儿室间隔完整型肺动脉闭锁 Hybrid 手术预后分析

目的 探讨室间隔完整型肺动脉闭锁（pulmonary atresia with intact ventricular septum，PA/IVS）新生儿行 Hybrid 手术的预后。 方法 回顾性分析 2015 年 7 月至 2017 年 11 月我院行 Hybrid 手术（食管超声引导下经胸切口右室流出道途径球囊扩张肺动脉瓣联合 BT 分流术） 9 例 PA/IVS 新生儿的临床资料，其中男 6 例、女 3例，手术年龄23（17～29）d。 结果 9 例新生儿手术顺利，手术时间 112（95～147）min。1 例死于肺部严重感染，余 8 例均顺利出院。8 例存活患儿机械通气时间 31（22～47）h，住院时间 17（12～23）d，出院时 8 例平均血氧饱和度升至 87.5%，平均右室收缩压降至 49 mm Hg，肺动脉瓣跨瓣压降至 28（21～46）mm Hg。1 例术后发生短暂的室上性心动过速，5 例患儿因为术中失血给予了输血，3 例患儿出现肺动脉瓣少量反流，1 例患儿出现肺动脉瓣中量反流，无 1 例患儿发生心脏穿孔、主肺动脉瘤或低心排血量综合征。随访时间为 17.5（7～26）个月。5 例患儿已完成根治手术，3 例患儿等待行根治手术。 结论 相对传统外科手术和经皮介入术，Hybrid 手术治疗可作为一种安全有效的替代治疗手段，对于条件合适的 PA/IVS 新生儿可获得满意的预后。

The Application of Palliative Operation in Staged Surgical Management of Pulmonary Atresia with Ventricular Septal Defect

Abstract： Objective To assess the effects of three different palliative procedures including modified BlalockTaussig (B-T) shunt, Waterston shunt, and reconstruction of right ventricularpulmonary artery (RV-PA) continuity for pulmonary atresia with ventricular septal defect (PAVSD). Methods We retrospectively analyzed the clinical data of 93 patients with PAVSD who had undergone palliative surgical procedures including modifie BT shunt, Waterston shunt, and RVPA econstruction in Fu Wai Hospital from September 1998 to September 2008. There were 53 males and 40 females, aged from 14.0 days to 14.4 years, with the body weight ranged from 3.6 to 33.0 kg (9.9±6.3 kg). According to International Congenital Heart Surgery Nomenclature and Database Project, these patients were categorized into 2 groups: 64 of type Ⅰ and 29 of type Ⅱ. The most common associated anomaly is rightsided aortic arch (except for ventricular septal defect). The application of the three kinds of palliative surgical procedures in staged management of PAVSD and the followup results were statistically analyzed. Results The corrective rate of the three palliative procedures were 28.12% (18/64) for modified BT shunt, 7.14%(1/14) for Waterston shunt, and 66.67% (10/15) for RV-PA reconstruction, respectively. RV-PA reconstruction had a significantly higher corrective 〖CM(1585mm〗rate than the other two surgical procedures (P=0.016). The percutaneous oxygen saturation (SpO2) increased by 4%59% and Nakata index by 31-104 mm2/m2. No tortuous pulmonary artery was found under echocardiogram or angiocardiography after palliative operation. The perioperative mortality of both surgical stages was 10 patients. Twostage radical surgery was successfully performed for 25 patients, among whom 20 were followed up till May 2009. During the followup, one died suddenly, 15 were classified as New York Heart Association (NYHA) Ⅰ, and 4 as NYHA Ⅱ. Conclusion The surgical management of PAVSD needs to be improved continuously. Compared with shunting procedures, the RVPA reconstruction is a better palliative operation method, and the modified B-T shunt is preferred in younger patients.

Surgical Treatment of Tetralogy of Fallot: A Report of 164 Cases

Objective To sum up the therapeutic results of corrective surgery of 164 cases of tetralogy of Fallot (TOF), and explore the optimal time and risk factors of operation,as well as perioperative management. Methods One hundred and sixty-four consecutive cases of TOF underwent corrective surgery. There were simple stenosis of infundibular portion in right ventricular outflow tract in 37 cases, stenosis of infundibulum and pulmonary valve in 14 cases, main pulmonary trunk and left/right pulmonary arteries stenosis in 113 cases, and pulmonary atresia in 5 cases. Autologous pericardial conduit, valved homograft were used for right ventriculo-pulmonary artery connection, respectively. Other anomalies were corrected. Results The surgical mortality was 3.66% (6/164). The cause of death were serious low cardiac output syndrome(2 case), fail to wean from cardiopulmonary bypass after coronary artery bypass grafting (1 case), ventricular arrhythmia(1 case) and postoperative acute respiratory distress syndrome (2 cases). Conclusion It’s necessary to perform corrective operation on younger TOF patients. Low cardiac output syndrome is not the key reason of leading to postoperative complications or death. Preventing remnant obstruction of pulmonary artery and pulmonary complication should be focused during and after operation.

Outcomes of complicated congenital heart diseases following multiple re-sternotomy operations: A single-center experience

ObjectiveTo analyze the outcomes of complicated congenital heart diseases (CCHD) patients accepting multiple (>2) re-sternotomy operations.MethodsWe retrospectively analyzed the clinical data of 146 patients undergoing multiple cardiac re-sternotomy operations between 2015 and 2019 in our center. There were 95 males and 51 females with an age of 4.3 (3.1-6.8) years and a weight of 15.3 (13.4-19.0) kg at last operation.ResultsThe top three cardiac malformations were pulmonary atresia (n=51, 34.9%), double outflow of right ventricle (n=36, 24.7%) and functional single ventricle (n=36, 24.7%). A total of 457 sternotomy procedures were performed, with 129 (88.3%) patients undergoing three times of operations and 17 (11.7%) patients undergoing more than three times. Fifty-two (35.6%) patients received bi-ventricular repair, 63 (43.1%) patients received Fontan-type procedures, and 31 (21.2%) patients underwent palliative procedures. Ten (6.8%) patients experienced major accidents during sternotomy, including 7 (4.8%) patients of urgent femoral artery and venous bypass. Eleven (7.5%) patients died with 10 (6.8%) deaths before discharge. The follow-up time was 20.0 (5.8-40.1) months, and 1 patient died during the follow-up. The number of operations was an independent risk factor for the death after operation.ConclusionSeries operations of Fontan in functional single ventricle, repeated stenosis of pulmonary artery or conduit of right ventricular outflow tract post bi-ventricular repair are the major causes for the reoperation. Multiple operations are a huge challenge for CCHD treatment, which should be avoided.

Long-term Outcomes of Surgical Repair for Pulmonary Atresia with Ventricular Septal Defect and Major　Aortopulmonary Collateral Arteries

Objective　To evaluate long-term outcomes of surgical repair for pulmonary atresia with ventricular septal defect （PA-VSD） and major aortopulmonary collateral arteries （MAPCAs）.?Methods?We retrospectively analyzed the clinical data of 29 patients with PA-VSD and MAPCAs who underwent surgical repair in Shanghai Children’s Medical　Center from February 2001 to February 2010. There were 13 patients in the one-stage operation group and 16 patients in the staged operation group. There were 8 males and 5 females in the one-stage operation group with their age ranging from 8 to 168 （26.0±17.0）months. There were 9 males and 7 females in the staged operation group with their age ranging from 4 to 149 （26.5±15.8）months. Eight patients underwent their second operation with the time interval between the two operations ranging from 14 to 40 （28.3±11.7） months. For MAPCAs， 9 patients underwent unifocalization， 16 patients underwent ligation or occlusion via cardiac catheterization， and the other 4 patients left them open or unrepaired. We compared postoperative mortality， complications， long-term outcomes， and quality of life between the two groups. Quality of life of the 29 PA-VSD patients was measured according to Pediatric Quality of Life Inventory version 4.0 and compared with 100 children from a kindergarten as a control group.?Results?Postoperative mortality of one-stage operation group was significantly higher than that of staged operation group at 15.4% （2/13） vs. 0.0% with χ2=4.12 and P=0.04. There was no statistical difference in the rate of postoperative complications between the two groups at 75.0% （9/12） vs. 56.2% （9/16） with χ2=0.51 and P=0.47. Patients were followed up for 3 to 88 months， and the follow-up mortality of one-stage operation group was not significantly higher than that of staged operation group at 27.3% （3/11） vs. 6.2% （1/16） with χ2= 2.28 and P=0.13. There was no statistical difference in overall quality of life score between one-stage operation group and staged operation group （66.7±8.6 vs 70.2±13.0， t?=?-0.71， P=0.48） . The overall quality of life score of PA-VSD patients was significantly lower than that of control group （68.7±11.2 vs. 77.8±15.7， t?= 3.14， P=0.01）， mainly because the physical functioning score of PA-VSD patients was significantly lower than that of control group （57.7±11.7 vs. 83.0±19.6， t?=5.67， P=0.00）， but there was no statistical difference in emotional well-being　score， social functioning score， and school functioning score between PA-VSD patients and the control group.?Conclusion　It is the key to analyze different pulmonary artery conformation， choose individualized surgical procedure and properly manage MAPCAs so as to reduce postoperative mortality and complications of surgical repair for patients with PA-VSD and MAPCAs.

先天性心脏病外科治疗中国专家共识（三）：肺动脉闭锁合并室间隔缺损

Change of Blood Gas and Hemodynamic Status after Palliative Procedurein the Patients with Pulmonary Atresia and Ventricular Septal Defect

Objective Comparing postoperative change of blood gas and hemodynamic status in patients underwent a right ventricletopulmonary artery (RVPA) conduit or a modified BlalockTaussig (mBT) shunt for pulmonary atresia with ventricular septal defect and without major arterial pulmonary collaterals (MAPCAs), to affirm the effect on oxygen supply /demand with different procedure. Methods From July 2006 to October 2007, 38 patients with pulmonary atresia and ventricular septal defect without MAPCAs were divided into two groups according to different procedures: RVPA group (n=25) and mBT group (n=13).Perioperative mortality, blood gas and hemodynamic data during postoperative 48 hours, including heart rate, blood pressure, systemic oxygen saturation, mixed venous oxygen saturation, oxygen excess factor, inotropic score were compared in both groups. Results The difference in the mortality between RVPA group (4.0％，1/25) and mBT group （7.7％，1/13) showed no statistical significance（Pgt;0.05）. The total of 33 patients were followed up, the followup time was from 6 to 18 months.11 patients (4 patients in mBT group, 7 patients in RVPA group) underwent corrected procedures during 9 to 18 months after palliative procedures, one case died of elevated pulmonary vascular resistance and right ventricle failure. The mixed venous oxygen saturation at 24h and 48h after surgery were higher than that at 6h after surgery (Plt;0.01) both in RVPA group and mBT group. The systolic blood pressures at 6h, 24h, 48h after surgery in RVPA group were lower than those in mBT group (P=0.048，0.043， 0.045)，the mean systemic blood pressures in RVPA group were higher than those in mBT group (P=0.048, 0.046, 0.049),the diastolic blood pressures in RVPA group were higher than those in mBT group (P=0.038, 0.034, 0.040)， the inotropic scores in RVPA group were lower than those in mBT group (P=0.035， 0.032，0.047). Conclusion The blood pressures and inotropic scores are found significantly different in RVPA conduit and mBT procedures, while postoperative systemic oxygen delivery areequivalent. Both RVPA and mBT patients decline to nadir in hemodynamic status at 6 h after surgery.

Right Ventricle-to-Pulmonary Artery Shunt as the First Stage Palliative Operation for Patients with Pulmonary　Atresia with Ventricular Septal Defect

Objective To summarize clinical outcomes of right ventricle-to-pulmonary artery shunt （Sano shunt）as the first stage palliative operation for patients with pulmonary atresia with ventricular septal defect （PA/VSD）. Methods　Between September 2009 and May 2011，17 PA/VSD patients underwent Sano shunt in Fu Wai Hospital. There were 10 male patients and 7 female patients with their median age of 9.7 （2.5-73.8） months and average weight of （8.3±3.4）kg. Preoperative McGoon ratio was 1.04±0.29 and Nakata index was （102.0±56.9） mm2/m2. Five children had severe intrapericardial left pulmonary aretery stenosis，11 patients had patent ductus arteriosus （PDA），and 1 patient had major aorto-pulmonary collateral arteries. Preoperative transcutaneous oxygen saturation （SpO2） was 72.6%±11.6%. Results　All the patients received Sano shunt placement successfully. Eleven patients underwent concomitant PDA ligation，and 7 patients underwent concomitant left pulmonary artery plasty. The Sano shunts were constructed with glutaraldehyde-fixed autologous pericardium in 10 patients，fresh autologous pericardium in 1 patient and Gore-Tex graft in 6 patients. All the patients underwent reconstruction of the right ventricular outflow tract via the right ventricular incision. The average cardiopulmonary bypass time was （75.0±30.0） min. Postoperative SpO2 was 89.8%±5.3% and significantly higher than preoperative SpO2 （P＜0.05）. All the patients were discharged alive and followed up for （12.1±6.7） months. During follow-up，8 patients underwent angiography study whose McGoon ratio and Nakata index improved to 2.05±0.37 and （304.8±51.3） mm2/m2 respectively，both of which were significantly higher than preoperative values（P＜0.05）. Four patients successful　underwent second stage total correction. Conclusion Sano shunt is a comparatively safe procedure as the first stage　palliative operation for PA/VSD patients，and can significant improve their pulmonary artery growth.

Strategy of surgical management for pulmonary atresia with intact ventricular septum and mid-term follow-up

ObjectiveTo evaluate the surgical strategy and follow-up for pulmonary atresia with intact ventricular septum (PA/IVS) in our heart center.MethodsFrom January 2008 to December 2018, 151 patients with PA/IVS were divided into two groups: a one-stage surgery group (26 patients), including 17 males and 9 females at an average age of 14.7±13.2 months, and a staged surgery group (125 patients) including 72 males and 53 females at an average age of 6.4±6.3 months. The clinical effectiveness of the two groups were analyzed.ResultsAll patients were followed up for 1-11 years. Eighteen patients died and 19 patients were lost to follow-up. The 1-year, 5-year and 10-year survival rate was 90.2%, 87.0%, and 85.2%, respectively. Two patients died in the one-stage surgery group. Twelve patients died after initial surgery, and 4 patients died after final operation in the staged surgery group. The Z value of tricuspid valve (P=0.013) and severe right ventricular dysplasia (P=0.025) were the risk factors of postoperative death in the patients with PA/IVS. Furthermore, 58 patients completed final operation, and the total number of the final operation (including one-stage radical surgery) accounted for 55.6% (84/151). Five patients accepted the re-operation intervention in the medium-term follow-up. The rest of the patients recovered well. Only 2 patients were classified as grade Ⅲ in cardiac function, and the rest patients were classified as gradeⅠ-Ⅱ.ConclusionAccording to the degree of right ventricular hypoplasia, the age at operation and the presence or absence of coronary artery malformation, the individualized surgical strategy could significantly improve the success rate of PA/IVS, and early completion of right ventricular decompression operation is conducive to improve the chance for biventricular repair.