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.
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.
ObjectiveTo analyze the mid-long-term outcomes of surgical balloon valvuloplasty (SBV) for right ventricular decompression in the treatment of pulmonary atresia with intact ventricular septum (PA/IVS).MethodsClinical data of consecutive 91 patients who were diagnosed with PA/IVS and underwent SBV in our institution from January 2005 to December 2017 were retrospectively analyzed, including 52 (57.1%) males and 39 (42.9%) females. The median age was 3 months (1 d, 24 months) and the median weight was 4.1 (2.5, 12.0) kg.ResultsThe SBV was performed in all patients, and 62 of whom received other simultaneous surgeries, including ligation of patent ductus arteriosus (PDA, 33 patients), ligation of PDA with modified Blalock-Taussig shunt (23 patients), ligation of PDA with bidirectional Glenn shunt (6 patients). There was no early postoperative death. The median follow-up time was 8.8 (2.5, 13.4) years, 4 patients were lost. There were 7 (8.0%) deaths and 1 (1.1%) patient with a re-SBV for pulmonary stenosis. The one and a half ventricular repair was performed in 5 (5.7%) patients and Fontan procedure in 2 (2.3%) patients. In addition, the mean Z-value of tricuspid valve annulus was −1.7±1.5, which was significant bigger than that before the operation (t=5.587, P<0.001).ConclusionSBV via right ventricular outflow tract for right ventricular decompression in the treatment of PA/IVS is safe and reliable. The majority of patients can receive biventricular repair instead of single ventricular palliation by SBV with individually customized shunt.
Objective Comparing postoperative change of blood gas and hemodynamic status in patients underwent a right ventricletopulmonary artery (RVPA) conduit or a modified BlalockTaussig (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: RVPA group (n=25) and mBT group (n=13).Perioperative mortality, blood gas and hemodynamic data during postoperative 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 RVPA 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 followup time was from 6 to 18 months.11 patients (4 patients in mBT group, 7 patients in RVPA 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 RVPA group and mBT group. The systolic blood pressures at 6h, 24h, 48h after surgery in RVPA group were lower than those in mBT group (P=0.048，0.043， 0.045)，the mean systemic blood pressures in RVPA group were higher than those in mBT group (P=0.048, 0.046, 0.049),the diastolic blood pressures in RVPA group were higher than those in mBT group (P=0.038, 0.034, 0.040)， the inotropic scores in RVPA 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 RVPA conduit and mBT procedures, while postoperative systemic oxygen delivery areequivalent. Both RVPA and mBT patients decline to nadir in hemodynamic status at 6 h after surgery.
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.
Objective To explore the feasibility and option of different surgeries for neonates with pulmonary atresia and ventricular septal defect (PA/VSD) through assessing the effect of common surgeries. Methods Fourteen neonates who underwent their first surgery in our center from July 2004 to October 2014 were included. Their basic characteristics, operation and pre- and postoperative clinical information were extracted. Follow up was conducted and the last visit was on October 10, 2016. Short- and midterm survival and total correction rate were compared among different surgeries. Results Among the 14 patients, there were 4 (28.6%) patients, 6 (42.9%) and 4 (28.6%) who underwent one-stage repair, right ventricular outflow tract (RVOT) reconstruction, and systemic to PA shunt operation respectively. The overall in-hospital mortality after the first operation was 28.6% (4/14). At last visit, no death occurred resulting the 5-year survival rate of 71.4% (10/14). The overall total correction rate for all neonates was 64.3% (9/14). Although no statistical difference was found in the mortality among the one-stage repair , RVOT reconstruction and systemic to PA shunt group(50.0% vs. 33.3% vs. 0.0%, P=0.280), the survival and hazard analysis implied better outcomes of the systemic to PA shunt palliation operation. There was no statistical difference in the total correction rate and months from the first palliative operation to correction between those who underwent RVOT reconstruction and systemic to PA shunt (75.0% vs. 50.0%, P=0.470; 32.0 months vs. 18.0 months, P=0.400). Conclusion Performing surgeries for neonates with PA/VSD is still a great challenge. However, the midterm survival rate was optimistic for the early survivors. Systematic to PA shunt seemed to be a better choice with lower mortality for the neonates with PA/VSD who need the surgery to survive.
Abstract： Objective To assess the effects of three different palliative procedures including modified BlalockTaussig (B-T) shunt, Waterston shunt, and reconstruction of right ventricularpulmonary 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 BT shunt, Waterston shunt, and RVPA 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 rightsided aortic arch (except for ventricular septal defect). The application of the three kinds of palliative surgical procedures in staged management of PAVSD and the followup results were statistically analyzed. Results The corrective rate of the three palliative procedures were 28.12% (18/64) for modified BT 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(1585mm〗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 mm2/m2. No tortuous pulmonary artery was found under echocardiogram or angiocardiography after palliative operation. The perioperative mortality of both surgical stages was 10 patients. Twostage radical surgery was successfully performed for 25 patients, among whom 20 were followed up till May 2009. During the followup, 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 RVPA reconstruction is a better palliative operation method, and the modified B-T shunt is preferred in younger patients.
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.