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.
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.
Pulmonary atresia (PA) with ventricluar septal defect(VSD) is a complex congenital heart disease. The lack of knowledge on embryology, anatomy and pathophysiology of this disease with or without heterogenetic major aortopulmonary collateral arteries(MAPCA)leads to chaos of its surgical classification and management as well as poor results of surgical management. The operative technique in this field has been advancing a lot since the innovation of unifocalization. The surgical result is getting better and better, boosted by the updating of the principle and knowledge, but there is still a large room for progress. We reviewed the literatures on classification,surgical strategy, procedures and operationrelated issues and put them together here to delineate its past, present and future.
Objective To investigate the surgical treatment methods and effects for pulmonary atresia with ventricular septal defect (PAVSD) in elder children and adults in order to promote the treatment effects. Methods From October 1996 to October 2008, we performed stage1 or staged biventricular repair on 39 PAVSD patients including 21 males and 18 females, ranging from 8 to 27 years old with an average age of 13.43 years. There were 14 cases of type A, 11 cases of type B, and 14 cases of type C. Among them, 23 patients underwent stage1 radical repair in which either human blood vessel with valves or bovine jugular vein with valves were used to connect the pulmonary artery and the right ventricular outflow tract. In these 23 patients, 3 patients complicated with major aortopulmonary collaterals(MAPCAs) underwent unifocalization (UF) operation. The other 16 patients received staged repair, including 9 cases of systemic to pulmonary artery shunt and 7 of staged radical cure. Results There were 6 perioperative deaths with a total mortality of 15.38%(6/39), including 4 (17.39%) stage1 radical repair cases and 2 (12.50%) staged radical repair cases. The former 4 were all type C patients, dying from low cardiac output due to increased pulmonary arterial pressure. In the latter 2 deaths, 1 was a type B secondary shunt patient, and the other was a type C staged radical repair case, both of whom died of bleeding caused by aortic injury in the succeeding operations. Followup was done on 28 cases with a followup rate of 84.85%. The followup time ranged from 14.0 months to 9.2 years with 5 cases missing. No patient died during the followup, and 9 patients maintained their cardiac function at class Ⅰ, 13 at class Ⅱ, 5 at class Ⅲ and 1 at class Ⅳ. Three patients had aortic valve regurgitation of small to medium volume, the treatment of which included an administration of oral potassium diuretic medication and regular follow-up. Conclusion Pulmonary vessels of elder children and adults with PAVSD are usually injured severely and oftentimes it is complicated with MAPCAs. Standard for stage1 radical repair should be defined more strictly based on the present one.
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.
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.
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.
ObjectiveTo identify the pulmonary artery growth, restenosis and regurgitation of the valve after right ventricle outflow (RVOT) reconstruction with pericardial tube in patients with pulmonary atresia and ventricle septal defect (PA/VSD). MethodsWe retrospectively analyzed the clinical data of 41 patients with PA/VSD undergoing PA/VSD repair to reconstruct RVOT for radical or palliative repair in our hospital from November 2002 through September 2013. There were 25 males and 16 females with operation age of 4.00 months to 22.70 years (56.60±63.92 months). Late pulmonary artery growth, pulmonary artery, and tricuspid regurgitation of the patients were followed up. Pulmonary atresia and ventricular septal defect repair with pericardial tube were performed in the patients. ResultsThere were 5 (12.19%) early hospital deaths. Thirty-three patients were followed up for 4.00 months to 10.75 years (3.00±2.35 years). Three patients (7.31%) were lost during the follow-up.One patient was dead after stageⅡsurgery. There was no significant growth on the diameters of the tube and the pulmonary artery branches during the follow-up. There were 10 patients with severe stenosis in pericardial tube and 5 patients with moderate or severe stenosis in pulmonary artery branches. The echocardiography suggested the pulmonary artery and tricuspid regurgitation were more serious (P<0.05). No correlation was found between regurgitation quantity and follow-up time. ConclusionThe early postoperative results is satisfactory. However, there is no potential growth on the pericardial tube. So the patients should be followed up closely for restenosis.
Objective To investigate and compare the different surgical strategies for typeⅠpulmonary atresia with ventricular septum defect (PA/VSD) and the outcomes of postoperative prognosis in early stage. Methods We retrospectively analyzed the clinical data of 61 typeⅠPA/VSD patients (40 males, 21 females) with a median age of 249 days (range, 13 days-19 years) in Guangdong Cardiovascular Institute from January 2005 to December 2014 . Among them, 42 patients (27 males, 15 females) with a median age of 11.11 months, ranging from 0.80–211.70 months received radical surgery as a radical surgery group. And 19 patients (13 males, 6 females) with a median age of 2.96 months, ranging from 0.47–161.83 months underwent palliative surgery as a palliative surgery group. We compared the two surgeries and their early outcomes. Results The mean postoperative oxygen saturation was 88.08%±9.64%, which showed significant improvement compared with preoperative oxygen saturation of 74.08%±12.99% (P＜0.05). Patients in the palliative surgery group had a lower body temperature during cardiopulmonary bypass and more respiratory complications than those in the radical surgery group (24.69 °C±3.11 °C vs. 27.18 °C±2.10 °C). Conclusion Both radical and palliative surgeries are good for the increase of pulmonary blood volume and the development of pulmonary vessels. Surgeons must pay more attention to choosing radical surgery for the babies, which is only considerd for those with well developed pulmonary arteries.
ObjectiveTo evaluate the effect of off-pump pulmonary valvulotomy for the patients with pulmonary atresia with intact ventricular septum (PA/IVS).MethodsThe clinical data of 61 PA/IVS patients who underwent off-pump right ventricular decompression surgery in our hospital from January 2013 to September 2019 were retrospectively analyzed, including 37 males and 24 females, with an average age of 29.7 (2.0-86.0) d and weight of 4.1 (2.5-6.9) kg. Thirty-nine patients received off-pump pulmonary valvulotomy (an open-view valvulotomy group) and 22 patients received balloon valvuloplasty through the right ventricle pulmonary valve (a hybrid therapy group). The postoperative mortality, early re-intervention, and completion of final operation of the two groups were compared.ResultsThere were 2 deaths in the study with a mortality rate of 3.3% (2/61), and the mortality rate of the two groups was not significantly different (2.6% vs.4.5%, P=0.68). The rate of early re-intervention in the two groups was 5.3% and 19.0%, respectively (P=0.09). There was no statistical difference in intubation time (56.0±25.9 h vs. 62.0±28.9 h, P=0.41), ICU retention time (4.7±2.9 d vs. 5.5±2.2 d, P=0.23) and postoperative hospital stay time (3.9±0.9 d vs. 4.3±1.1 d, P=0.38) between the two groups. The follow-up time was 45.3 (4.0-84.0) months. There were 5 patients lost to follow-up. During the follow-up period, in the open-view valvulotomy group, 17 patients did not need further operation, 13 patients completed the final operation. In the hybrid therapy group, 7 patients did not need further operation, 8 patients completed the final operation. Heart function classification of all patients was in New York Heart Association class Ⅰ-Ⅱ.ConclusionCompared with the hybrid therapy, off-pump pulmonary valvulotomy for PA/IVS also has the advantages of simple operation, short operation time and high survival rate, and it may be easier to be promoted in clinical application because of its more economic benefits and relatively lower re-intervention rate.