ObjectiveTo summarize the experience and lessons of right ventricular decompression in children with pulmonary atresia and intact ventricular septum (PA/IVS) and to reflect on the strategies of right ventricular decompression.MethodsThe clinical data of 12 children with PA/IVS who underwent right ventricular decompression in our hospital from March 2015 to December 2019 were reviewed retrospectively. There were 10 males and 2 females with a median age at the time of surgery was 5 d (range, 1-627 d). Correlation analysis between the pulmonary valve transvalvular pressure gradient and changes in Z score of tricuspid valves after decompression was performed.ResultsOne patient died of refractory hypoxemia due to circulatory shunt postoperatively and family members gave up treatment. There were 2 (16.67%) patients received postoperative intervention. The pulmonary transvalvular gradient after decompression was 31.95±21.75 mm Hg. Mild pulmonary regurgitation was found in 7 patients, moderate in 2 patients, and massive in 1 patient. The median time of mechanical ventilation was 30.50 h (range, 6.00-270.50 h), and the average duration of ICU stay was 164.06±87.74 h. The average postoperative follow-up time was 354.82±331.37 d. At the last follow-up, the average Z score of tricuspid valves was 1.32±0.71, the median pressure gradient between right ventricle and main pulmonary artery was 41.75 mm Hg (range, 21-146 mm Hg) and the average percutaneous oxygen saturation was 92.78%±3.73%. Two children underwent percutaneous balloon pulmonary valvoplasty at 6 and 10 months after surgery, respectively, with the rate of reintervention-free of 81.8%. There was no significant correlation between pulmonary transvalvular gradients after decompression and changes in Z score of tricuspid valves (r=–0.506, P=0.201).ConclusionFor children with PA/IVS, the simple pursuit of adequate decompression during right ventricular decompression may lead to severe pulmonary dysfunction, increase the risk of ineffective circular shunt, and induce refractory hypoxemia. The staged decompression can ensure the safety and effectiveness for initial surgery and reduce the risk of postoperative death.
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.
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 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.
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.
ObjectiveTo compare the benefits and drawbacks of primary patch expansion versus pericardial tube right ventricular-pulmonary artery connection in patients diagnosed with pulmonary atresia with ventricular septal defect (PA/VSD). MethodsA retrospective study was conducted on patients diagnosed with PA/VSD who underwent primary right ventricular-pulmonary artery connection surgery at our center between 2010 and 2020. Patients were categorized into two groups based on the type of right ventricular-pulmonary artery connection: a pericardial tube group and a patch expansion group. Clinical data and imaging findings were compared between the two groups. ResultsA total of 51 patients were included in the study, comprising 31 males and 20 females, with a median age of 12.57 (4.57, 49.67) months. The pericardial tube group included 19 patients with a median age of 17.17 (7.33, 49.67) months, while the patch expansion group consisted of 32 patients with a median age of 8.58 (3.57, 52.72) months. In both groups, the diameter of pulmonary artery, McGoon index, and Nakata index significantly increased after treatment (P<0.001). However, the pericardial tube group exhibited a longer extracorporeal circulation time (P<0.001). The reoperation rate was notably high, with 74.51% of patients requiring further surgical intervention, including 26 (81.25%) patients in the patch expansion group and 12 (63.16%) patients in the pericardial tube group. No statistical differences were observed in long-term cure rates or mortality between the two groups (P＞0.005). Conclusion In patients with PA/VSD, both patch expansion and pericardial tube right ventricular-pulmonary artery connection serve as effective initial palliative treatment strategies that promote pulmonary vessel development and provide a favorable foundation for subsequent radical operations. However, compared to the pericardial tube approach, the patch expansion technique is simpler to perform and preserves some intrinsic potential for pulmonary artery development, making it the preferred procedure.
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 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.
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 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.