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.
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.
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 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.
ObjectiveTo evaluate the possibility of monitoring regional tissue oxygen saturation by near-infrared spectroscopy (NIRS) for early predicting adverse events in patients with pulmonary atresia.MethodsTwenty-six patients aged under 3 months who were diagnosed with pulmonary atresia and admitted to cardiovascular intensive care unit in our hospital between January 2016 and May 2017, accepted regional tissue oxygenation (cerebral and splanchnic) by near-infrared spectroscopy. There were 19 males and 7 females at age of 2–89 days. A total of 625 times of heart rate, blood pressure, pulse saturation, regional tissue oxygenation, and 98 serum lactate were retrospectively analyzed. The relationship of the tissue oxygen saturation and clinical adverse events was explored.ResultsThe adverse event by routine monitoring was 69 (11.04%) person-time: isolated hypoxia in 27, hypoxia combined increased lactate in 16, hypotension in 6, hypotension combined increased lactate in 17, isolated increased lactate in 3. A reduction of 12.80% in cranial oxygen predicted the high probability of adverse events, with a sensitivity of 85.30% and a specificity of 87.00%. A reduction of 20.60% in splanchnic oxygen predicted the high probability of adverse event, with a sensitivity of 73.50% and a specificity of 91.2%. On average, the splanchnic oxygenation had fell 3 minutes before a reduction of blood pressure, or 45 minutes before an increase in lactate.ConclusionFor preoperative patients with pulmonary atresia, a fall of 12.80% in cranial oxygen saturations, or of 20.60% in splanchnic oxygen saturation, should attract clinician’s awareness.
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 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.
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 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 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.