| Pulmonary atresia(PA)is a rare cyanotic congenital heart disease.According to the anatomical structure and pathophysiological changes,it is divided into two types:pulmonary atresia with intact ventricular septum(PA/IVS)and pulmonary atresia with ventricular septal defect(PA/VSD).Although they are different in pathological anatomy,pathophysiological changes and surgical treatment strategies,both of them have severe systemic hypoxia symptoms,the operation is difficult and the mortality rate is high.The anatomical structure of PA/IVS is complex and generally has the following characteristics:(1)pulmonary valve atresia,resulting in no functional connection between the right ventricular outflow tract and the pulmonary artery bed,(2)there is a complete interventricular septum,so that there is no connection between the left and right ventricles.(3)Tricuspid valve open.Due to the heterogeneity of PA/IVS,the surgical treatment varies greatly.In general,the surgical strategy of PA/IVS mainly depends on the development of the right ventricle.Biventricular repair is performed when right ventricle is well developed;otherwise,palliative surgery may be used.Treatment options include:surgical treatment,interventional therapy and hybrid surgery.However,there is still no consensus among cardiac centers on the specific surgical methods and timing of surgical intervention for children with different PA/IVS.The basic characteristics of PA/VSD include:(1).There is a large malaligned VSD with right ventricular hypertrophy.(2).The main pulmonary artery is completely occluded,and there is no direct connection between the right ventricle and the pulmonary circulation.The main pulmonary arteries of these children can be completely occluded or absent.Pulmonary blood supply comes from extracardiac blood flow,most commonly through PDA or major aortopulmonary collateral arteries(MAPCAs).Due to the complex anatomical and pathophysiological characteristics of PA/VSD,its surgical treatment has always been difficult.At present,most cardiac centers in China and abroad have adopted one-stage palliative surgery to promote pulmonary vascular development,and staged surgery is performed depending on the development of pulmonary vessels.However,different centers have different reports on the therapeutic effects of different radical procedures,and have different conclusions on their long-term prognosis.In addition,the mortality of children with PA undergoing biventricular repair is still high,and there are few reports about risk factors of death in children with pulmonary atresia after biventricular correction at home and abroad.Therefore,based on the previous research of our center,this series of studies focused on the direction of a range of studies on the surgical treatment strategy and prognosis of pulmonary atresia,and carried out three parts of the discussion:In the first part,we retrospectively analyzed the effects of different one-stage procedures on the prognosis of type I and II PA/IVS in our center during the past 10 years,evaluated the therapeutic effect,and provided reliable clinical data for optimizing the selection of one-stage procedures for type I and II PA/IVS.In the second part,we retrospectively analyzed the clinical data of children with PA/VSD who had received modified BT shunt,central shunt,Melbourne shunt and right ventricular outflow tract reconstruction in the first stage,as well as the differences in the prognosis of the corresponding staging surgery,in order to clarify the therapeutic effect of different one-stage radical surgery and its impact on long-term prognosis.In the third part,the prognosis of children with PA who had been admitted to our center and finally been performed biventricular correction was retrospectively analyzed to clarify the risk factors of death in children with PA after biventricular correction,and to provide reference for biventricular correction in children with PA.Part One Type I and II Pulmonary Atresia with Intact Ventricular Septum in Infants:A 10-year Experience in Initial Surgery at One CenterObjective:To explore the effect of initial surgery for type I and II pulmonary atresia with intact ventricular septum(PA/IVS).Methods:50 children with type I PA/IVS and 50 with type II PA/IVS who had undergone initial surgery were enrolled.Children with Type I were divided into groups A(n=25)and B(n=25).Group A had received BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve,whereas group B had undergone BT shunt combined with PDA ligation and pulmonary valve incision.Children with type II were divided into groups C(n=25)and D(n=25).Group C had received BT shunt combined with PDA ligation,right ventricular outflow tract(RVOT)incision and transannular patch.Group D had undergone BT shunt combined with PDA ligation,RVOT incision,transannular patch and artificial pulmonary valve implantation.The differences in mechanical ventilation time,length of ICU stay,mortality rate,tricuspid Z value,tricuspid regurgitation,oxygen saturation,pulmonary regurgitation,Mc Goon ratio,pulmonary artery transvalvular pressure,survival rate were compared between groups A and B,between groups C and D respectively.Results:The ventilator assistance time and length of ICU stay were greater in group C than in group D(80.96±8.42 h vs.65.16±4.85 h,P=0.045;222.00±11.72 h vs.162.48±7.91 h,P=0.048).The pulmonary artery transvalvular pressure was significantly higher in group A than in group B at 3,6,12,24 and 36 months after surgery(64.86±4.13 mm Hg vs.53.04±5.64mm Hg,P=0.045;69.47±1.93 mm Hg vs.55.95±4.04 mm Hg,P=0.005;80.16±3.76 mm Hg vs.73.24±2.34 mm Hg,P=0.035;62.95±5.64mm Hg vs.48.47±7.44 mm Hg,P=0.04;53.69±4.89 vs.45.77±3.26,P=0.02).Furthermore,the tricuspid Z value was significantly greater in group B than in group A at 3 and 24 months after surgery(-(1.37±0.04)vs.-(1.43±0.06),P=0.03;-(0.41±0.06)vs.-(0.51±0.11),P=0.02).Summary:1.The effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve.2.The effect of BT shunt combined with PDA ligation,RVOT incision,transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation,RVOT incision and transannular patch.Part Two Clinical Effect Comparison of One-stage Palliative Surgical Treatment Strategy for Pulmonary Atresia with Ventricular Septal Defect and Prognostic Analysis of Staged SurgeryObjective:To investigate the clinical effect of one-stage palliative surgery for pulmonary atresia with ventricular septal defect and its effect on long-term prognosis.Methods:Children with pulmonary atresia and ventricular septal defect who had undergone one-stage palliative surgery in our hospital from January2009 to December 2018 were selected.There were 72 males and 73 females,with a body mass of(8.94±2.55)kg.78 cases were included in type A and 67cases in type B.According to the one-stage palliative operation,they were divided into four groups:modified BT shunt group(40 cases),central shunt group(46 cases),Melbourne shunt group(41 cases)and right ventricular outflow tract reconstruction group(18 cases).The ventilator assisted time,ICU time,percutaneous oxygen saturation,positive inotropic drug score and mortality were compared among the four groups.The Mc Goon ratio,Nakata index,the increased value of Mc Goon ratio and Nakata index of the four groups were monitored and compared.The operation time,cardiopulmonary bypass time,ICU time,tracheal intubation time,positive inotropic drug score and radical rate were compared among the four groups.Results:Compared with the four groups,the time of ventilator assistance and ICU time in the right ventricular outflow tract reconstruction group were longer than those in the latter three groups,but the differences were not statistically significant(all P>0.05).There were no significant differences in mortality between the first-stage palliative surgery group and the other three groups(all P>0.05).The positive inotropic drug score was higher than the other three groups,and the difference was statistically significant(P<0.05).The degree of pulmonary vascular development in the right ventricular outflow tract reconstruction group was better than that in the other three groups,and the difference was statistically significant(P<0.05).The 3-year radical cure rate was 73.33%,which was higher than the other three groups,and the difference was statistically significant(P<0.05).There were no significant differences in the mean radical operation time,cardiopulmonary bypass time,ICU time and ventilator time in the later radical operation between the right ventricular outflow tract reconstruction group and the other three groups(all P>0.05).Summary:1.Four kinds of palliative surgical methods can promote the development of pulmonary vessels.2.The recovery of cardiopulmonary function in children with one-stage right ventricular outflow tract reconstruction was slower than the other three groups.3.In terms of promoting pulmonary vascular development and improving the radical rate of children in the later stage,the right ventricular outflow tract reconstruction is the best.However,due to the degree of adhesion of the heart and surrounding tissues in children undergoing one-stage right ventricular outflow tract reconstruction is more serious than the other three groups,it is necessary to be cautious when dissociating during radical surgery to avoid the occurrence of massive hemorrhage.Part Three Risk Factors of Death in Children with Pulmonary Atresia after Biventricular CorrectionObjective:Analysis of risk factors of death in children with pulmonary atresia after biventricular correction.Methods:Children with pulmonary atresia who had undergone surgical treatment in our hospital from January 2010 to December 2020 were selected,including 42 cases of pulmonary atresia with intact ventricular septum(PA/IVS),26 males and 16 females,aged 5-27 days,body weight 3.0-3.9 kg;there were 64 cases of pulmonary atresia with ventricular septal defect(PA/VSD),including 36 cases of type A and 28 cases of type B;20 males and16 females were assigned to type A,aged 1.0-12.3 months,with body mass of3.3-7.8 kg;15 males and 13 females were distributed to type B,aged 1.2-12.9months,weighing 3.2-7.9 kg.According to the development of the right ventricle or pulmonary vessels,a one-stage surgical plan was developed for the selected children.Biventricular correction was performed when the PA/IVS children met the criteria of tricuspid Z value>-2,the PA/VSD children met the Mc Goon ratio>1.2,and the Nakata index>150mm~2/m~2 or NAPAI>150mm~2/m~2.Preoperative and posto-perative data of all patients were collected.Binary logistic regression model was used to analyze the risk factors of biventricular repair in children with pulmonary atresia.Results:Among the 42 children with PA/IVS who had undergone one-stage surgery,5 cases died,with a mortality rate of 11.90%.In 64 cases with PA/VSD,9 cases died,and the mortality rate was 14.06%.Finally,23 cases with PA/IVS underwent biventricular repair,the radical rate was 54.76%,4cases died,the mortality rate was 17.39%.Biventricular repair has been performed in 25 children with PA/VSD,and the cure rate was 39.06%.4cases died after surgery,and the mortality rate was 16.00%.Small tricuspid Z value(OR=0.428,P=0.044)and high positive inotropic drug score(OR=3.957,P=0.0 15)were the risk factors of death in children with PV/IVS after biventricular correction.Large right ventricular systolic pressure/left ventricular systolic pressure ratio(OR=2.536,P=0.043),small Nakata index(or small TNPAI)(OR=0.396,P=0.023),small Mc Goon ratio(OR=0.323,P=0.017)were the risk factors of death in children with PA/VSD after biventricular correction.Summary:1.PA/IVS with small tricuspid Z value and high positive inotropic drug score had poor prognosis during biventricular correction.2.PA/VSD with right ventricular systolic pressure/left ventricular systolic pressure ratio,small Nakata index(or small TNPAI),and small Mc Goon ratio had a high risk of biventricular correction.Conclusions:1.For type I PA/IVS,the effect of BT shunt combined with PDA ligation and pulmonary valve incision is superior to BT shunt combined with PDA ligation and balloon dilatation of pulmonary valve;for type II,the effect of BT shunt combined with PDA ligation,RVOT incision,transannular patch and artificial pulmonary valve implantation is superior to BT shunt combined with PDA ligation,RVOT incision and transannular patch.2.For PA/VSD,Modified BT shunt,central shunt,Melbourne shunt and right ventricular outflow tract reconstruction can promote the development of pulmonary vessels;the recovery of cardiopulmonary function in children with one-stage right ventricular outflow tract reconstruction was slower than the other three groups.In terms of promoting pulmonary vascular development and improving the radical rate of children in the later stage,the right ventricular outflow tract reconstruction is the best.However,due to the degree of adhesion of the heart and surrounding tissues in children undergoing one-stage right ventricular outflow tract reconstruction is more serious than the other three groups,it is necessary to be cautious when dissociating during radical surgery to avoid the occurrence of massive hemorrhage.3.PA/IVS with small tricuspid Z value and high positive inotropic drug score had poor prognosis during biventricular correction.PA/VSD with large PRV/PLV ratio,small Nakata index(or small TNPAI),and small Mc Goon ratio had a high risk of biventricular correction. |
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