Percutaneous pulmonary valve implantation (PPVI) is an exciting growing field in cardiovascular medicine.With the sophisticated improvement of biomedical engineering,development of PPVI offers people a new way of less invasive techniques to treat these groups of patients with pulmonary valvular regurgitation over the past several years.New stent designs and operative and interventional hybrid approaches are under investigation.This nonsurgical approach has been proven to be feasible and holdpromise,although many obstacles still exist.At the present time,PPVI has been widely used in clinical in the United States and European countries,but in our country,only a small number of cases reports have been published.With wider application and development of PPVI,cumulative experiences and long-term follow-up of PPVI therapies,better criteria will be established for patient election.Complications will be reduced and safer and more effective treatment results will be achieved.PPVI therapies will be applied to a larger subset of patients and become an important alternative to future conventional pulmonary valve replacement.

Lung injury was one of the most complications after extracorporeal circulation. As a result of blood exposure to the surface of the extracorporeal circulation circuit, the complements and leukocytes were activated. The activated neutrophil adhered to endothelial cells and released many inflammatory mediators, as protease, oxygen free radicals, arachidonic acid metabolites. All of these inflammatory mediators caused lung injury. In vivo and in vitro, many studies demonstrated that leukocyte depletion in extracorporeal circulation of heart operation could reduce lung injury and improve lung function. Leukocyte depletion could reduce pulmonary resistance, particularly effective in patients with a low preoperative oxygenation capacity and in those for whom an extended period of extracorporeal circulation was required.

Purpose Using quantitative morphometric techniques, To analyzse the developmental characteristics of pulmonary arterial and alveolar with a porcine model of cyanotic congenital heart defect with decreased pulmonary blood flow that we had established. Methods Lung biopsy specimens were taken from animals of control ( C) and cyanosis ( D) groups and than sectioned and stained. The morphological observation and the method of half-quantitative morphometric technique were applied to measure the following char-acteristics:the media thickness ( MT) and the percentage of media thickness ( MT%) , the media section area ( MS%) and the per-centage of media section area ( MS) , the numbers of micro-arteries per square centimeter ( APSC) . In addition, we calculated the ratio of CMA, PMA and NMA in the same section of lung biopsy specimens. Results There showed universally distended, irregular and thin-walled vessels in the lung biopsy specimens of group D under the light microscope. There was an increased number of pulmonary arteries whose media were hypoplastic. The MT, MT% and MS, MS% in group D were significantly less than those of group C ( all P<0. 001), the APSC was also less in group D than in group C (P<0. 01), but MAN were not significantly different between two groups (P>0. 05). Electron microscopy revealed endothelial swell and irregularity, a thinned basement membrane, a disorganized or disrupted elastic fibers layer. The smooth muscle of vascular media shrunk. In addition to these, other changes included the mitochon-drion vacuolization, a diminished cellular organelle and type Ⅱ alveolar epithelial hyperplasia. Conclusion The pulmonary vessels are evidently hypoplastic or degenerated in cyanotic congenital heart defect with decreased pulmonary blood flow, and the function is thought to be influenced by the morphological changes. To promote the pulmonary artery growth and improve this pathophysiological state, it is critical to increase the pulmonary blood flow.

Non-coding RNA refers to a class of RNAs that cannot encode proteins, and they play a very important role in regulating cellular activities. Pulmonary arteryhypertension is a group of diseases characterized by progressive elevation of pulmonary vascular pressure. Its pathogenesis is complex and its influencing factors are numerous. The study found that non-coding RNA, as a transcription product that does not participate in translational functions, plays an important role in the pathogenesis of patients with pulmonary arteryhypertension. With regard to the more well-studied and relatively mature circRNAs, lncRNAs, and miRNAs in non-coding RNAs, this article review the pathophysiological processes involved in the formation of pulmonary arteryhypertension.

ObjectiveContrast research the operative effect of the pulmonary artery banding (PAB) in the congenital heart disease with pulmonary artery hypertension,underwent the single ventricle repair procedure in different age.Methods 49 cases,male 31 and female 18.Age (7.8 ± 3.8 ) years old,weight ( 8.82 ± 4.24) kg,percutaneous blood oxygen saturation ( SPO2 ) 0.90 ± 0.04,preoperative mean pulmonary artery pressure (mPAP) ( 54.6 ± 16.8 ) mm Hg.single ventricle 13cases,tricuspid atresia 12 cases,double outlet of right ventricle with left ventricular dysplasia 11 cases,tricuspid stenosis 5 cases,ventricular imbalance type complete atrioventricular canal defect 5 cases and cross heart cases 3 cases.divided into three groups:≤0.5 years old of 17 cases,0.5 to 2.0 years old of 17 cases,≥2.0 years old 15 cases.All cases taken PAB under anesthesia and cpb,inhaled oxygen concentration 40％,SPO2 0.85,MPAP 20 mmHg.comparatively analysis postoperative SPO2,MPAP,Ventilator using time,ICU stay time and operation mortality of the three groups.ResultsThe postoperative PAP obviously decreased.Three groups of postoperative SPO2,mPAP,ventilator using time,ICU stay time were not significantly different.More than were followed up 6-72 months,1 cases ( 1-2 months) dead in aspiration two months postoperation.the other three cases had been completed Glenn and/or Fontan.ConclusionPAB can effectively reduce the pulmonary artery pressure in the different age children with congenital heart disease and pulmonary artery hypertension,the effect is good.more than 2 years old children still PAB feasible operation,and likely to complete the Glenn and/or Fontan procedure.

Objective To review our experiences of diagnostic methods and surgical treatment of the left superior vena cava (LSVC) draining into the left atrium.Methods Nineteen patients with LSVC draining into the left atrium were diagnosed and treated surgically from February 1998 to January 2012.All the cases were combined with other congenital heart diseases including patent ductus arteriosus,ventricular septal defect,atrial septal defect,single atrium,triatriatum,partial endocardial cushion defect,anomalous pulmonary venous drainage,right ventricle outflow stenosis,pulmonary valve stenosis,tetralogy of Fallot,double outlet right ventricle,complete endocardial cushion defect,tricuspid atresia.The patients were diagnosed through different methods including echocartiographic examination,cardiac catheterization,computer tomography,and explored during the operation,even postoperatively.All were treated surgically with four techniques including simple ligation to the LSVC,including ligation during a redo procedure; intra atrial rerouting to drainage the flow from the LSVC to the right atrium,atrial septum reconstruction to make the outlet of the LSVC lying in the right side of the patched atrial septal,and bidirectional Glenn shunt to get a physiological result.Results No mortality postoperatively.All the cases were uneventful postoperatively.And the main postoperative course was related only to the main diagnosis of congenital heart disease,not to the left superior vena cava draining into the left atrium.The echo examination result was satisfied before the discharge.The early and long term follow-up(1-11 years) results are excellent,no arrhythmia,no cardiac deficits after echo examination,including stenosis obstruction and residual shunt.No death.Conclusion The diagnostic methods of the LSVC draining into the left atrium are difficult,the only way to make the diagnosis clearly enough before the operation is depending on improving of more and more comprehending to this rare cardiac anomaly,by the pediatric cardiologists,the sinologist,the intensive care unit,and the pediatric cardiae surgeons.The choice of different surgical treatment is depending on the diagnosis and the findings during the operations,and the results are excellent after a suitable choice made.

Objective To analyze and summarize the applicative experience and operative effective of the diaguostictreatment-repair strategy in the transpossion of great arteries(TGA) infants with ventricular septal defect and severe pulmonary hypertension more than 6 months.Methods From January 2010 to October 2011,17 TGA cases with ventricular septal defect and severe pulmonary hypertension.There were 13 male and 4 female.≥0.5-＜ 1.0 years old 6 cases,≥ 1.0-＜ 3.0 years old 3,≥3.0 years old 8 cases.Combine anomalies: patent ductus arteriosus in 6 cases,atrial septal defect in 5 cases,valve insufficency in 2 cases.All preoperative cases were performed echocardiography,right-sided heart catheterization 3 cases,coronary CT examinationll cases.After diagnostic-treatment 2-4 weeks,all cases performed arterial switch operation under compound intravenous and inhaled anesthesia.Results No operative death.After diagnotic-treatment,SPO2 improved 10％-21％,and mPAP decreased 10-20 mm Hg.Follow-up 11.2 (6,20) months,one dead.Postoperative residual pulmonary arterial hypertension in 35.29％,6/17cases,all of them were ≥3 years old.Continue to pulmonary arterial hypertension targeted drugs treatment for 6-20 months later,pulmonary artery pressure decreased obviously.Conclusion The TGA infants with ventricular septal defect pulmonary arterial hypertension more than 6 months,can be selectively performed arterial switch operation under went diagnostic-treatment-repair strategy,continue to pulmonary arterial hypertension targeted drug therapy postoperation,the effect is good.

Objective:To improve the understanding of special type of atrioventricular septal defect (AVSD) and reduce clinical missed diagnosis and misdiagnosis by investigating its echocardiographic features.Methods:Echocardiographic images of patients with special type of AVSD diagnosed by echocardiography and confirmed by surgery in Pediatric Cardiovascular Center, Beijing Anzhen Hospital, Capital Medical University from November 2011 to November 2021 were retrospectively analyzed. Depending on the presence of ventricular septal defect(VSD), special type of AVSD were divided into two types, namely AVSD with intact ostium primum and AVSD with intact ostium primum and ventricular septum. The echocardiographic features of special type of AVSD were summarized.Results:Twenty-five cases of special type of AVSD were diagnosed by echocardiography and 13 cases were confirmed by surgery, including 12 cases with intact ostium primum and 1 case with intact ostium primum and ventricular septum. There were 5 cases without surgical indications, and 1 case was kept under observation due to small VSD. One patient with complex malformation only underwent pulmonary artery banding operation without treating intracardiac malformation. The other 4 cases of perimembrane VSD and 1 case of partial AVSD were misdiagnosed as special type of AVSD by echocardiography. All patients had mild to severe left atrioventricular regurgitation before surgery, and most patients remained left atrioventricular regurgitation of varying degrees after left atrioventricular valve formation. Characteristic sonographical findings included: ①The ostium primum was continuous and there was no ostium primum defect. ②The attachment points of the left and right atrioventricular valves were at the same level. ③The left atvioventricular valve was a trileaflet left atrioventricular valve and attached to the crest of the ventricular septum, forming the so-called "anterior mitral valve leaflet cleft". ④The two groups of papillary muscles of the left ventricle changed the normal anterolateral and posterolateral alignment, but showed a anteriorly and posteriorly relationship (counterclockwise transposition). ⑤In the presence of VSD, an inlet ventricular septal defect would be present. ⑥Color Doppler showed varying degrees of regurgitation signals from the left atrioventricular valve during systolic period. ⑦Other complex deformities may be combined with special type of AVSD.Conclusions:Echocardiography can make a definite diagnosis for special types of AVSD. The prognosis of this malformation is closely related to the left atrioventricular valve, and the shaping effect depends on the development of left atrioventricular valve and regurgitation.

Objective:To analyze the characteristics and surgical outcomes of Shone′s syndrome in children, and to explore the surgical treatment strategy and technical key.Methods:Retrospective study.Children with Shone′s syndrome treated in the Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University from May 2013 to June 2019 were retrospectively analyzed for their baseline characteristics and surgical data.The data were compared by Student t-test. Results:A total of 22 children with Shone′s syndrome were recruited, involving 15 males (68.2%) and 7 females (31.8%). There were 6 cases (27.3%) of complete form of Shone′s syndrome and 16 cases (72.7%) of incomplete form.No deaths were reported.The postoperative mitral valve velocity [(149.7±38.2) cm/s vs.(234.9±34.0) cm/s, t=7.341, P<0.05], left ventricular outflow tract velocity [(202.0±105.0) cm/s vs.(328.6±120.3) cm/s, t=6.575, P<0.05] and aortic arch coarctation velocity [(186.1±60.9) cm/s vs.(347.9±100.8) cm/s, t=7.630, P<0.05]were significantly lower than those of preoperative levels.There were no complications occurred at 1-year follow-up, and 91.7% of the patients were followed up for 3 years, and 80.2% were followed up for 5 years without complications, 2 cases needed reoperation. Conclusions:Surgical treatment of Shone′s syndrome achieved satisfactory outcomes.Early diagnosis and early intervention are beneficial to children with Shone′s syndrome, although they need to be followed up and have the risk of reoperation in the long term.

Objective:To explore the therapeutic strategy and result of adult total anomalous pulmonary venous connection(TAPVC).Methods:From November 2011 to November 2019, 6 adult patients with TAPVC underwent surgical correction. The Darling types include 4 cases of supracardiac , 1 case of intracardiac and 1 case of mixed type. There were 1 male and 5 female. The mean age was(28.6±4.8) years old and the mean weight was(47.3±3.67) kg. Preoperative oxygen saturation was 0.91±0.05.Results:All patients underwent primary repair successfully without perioperative death and complications. The average cardiopulmonary bypass time was(122.0±35.9) min, and the aortic cross-clamp time was(78.2±20.4) min. The mean postoperative hospitalization was(9.7±2.9) days, and the mean intensive care unit time was(3.5±1.4) days.The mean mechanical ventilation was(17.1±2.9) h. There were no later left heart dysfunction and pulmonary vein obstruction during the follow-up of 6-100 months.no pulmonary artery hypertension was identifed.Conclusion:TAPVC can be repaired savely in adult and satisfied result can be anticipated.