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 explore the surgical treatment strategy and the results of medium and long-term follow-up of Ebstein's anomaly(EA) through analyzing 127 patients.Methods:From January 2010 to Decembe 2018, 127 patients with EA underwent operation. There were 59 male and 68 female patients, with age of(9.5±3.7) years(0.67-18.0 years), weight of(27.1±11.7) kg(6.4-76.0 kg).13 cases were diagnosed as WPW syndrome by Echocardiography. There were 11 cases of VSD, 42 cases of ASD, 34 cases of PFO, 6 cases of PS and 127 cases of TI. The surgical strategy included 92 cases of biventricular repair(including 20 cases retained atrial shunt) and 35 cases of one and a half ventricular repair(including 8 cases retained atrial shunt). Except 2 patients underwent simple tricuspid valve repair(including 1 case of tricuspid annuloplasty ring), 125 EA patients in this group were all treated with cone reconstruction method for atrioventricular folding and tricuspid valvuloplasty.Results:6 patients died within perioperative period because of low cardiac output, pulmonary infection and cerebral hemorrhage, with a mortality of 4.7%. The complete atrioventricular block occurred in 2 patients and pacemaker were implanted postoperatively. 7 patients(5.5%) underwent pericardial fenestration due to pericardial effusion. All the other patients recovered successfully and were discharged from hospital without serious complications. The echocardiographic result indicated that the degree of tricuspid insufficiency were significantly reduced and cardiac function were improved before all patients discharged. The ratio of functional right ventricle to atrialized right ventricle(FRV/ARV) in the whole group was 0.87±0.37. FRV/ARV ratios of biventricular repair group and one and a half ventricular repair group were 1.12±0.39 and 0.48±0.28, respectively. And the difference between the two groups was statistically significant( t=5.685, P< 0.001). 110 cases were followed up in 3 to 96 months. 3 patients(2.7%) died during medium and long-term follow-up. Conclusion:The principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. Surgical treatment strategies should be decided according to the condition of tricuspid valve, ratio of FRV/ARV. Surgical treatment has a good effect for Ebstein’s anomaly in the medium and long-term.

Objective:To analyze the results of surgical treatment in patients with obstructed total anomalous pulmonary venous connection.Methods:Inclusion criteria: Patients with obstructed total anomalous pulmonary venous connection, who underwent surgical treatment in our hospital between December 2012 to January 2018. Exclusion criteria: (1)With complicated malformations, such as single ventricle and visceral inversion; (2)The maximum flow velocity of pulmonary vein was less than 1.2 m/s; (3)Had not underwent surgery. 57 patients[33 males and 24 females, age: (3.2±1.7) months, weight: (5.0±0.9) kg] were enrolled. There were four anatomical subtypes: supracardiac type(n=30), cardiac type(n=16), infracardiac type(n=5), and mixed type(n=6). Patients were followed up after operation.Results:During the follow-up of(52±21) months, 8 cases(14.0%, 8/57) died postoperatively. Postoperative pulmonary vein stenosis was present in 7 patients(12.3%, 7/57). The mortality of patients with postoperative pulmonary vein stenosis(57.1%, 4/7) was significantly higher than that of patients without postoperative pulmonary vein stenosis(8.0%, 4/50).Conclusion:The results of surgical treatment in patients with obstructed total anomalous pulmonary venous connection were encouraging, however, recurrent pulmonary venous stenosis still should be taken care.