Brain natriuretic peptide (BNP) is a neuroendocrine hormone,which has a positive correlation with degree of cardiac dysfunction and pulmonary arterial hypertension of children with congenital heart disease (CHD).BNP has been shown to be a simple,accurate and useful biochemical indicator in assessing cardiac dysfunction degree and reflecting perioperative cardiac function state of children with CHD.It has prognostic value in congenital heart surgery and can guide the perioperative treatment and reduce the postoperative complications and mortality.It is a quick,instant bedside inspection supplementary means.This article describes the relevance between BNP and CHD and the application progress of BNP in pefioperative of CHD.

Objective To study the feasibility and safety of interventional closure for the treatment of infant’s perimembranous ventricle septal defect (PmVSD). Methods During the period from Jan. 2012 to Jan. 2013, a total of 229 single PmVSD infants less than three years of age were admitted to authors ’ hospital. The infantile patients were divided into intervention group (n = 88) and surgery group (n = 141). Patients in the intervention group underwent interventional closure procedure , while patients in the surgery group received conventional cardiac surgery. The operation success rate , the main and minor complication rates, the operation time, the postoperative hospitalization days, the incidence of postoperative pulmonary infection and the medical cost were recorded , and the results were compared between the two groups. Results No statistically significant differences in the operation success rate, the main complication rate, the postoperative hospitalization days and the medical cost existed between the two groups (P > 0.05). But the minor complication rate and the operation time of the intervention group were better than those of the surgery group. In the surgery group, the minor complication was mainly the respiratory infection, which was manifested as higher leukocyte count, higher C-reactive protein level, higher myocardial damage marker level as well as higher vasoactive drug scores in 24 hours after the operation , and the above items were significantly higher than those in the intervention group. Conclusion For the treatment of infant’s perimembranous ventricle septal defect, percutaneous transcatheter closure is clinically feasible. This technique is safe and reliable with obvious advantages when the indication is strictly observed and the procedure is carefully manipulated. This treatment can partly replace the conventional surgery.

Objective To study on the evaluation of haemodynamics in the normal and pulmonary hypertensive mouse hearts using pressure volume loops measured by electric catheter.Methods Compared the difference in haemodynamics between mice exposed to chronic hypoxia for 10 days,which causes hypoxia-induced pulmonary hypertension (experimental group,n =8),and mice raised under normal atmospheric pressure (control group,n =8).The right carotid artery was cannulated with a 1.2 F catheter and advanced into the ascending aorta,then punctured towards the right ventricular apex.A 1.2 F admittance pressure-volume catheter was introduced using a 20-gauge needle to obtain the pressure-volume measurements and calculate hemodynamic parameters.Results There were no significant differences in average by weight,ratio of right atrial weight to body weight,left atrial weight/body weight,left ventricular free wall and septum weight/body weight between the 2 groups(all P ＞ 0.05).The ratio of right rentricle/left rentricle and septum weight as well as right rentricular weight/body weight was increased in the experimental group and of significant difference when compared to the control.The mice in in the experimental group had a 61％ mean decrease in cardiac output,a 55％ decrease in ejection fraction,and a 63％ decrease in ventricular compliance(P ＜0.05).The increase in dP/dtmax-EDVand PRSWfound in the experimental group reflected significant increase in myocardial contractility.Increase in Ees was observed but without significant difference as compared to the control.Ea significantly increased in the experimental group resulting in significant decrease in Ees/Ea from (0.71 ±0.27) to (0.35 ±0.17) (P＜ 0.005).Conclusion This study demonstrates the feasibility of obtaining RV pressure-volume measurements in mice using electric catheter.These measurements provide insight into right ventricular-pulmonary artery interactions in healthy and diseased conditions.

Objective To study pulmonary wedge angiography ( PWA ) with hemodynamic the evaluation of children with congenital heart disease and pulmonary artery hypertension ( PAH) . Methods Hemodynamic measurement and pulmonary wedge angiography were performed in 50 children with congenital heart disease. Comparison and analysis were made from the data obtained from PWA and catheterization. Results After PWA, the patients were categorized into 3 groups according to the measured hemodynamics parameters:group A [ n=15, patients with normal mean pulmonary artery pressure ( mPAP≤25 mmHg) and normal pulmonary vessel resistance (PVR﹤300 dyne?s?cm5)], group B [n=24, patients with PAH (mPAP﹥25 mmHg) but normal PVR] and group C (n=11, patients with PAH and elevated PVR (PVR≥300 dyne?s?cm5). Rote of tapering (ROT) was significant lower in group C than in group A and B (F=42. 559,P﹤0. 05). Pulmonary circulation time (PCT) was higher in group C than in group A and B (F=6. 037,P﹤0. 05). ROT correlated negatively with PVR (r = -0. 606, P ﹤0. 05). PCT index correlated positively with PVR (r=0. 783,P=0. 01). There was no significant correlation between PCT and mean pulmonary artery hypertension (mPAP). Conclusions PWA may help to make quantitative analysis of the pulmonary vascular status in patients with congenital heart disease.

Objective To investigate the feasibility of the simultaneous measurement of right ventricular pressure-volume loops by cardiac catheterization and 2D electrocardiogram. Methods Patients referred for pulmonary hypertension underwent right heart catheterization in our hospital between June 1st, 2015 and June 1st, 2017 are to be enrolled in this study. The right ventricular volume was measured simultaneously by catheter and electrocardiogram. The pressure-volume loops were constructed by the parameters of the pressure and volume in the same cardiac cycle. Results The study completed in four cases and their pressure-volume loops were drawn. The obtained images were irregular and there was no relationship among them. As a result, the construction was a failure. Conclusions The construction of the right ventricular pressure-volume loops of pulmonary hypertension patients by simultaneous catheterization and 2D electrocardiogram is difficult to overcome the technology defects.

Objective To evaluate the role of MSCT in diagnosing Cantrell syndrome.Methods Five patients with Cantrell syndrome were enrolled in this study retrospectively.All clinical data,especially imaging data were collected at enrolment.Maximum intensity projection(MIP),multi planar reconstruction(MPR)and volume rendering(VR)of the analysis method of MSCT were used to describe the characteristics of Cantrell syndrome.Results The age of 5 patients ranged from 2 days to 24 years,4(4/5)cases were Cantrell syndrome and 1(1/5)case was incomplete Cantrell syndrome,3(3/5)cases were confirmed by surgery.Five cases were all diagnosed as ectocardia,thoracocyllosis,pericardium defect and diaphragm defect with MSCT.Four had sternum dysplasia,abdominal wall defect, 3 had ventricular diverticulum,2 had umbilical hernia.Conclusion MSCT can be used in accurately diagnosing Cantrell syndrome in clinical work.

Objective:To investigate the risk factors of fulminant myocarditis in children.Methods:The clinical data of 67 children with clinical diagnosis of viral myocarditis from January 2015 to December 2018 in our hospital were retrospectively analyzed.The children were divided into fulminant myocarditis group( n=13)and common myocarditis group( n=54). The clinical data of gender, age, history of pre-infection, clinical manifestations, laboratory tests, electrocardiogram, echocardiography, and imaging findings were compared between the two groups.The multiple Logistic regression analysis was used to identify the independent risk factors of fulminant myocarditis. Results:(1)Seven cases(53.8%)died in the fulminant myocarditis group, 4 cases(30.8%) of them died within 24 hours after admission, and all the children in the common myocarditis group improved and discharged.(2)The incidences of facial cyanosis, abdominal distension, convulsions, and chills were higher in the fulminant myocarditis group than those in the common myocarditis group( P<0.05). (3)The level of creatinekinase-MB, lactate dehydrogenase, α-hydroxybutyric dehydrogenase and aspartate transferase in the fulminant myocarditis group were higher than those in the common myocarditis group( P<0.05). (4)On electrocardiogram, QRS wave duration in the fulminant myocarditis group was longer than that in the common myocarditis group[118(82, 127)ms vs.62(62, 122)ms, P<0.05]. The incidences of ventricular tachycardia in the fulminant myocarditis group was higher than that in the common myocarditis group( P<0.05). (5)In the fulminant myocarditis group, the incidences of left ventricular ejection fraction(LVEF)decreased, the left ventricular short axis fraction shortening(LVFS), and the incidence of left ventricular enlargement were higher than those in the common myocarditis group[92.3%(12/13)vs.18.5%(10/54), 84.6%(11/13)vs.9.3%(5/54), 76.9%(10/13)vs.13.0%(7/54), P<0.05]. Chest X-ray examination of the fulminant myocarditis group showed that the incidences of heart shadow enlargement and pulmonary blood stasis were higher than those in the common myocarditis group( P<0.05). (6)Multiple Logistic regression analysis revealed that LVEF reduction( OR=19.015, 95% CI 1.456-248.348, P=0.025), LVFS reduction( OR=18.691, 95% CI 2.062-169.453, P=0.009)and prolonged QRS wave duration( OR=1.040, 95% CI 1.001-1.082, P=0.046) were independent risk factors for fulminant myocarditis. Conclusion:The early mortality of fulminant myocarditis is high in children, and the LVEF reduction, LVFS reduction and prolonged QRS wave duration are independent risk factors for fulminant myocarditis.

OBJECTIVE: To discuss the advantages and technical limitations of various molecular genetic techniques in the diagnosis of two infants featuring all-round developmental retardation. METHODS: The two patients were initially screened by using chromosomal microarray analysis (CMA). For patient 1, his parents were also subjected to CMA analysis, and the data was analyzed by using ChAS and UPD-tool software. For patient 2, methylation-specific PCR (MS-PCR) was carried out. RESULTS: Patient 1 was diagnosed with maternal uniparental disomy (UPD) type Prader-Willi syndrome (PWS) by CMA and UPD-tool family analysis. His chromosomes 15 were of maternal UPD with homology/heterology. Patient 2 was diagnosed with deletion type PWS by combined CMA and MS-PCR. CONCLUSION Correct selection of laboratory methods based on the advantages and limitations of various molecular techniques can help with diagnosis of genomic imprinting disorders and enable better treatment and prognosis through early intervention.

Pulmonary arterial hypertension (PAH) is a severe, progressive disease leading to right ventricular failure and finally death. Lung transplantation is recommended for PAH patients who do not respond to targeted drug combination therapy or World Health Organization functional class (WHO FC) Ⅲ or Ⅳ. However, only 3% of PAH patients can recieve the lung transplantation. A novel implantable interatrial shunt device (ISD) can create a relatively fixed right-to-left shunt established by balloon atrial septostomy (BAS). The device may decompress the right sided chambers, facilitate left heart filling, improve organ perfusion and reduce the likelihood of syncope, acute pulmonary hypertensive crisis and death. The systemic oxygen transport improves despite hypoxemia. Implantation is simple, feasible and safe, and the X-ray time and operation time are short. There is no severe complication or thrombosis during the mid-term follow-up of the clinical studies and the device remained patent. The syncope symptoms, six-minute walk distance, cardiac index and systemic oxygen transport improve significantly in the patients. ISD may be currently the last alternative treatment to improve symptoms and prolong survival in currently drug-resistant patients with severe PAH.