Objective To probe into the causes,therapeutic and preventive methods of complications associated with transcatheter occlusion of atrial septal defect using the Amplatzer septal occluder in children. Methods Complications occurred in 9 of 211 cases. The complications in procedure of occlusion were those in which there appeared systemic circulatory systemic air embolism in 2,pulmonary air embolism in 1,pericardial tamponade in 1,ASO malposition requiring emergency surgical removal in 1,transient atrial extrasystoles in 1 and sizing balloon rupture in 1 case. Two cases with postoperative complications were found in the follow-up studies. These included perforation of mitral valve and ASO partially dislodged. In this study,5 children with intra-procedure complication received emergency therapy including surgical intervention,and others were needed only in the follow-up,as the complications were transient or asymptomatic. Results There were no children′s deaths in this study. The 5 cases that received treatment completely healed,and the others with intra-procedure complications were also had no sequela. Cases with mitral valve perforation and ASO partially dislodged were still in our follow-up studies,as the 2 patients had no symptoms. Conclusion Air embolisms occurred easily in atrial septal defect cases that received ASO therapy. Complications were mainly caused by inappropriate operative procedure and some complications needed emergency treatments. Follow-up studies were important to cases with transcatheter occlusion therapy.

Objective To summarize the experience of interventional catheterization conjoined with cardiac surgical procedures performed in 23 children with congenital heart disease(CHD). Methods 12 cases with residual patent ductus arteriosus after surgical ligation and 2 postoperative cases with severe cyanosis CHD with leftover ventricular septal defect (VSD) received transcatheter closure procedures. 2 children with branch pulmonary stenosis after total correction of tetralogy of Fallot (TOF) received angioplasty and stent placement, respectively. Another postoperative case with aortopulmonary collateral arteries (APCAs) angiorrhexis underwent transcatheter haemostasis. 6 cases underwent interventional catheterization in the preoperative period. 5 cases with transposition of great arteries (TGA) underwent balloon atrial septostomy (BAS) before artery switch procedure and 1 case of TOF underwent APCAs transcatheter occlusion before total correction. Results All cases with residual left to right shunts after surgery were occluded by transcatheter therapy. Interventional catheterization procedure relieved stenosis of branch pulmonary artery in postoperative case with TOF. Haemorrhage was stopped with embolization of ruptured APCAs. Hypoxia and acidosis improved after BAS therapy in 5 cases with TGA and intact ventricular septum and 3 survived from following artery switch procedure while 2 died. The APCAs was transcatheter occluded before TOF total correction. Conclusion Interventional catheterization therapy conjoined with cardiac surgical procedure was an effective and essential method in some postoperative CHD cases and in some of complex cyanosis CHD cases.

Objective To compare the impacts of right ventricular outflow tract pacing and right ventricular apical pacing on short-term cardiac function, QRS duration and pacing parameters in children undergoing VVI pacemaker therapy. Methods The clinical data of 38 children undergoing VVI pacemaker treatment in our department from July 2006 to November 2013 were retrospectively reviewed and analyzed to make grouping: 22 with right ventricular outflow tract pacing based on the operational records were assigned in one group and 16 with right ventricular apical pacing as the other group. The two groups were compared in terms of cardiac function indexes , QRS complex width and intra- and post- operative parameters of the implanted pacemakers. Results There were no significant differences between the two groups in pre-and post-operative cardiac function indexes , intra- and post-operative difference in pacing thresholds, pacing wire impedance and R-wave amplitude. The pre-and post-operative QRS duration [(134.95 ± 12.86)ms vs.(147.44 ± 22.35)ms, t=1.35, P=0.01] was statistically significant between the groups. Conclusion The right ventricular outflow tract pacing for children patients is safe and feasible. Although the two pacing approaches achieved lengthened duration of QRS, the right ventricular outflow tract pacing is more effective in lengthening the duration of QRS.

Objectives To summarize the diagnosis and treatment of cardiomyopathy caused by inborn errors of metabo-lism (IEM). Methods The retrospective study included 11 cases diagnosed as metabolic cardiomyopathy through tandem mass spectrometry, activity of serum enzyme, detection of urine mucopolysaccharide and gene analysis from 2012 to 2013. Six cases were diagnosed as primary carnitine deficiency (PCD). Four cases were diagnosed as glycogen storage disease (GSD) and only 1 case was diagnosed as mucopolysaccharidosis. Six PCD cases received carnitine supplementation and anti-heart failure thera-py and received follow-up for 2-10 months. Other 5 cases received supportive treatment and follow-up. Results Patients with PCD recovered soon after treatment but other 5 cases have died within 5 months. Conclusion IEM is an important cause of chil-dren cardiomyopathy which varied in clinical manifestation, diagnosis, treatment and prognosis of different kinds of metabolic cardiomyopathy. Early diagnosis and treatment could be lifesaving for cardiomyopathy caused by IEM.

Objective To monitor the degradation and bio-safety features of the bioabsorbable iron-based stent planted in the pig pulmonary artery.Methods A total of 19 miniature pigs were randomly assigned into 3 groups.Three cases were assigned as the control group; ten cases were assigned as stent implantation group A with one stent implanted in the pig pulmonary artery; the other six cases were assigned as stent implantation group B with two stents implanted in the pig pulmonary artery.Follow-up studies for 24 months were conducted in all cases.Repeated measures ANOVA were used to collect and analyze statistical processing data at multiple time points of the follow-up.Results After surgery,pulmonary artery blood flow velocity and right ventricular pressure were measured by echocardiography and cardiac catheterization.There was no significant difference in pulmonary artery blood flow velocity (Fgroup×time =1.06,P =0.40) and right ventricular pressure (Fgroup ×time =0.58,P =0.86) among each group,and no vascular renarrow circumstance was indicated.Pulmonary artery diameter expansion rate between stent group and control group was statistically different (Fgroup =3.57,P =0.05 ; Ftime =12.89,P ＜ 0.001 ; Fgroup ×time =2.99,P =0.03),suggesting that the stent could maintain a good expansion of the role of vascular in the follow-up period even though in degradation.Difference in the level of serum iron concentrations among the groups was not statistically significant (Fgroup ×time =0.94,P =0.52),suggesting that there was no evidence of iron overload.All animals survived to the follow-up endpoint,and no serious side effects caused by stent implantation were found.Conclusions Bioabsorbable iron-based stent planted in the pig pulmonary artery may experience a certain degree of degradation,and it is safe and stabile in animals.

Objective To analyze the causes of failed transcatheter closure for ventricular septal defects (VSD)in children. Methods One thousand two hundred and eighty children aged 13 to 141 months who underwent transcatheter closure from June 2009 to September 2013 in Guangdong General Hospital were selected. There were 43 failures(3. 36% ). The clinical data including transthoracic echocardiograph( TTE),radiography,interventional ap-proach and surgical findings were analyzed. Results Forty - three patients included 25 male and 18 female. The pa-tients' ages ranged from 13 to 141(43. 0 ± 31. 9)months and their weight ranged from 10 to 35(16. 3 ± 5. 59)kg. The causes of failure including doubly committed subarterial VSD misdiagnosed as perimembranous VSD(PMVSD)or intracristal VSD were in 6 patients. The size of occluder was too small in 13 cases,and there were statistical differences between three measurements of size of VSD(F = 19. 134,P = 0. 001). The size of VSD measured by left ventricular an-giography was significantly smaller than that measured by TTE,and there was statistical difference[(4. 78 ± 1. 11) mm vs(6. 48 ± 1. 43)mm,t = 4. 50,P = 0. 001]. The dimension of VSD measured by left ventricular angiography was significantly smaller than that measured by surgical findings,and there was statistical difference[(4. 78 ± 1. 11) mm vs(7. 02 ± 1. 08)mm,t = 5. 92,P = 0. 001]. But,the size of VSD measured by TTE had no significant difference compared with that measured by surgical findings(t = 1. 42,P = 0. 168). Aortic regurgitation occurred in 14 cases;atrioventricular block or left bundle branch block in 3 patients;tricuspid stenosis in 2 cases and residual shunt in 5 pa-tients. Conclusions Doubly committed subarterial VSD may be misdiagnosed as PMVSD or intracristal VSD. In the ca-ses of VSD concomitant with aortic valve prolapse,size of the occluders should be referred to VSD dimensions measured by TTE. In the cases of VSD adjacent to aortic valve,suitable occluders should be selected and operation technique should be improved to avoid aortic regurgitation.

Objective To evaluate the clinical effect for transcatheter closure of intracristal ventricular septal defects (IVSD) using duct occluder.Methods Between Jun.2012 and Sep.2013,implantaion of duct occluder was conducted in 27 IVSD pmients,which was compmed with acentric ventricular septal occluder attempted in 53 IVSD patients.The transthoracic echocardiography,electrocardiography,and clinical examination were observed at 24 hours,and after 1,3,6 and 12 months follows-up.Results Implantation of duct occluders were achieved in 26 (96.23 ％) patients and implantation of acentric ventricular septal occluder in 42 (79.25％) patients.The mean follow-up time was 6.78 months (1-17 months).The procedure time,fluoroscopic time and residual shunt were not statistically different between the 2 groups (all p ＞ 0.05).The size of VSD (t =-3.124,P =0.003) and occluder diameter (t =-4.285,P ＜0.001)were smaller and the procedure success rate was higher in the duct occluder group (x2 =9.099,P =0.011).Left ventricular end diastolic dimension,left atrial diameter,left ventricular end diastolic dimension,right ventricular end diastolic dimension and pulmonary artery diameter were decreased significantly (F =57.62,5.002,4.754,2.782,P =0.000,0.033,0.001,0.030) after surgical procedure compared with those before procedure,except for the right atrial diameter and left ventricular ejection fraction.No new serious aortic regurgitation or increased mitral regurgitation,tricuspid regurgitation was shown after procedure in 2 groups.Complete left bundle branch block occurred in 2 patients respectively,pericardial effusion and thromboembolism of right femoral artery occurred in 1 patient in the acentric ventricular septal occluder group.Conclusions The duct occluder for IVSD closure has presented a good result,without serious complications,which provides a new method for IVSD closure.Further studies are necessary to determine the long-term results in a larger population of patients.

abnormality from cardiomyopathy and endocardial fibroelastosis. Some pediatric patients have overlapped characteristics of both infant and adult type.

Objective To study the well-related cardiomyopathy genes in children with cadiomyopathy living in south china.Methods 43 childrens with cardiomyopathy admitted to Guangdong General Hospital between January 2015 to March 2017 were enrolled in this study.A standardized protocol for ultra-high coverage nextgeneration sequencing of the well-related cardiomyopathy genes were performed in all patients.A sequencing of Sanger were used to their immediate family members.Results A total of 28 mutations of pathogenic and suspected pathogenic in 23 genes were identified in 21 patients (48.8％).All of the mutations occurred only once.Conclusions Most cases with cardiomyopathy have gene mutations.The sequencing of the well-related cardiomyopathy genes can assist the clinical diagnosis.And many variants which the test detected need to be followed-up in order to gain benefit for the patients and their families.