Radiofrequency ablation of ventricular arrhythmias from the pulmonary sinus cusp in pediatric patients and the follow-up / 中华实用儿科临床杂志

Objective:To evaluate the strategy and safety of the radiofrequency ablation (RFA) on ventricular arrhythmias (VAs) originating from the pulmonary sinus cusp (PSC) in pediatric patients.Methods:Retrospective study.Fifteen patients with VAs originating from the PSC who were intervened by RFA in the Department of Pediatric Cardiology, Guangdong Provincial People′s Hospital between March 2014 to July 2020 were enrolled.All the patients met the indication criteria for RFA in pediatric patients.The electrocardiogram, ablation method of ablation were analyzed.Different curved catheters were selected for RFA according to the age and weight of the patients.The catheter was then inserted in a " U" or inverted " P" shape to the PSC.The long-term effect of ablation were reviewed.Results:The mean age and body weight of 15 patients with VAs originating from the PSC were (11.6±2.6) (6-15) years and (39.9±12.2) (19-65) kg, respectively.The electrocardiogram recorded during VAs originating from the PSC showed left bundle branch block and inferior axis with monomorphic R pattern, as well as a QS-wave in aVR and aVL.The electrocardiogram characteristics varied in patients with VAs originating from the PSC.The ideal excitation point was not found in the right ventricular outflow tract or the ablation was unsuccessful in all patients, and the earliest target was mapped and RFA was successful.Among the 15 patients, the successful ablation site was in the lower regions of the PSC, involving the right cusp in 11 patients(73.3%), the anterior cusp in 3 patients(20.0%), and the left cusp in 1 patient(6.7%). The earliest potential recorded at the PSC ablation site preceded the QRS complex onset by (27.3±6.0) ms.During the follow-up period for (2.7±2.0) years, no recurrence of VAs or complications were recorded.Conclusions:Under the premise of gentle catheterization procedure and appropriate radiofrequency energy, ablation was effective, safe and with low recurrence rate to eradicate VAs originating from the PSC in children.

Advances in microdialysis-coupled microfluidics for body fluid monitoring / 中华检验医学杂志

Microdialysis is a novel technique for rapid and continuous sampling of body fluid in the extracellular space, especially for some hard-to-obtain samples, e.g. cerebrospinal fluid, interstitial fluid. Microfluidic technology plays a significant role in body fluid analysis because of its miniaturization, high-throughput, and automation, offering a feasible method for rapid and low-cost biochemical analysis. In clinical practice, body fluid analysis is often required to be fast and/or capable of long-termly monitoring certain biomarkers. However, current technologies are insufficient to meet this requirement. The combination of microdialysis and microfluidic technologies could provide a new perspective to solve this problem.

Case report of Bentall operation for child Loeys Dietz syndrome / 中华实用儿科临床杂志

The clinical data, follow-up records and genetic results of a child with growth retardation and systemic multiple malformations, of whom was diagnosed Loeys Dietz syndrome (LDS), were retrospectively analyzed.This was a 3 years and 3 months old boy presented with incomplete cleft palate, spasm of middle fingers in both hands, hernia and hyperextension of knee joint.Color doppler ultrasound showed dilation of aortic sinus and ascending aorta, and mo-derate aortic regurgitation in the child.The analysis of genetic cardiovascular gene sequencing showed that there was a heterozygous mutation in TGFβR2 gene, which was a missense mutation.The child was diagnosed LDS type 2 according to the results of gene detection and clinical characteristics.He underwent " Bentall" aortic valve replacement + coronary artery lengthening transplantation (Cabrol) in Guangdong Provincial People′s Hospital.Postoperative recovery was satisfactory.This is the youngest case of Bentall operation reported in China.Connective tissue and organs are involved in the LDS.Systemic arteries, especially big arteries, are seriously involved and progressing rapidly.The combination of gene detection and clinical symptoms is the great value in the differential diagnosis of LDS.Echocardiography is an important method to monitor the progress of cardiovascular disease in children with LDS, which is important for the selection of surgical methods and timing.Bentall operation can also achieve good prognosis in children under 4 years old.

Application and follow-up of implantable cardioverter defibrillator for children and adolescents / 中华实用儿科临床杂志

Objective:To explore the application of implantable cardioverter defibrillator(ICD) in the prevention of sudden cardiac death for children and adolescents, the treatment of adverse events and follow-up studies.Methods:A total of 19 pediatric patients (12 males, 7 females) were admitted in the Department of Pediatric Cardio-logy, Guangdong Provincial People′s Hospital from January 2008 to March 2019, and their previous inpatient and outpatient data including medical history, ICD implantation method, postoperative program control, the use of antiarrhythmic drugs, proper/improper discharge of ICD and management of ventricular electrical storm were collected retrospectively and follow-up study was carried out.Results:A total of 19 pediatric patients were included, all of whom had once syncope or more before ICD implantation.The age at the time of ICD implantation was (12.30±2.08) years, and the weight was (40.00±13.93) kg.Eleven patients were implanted in the subpectoralis major space, 6 cases in the subfascial space, and 2 cases in the left abdomen and left armpit, respectively.All patients received oral antiarrhythmic drugs after surgery.The follow-up period was 3.2 years(0.3-11.1 years). All the children survived without complications such as electrode fracture, dislocation, venous thrombosis, cardiac perforation, capsular hematoma, rupture, and infection.Twelve cases (63.2%) had discharge events, including 10 cases (83.3%) of appropriate recognition/treatment events, and 2 cases (16.7%) of inappropriate recognition/treatment events, while 5 cases were ventricular electrical storms.Totally, 7 ICD cases (36.8%) were equipped with remote monitoring system, and 4 cases (57.1%) were detected whit adverse reactions, which were timely handled online and offline, thus avoiding adverse consequences.Conclusions:Currently, ICD is the most effective treatment for the prevention of sudden cardiac death in children and adolescents.According to age, weight and developmental conditions, different implantation methods should be selected.Appropriate/inappropriate discharge events after implantation should be identified in time, drug treatment should be optimized, and radiofrequency ablation should be performed if necessary.

Transthoracic echocardiographic diagnosis for partial anomalous pulmonary venous connection in children / 中华实用儿科临床杂志

Objective:To investigate the cause of missed diagnosis by transthoracic echocardiography (TTE) based on the characteristics of partial anomalous pulmonary venous connection (PAPVC), and to improve the ultrasonic diagnostic accuracy of PAPVC in children.Methods:The TTE results of 252 children under 12 years old who were confirmed with PAPVC at Guangdong Provincial People′s Hospital from January 2011 to June 2019 were reviewed retrospectively.The types of PAPVC and the associated atrial septal defects (ASD) as well as the confirmed and missed cases by TTE were analyzed.Results:PAPVC was right-sided in 238 patients (94.4%), left-sided in 8 patients (3.2%), and bilateral in 6 patients (2.4%). There were 177 cases (70.2%) whose pulmonary veins were abnorma-lly connected to the right atrium(RA), 37 cases (14.7%) to the junction of the RA and the superior vena cava (SVC), 27 cases (10.7%) to the distal SVC, and 6 cases (2.4%) to the inferior vena cava.Besides, pulmonary veins of 5 cases (2.0%) flew back to the RA through the coronary sinus.One hundred and ninety PAPVC cases were combined with sinus venous defects (SVD) and 53 cases combined with secondum ASD.Two hundred and twenty-one cases were accurately diagnosed while 31 cases were underestimated by TTE.The omission diagnostic rates of right superior pulmonary veins connecting to the distal SVC, 1 or 2 right pulmonary veins connecting to the RA or the junction of the RA and SVC, and left-sided PAPVC were 8 out of 18 (44.4%), 22 out of 215 (10.2%) and 1 out of 8 (12.5%), respectively.Among 54 cases with right superior pulmonary veins anomalously connected to the RA or the junction of the RA and SVC, 88.9% of them (48/54 cases) were combined with superior SVD.Among 161 cases with the right inferior pulmonary veins or 2 right pulmonary veins connected to the RA, 78.2% of them (126/161 cases) were combined with inferior SVD.There were statistically significant differences in ASD types between the 2 right-sided PAPVC groups.Conclusions:SVD is often associated with 1 or 2 right pulmonary veins connected to the RA or the junction of the RA and SVC.The cases with right superior pulmonary veins connected to the distal SVC are prone to be underestimated by TTE.Whether there is abnormal blood flowing into SVC should be noticed during superior sternal fossa examinations.Each pulmonary vein should be examined in detail in the TTE test and accurate diagnosis of PAPVC can be made in most cases.

Analysis of 30 cases of inherited cardiac arrhythmia syndrome in children / 中华儿科杂志

Objective@#To analyze and summarize the diagnosis and treatment experience of common inherited cardiac arrhythmia syndrome in pediatric patients, and explore the most appropriate therapy.@*Methods@#A retrospective review identified 30 pediatric cases (19 males, 11 females) diagnosed with long QT syndrome (LQTS), Brugada syndrome (BrS), catecholaminergic polymorphic ventricular tachycardia (CPVT), hypertrophic cardiomyopathy (HCM), arrhythmogenc right ventricular cardiomyopathy (ARVC) from January 2008 to December 2018 in the Pediatric Cardiology Department, Guangdong Provincial People′s Hospital. Data obtained included the diagnosis, treatment and follow-up outcome.@*Results@#The most common inherited cardiac arrhythmia syndromes were LQTS (n=14) including 1 case with epilepsy, CPVT (n=5), HCM (n=7), ARVC (n=1), and BrS (n=3). Twenty-seven cases were admitted to hospital due to syncope, whereas the remaining 3 cases of BrS had not presented with syncope before admission. The average onset age of inherited arrhythmia was (10.0±3.3) years. Genetic testing was performed on 20 patients. The median follow-up time was 40 months. Among 15 patients who underwent implantable cardioverter defibrillator (ICD) and survived, 2 patients had frequent ICD discharge. One patient underwent radiofrequency ablation, and the other one received left cardiac sympathetic denervation and an increased ICD defibrillation threshold, and the number of ICD discharge was significantly reduced. Among 10 patients who received drug therapy, 4 patients including two patients who discontinued treatment without advices died. Two patients whose parents refused treatment died, 1 case diagnosed with unexplained sudden cerebral death, and the remaining 2 cases without indication for drug therapy survived without any treatment.@*Conclusions@#Mortality rate is high in pediatric patients with inherited cardiac arrhythmia and syncope. The therapeutic effect of drugs are not satisfactory, ICD implantation is the most effective treatment to prevent sudden cardiac death currently, but the postoperative frequent discharge should be brought to the forefront and handled in time.

Congenital heart disease and neurodevelopment in children from the perspective of genetics / 中华实用儿科临床杂志

Children with congenital heart disease (CHD) are at risk of neurodevelopmental disorder (NDD) which can be manifested as a diversity of clinical type.Up to 20％-30％ of CHD children have genetic abnormalities.As an independent risk factor for NDD,genetic abnormalities may predict a poorer neurodevelopmental outcome in these children.Because neurodevelopmental outcomes are closely related to the presence of underlying syndromes or genetic abnormalities,early diagnosis is the key to establish the prognosis of it,which is conducive to provide guidance for the children and their families in early time.In order to draw the attention of pediatric clinicians and CHD families and provide more comprehensive support for those children,this brief review is written for illustrate the relationship between CHD children and their neurodevelopmental outcome from the perspective of genetics.

Analysis of gene mutation in children with cardiomyopathy / 实用医学杂志

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.

Diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus in children and adolescents / 中华实用儿科临床杂志

Objective To observe the clinical characteristics and improve the diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus (AAOCA)in children and adolescents. Methods Between January 2012 and December 2016,11 children/ adolescents [8 were boys,3 were girls,age was (10. 1 ± 5. 6) years old]diagnosed with AAOCA were retrospectively reviewed at Department Pediatric Cordiology,Guangdong General Hospital. The clinical features,transthoracic echocardiography (TTE),computed tomography images,electro-cardiogram (ECG),cardinc troponin I(CTnI)and creatinine kinase - MB(CK - MB)were analyzed. Results The di-agnosis of AAOCA was confirmed by TTE and computed tomography images in all 11 cases. Left coronary artery origina-ted from the right sinus in 5 patients,and right artery coronary originated from the left sinus in 6 cases. Five patients with intramural course had the history of exercise - related syncope. Of these 5 cases with exercise - related syncope,4 cases were diagnosed as left coronary artery originated from the right sinus and 1 case was diagnosed as right artery coronary originated from the left sinus. ECG,CTnI and CK - MB revealed acute myocardial ischemia in these patients with exercise - related syncope. Unroofing procedures were undergone in these 5 patients. The remaining 6 patients had no syncope and symptoms of cardia ischemia,sports activities were restricted and follow - ups were counseled in these 6 patients. There was no sudden death events later,and none of the patients demonstrated any evidence of new myocardial ischemia in the follow - up study. Conclusions AAOCA can be associated with syncope and myocardial ischemia in children and adolescents. The correct diagnosis of AAOCA requires a very high index of suspicion. Sports activities restriction and follow - up should be counseled in cases with AAOCA. Unroofing procedures should be performed in symptomatic cases with AAOCA and an intramural course.

Surgical intervention for cardiac neuplasm in fetus / 中华胸心血管外科杂志

Objective To summarize the clinical experience of surgical intervention for cardiac neoplasm in a fetus . Methods A 32-year-old pregnant woman was admitted to our hospital for complaint of fetal cardiac neoplasm .A separated het-erogenic cardiac occupying lesion was identigied at right atrium of the fetus by echocardiography , whose size is 2.85 cm ×2.25 cm, but the pathogenic origin still remained uncertain, maybe originate from ether pericardium or atrium.The annulus of tri-cuspid valve was compressed nearly 50% with the presence of amount of pericardial effusion.The fetal heart rate decreased at some fetal position resulting in the compression to the heart.So an Ex-utero Intrapartum Therapy(EXIT) procedure was per-formed under the supply of placenta at the 32 weeks of pregnancy.Cesarean section was performed with intact umbilicus and fe-tal circulation by obstetricians.Consequently, the median sternotomy of this fetus and pericardiotomy were performed , with 30 ml clear pericardial effusion drained .The tumor was confirmed to be giant right atrial neoplasm after the intraoperative explora-tion.Considering on the high risk of the cardiopulmonary bypass and limited time for EXIT , the giant atrial neoplasm was left alone with delayed sternum closure after the effectively decompression of the heart .The omphalotomy was successfully per-formed after the EXIT surgery.The neoplasm resection and the repair for its defect on right atrium were performed with cardiop-ulmonary bypass 2 days later.Results Convalesce of this mother was quite good after cesarean resetion .Hemodynamics of the premature baby was satisfatory after the resection of right atrial neoplasm which pathological report was benign hemangioma . Conclusion Via multiple disciplines collaboration , EXIT intervention for fetus is feasible and safe under adequate prepara-tion.

Value of transthoracic echocardiography and cardiac multi-slice spiral computed tomography in the diagnosis of partial anomalous pulmonary venous connection in children / 中华实用儿科临床杂志

Objective To make a comparison of transthoracic echocardiography (TTE) and cardiac multi-slice spiral computed tomography (MSCT) in the diagnosis of partial anomalous pulmonary venous connection (PAPVC) in children.Methods One hundred and one patients admitted from February 2008 to April 2014 in Guangdong General Hospital primarily diagnosed with PAPVC with or without other congenital heart diseases were analyzed retrospectively,all the cases underwent routine TTE,which 74 cases underwent cardiac MSCT examination,and the TEE and cardiac MSCT diagnosis results were compared with final surgical findings,then the TTE and cardiac MSCT diagnosis accuracy was calculated.SPSS 13.0 software was used to analyze the data.Results All the patients underwent TTE examination,meanwhile,74 cases received cardiac MSCT examination.Ninety-three cases were confirmed by surgical findings,while 8 cases were misdiagnosed with PAPVC.Sixty-eight cases diagnosed by TTE coincided with surgical findings,and the diagnosis accuracy was 73.12％ (68/93 cases);65 cases diagnosed by cardiac MSCT coincided with surgical findings,and the diagnosis accuracy was 94.20％ (65/69 cases).Among the patients who received both TTE and cardiac MSCT examinations,66 cases were confirmed through final surgical operation,2 cases were mis-diagnosis,so the diagnosis accuracy was 97.06％ (66/68 cases).Conclusions In terms of the diagnosis accuracy of PAPVC,cardial MSCT examination is superior to TTE,as almost all of the cases could be diagnosed by TTE combined with cardial MSCT.In consideration of the acoustics window of children,apart from the conventional parasternal approach of detecting pulmonary veins,the importance of suprasternal and subcostal views should also be attached.

Follow-up results of heart conduct block after transcatheter closure of ventricular septal defect surgery / 实用医学杂志

Objective To study the prevalence and risk factors of heart conduct block after transcatheter closure of ventricular septal defect (VSD)surgery. Methods A total of 1 069 cases underwent transcatheter closure VSD were retrospectively analyzed. The risk factors were assessed by multivariable logistical analysis. Results The median follow-up time was 2.2 (1 to 4.16) years. The early post-procedure heart conduct block was 20.5 %(219 cases), and 35 cases underwent severe conduct block (3.3%). During the follow-up, there were 43 late onset heart conduct block (4.0%), including 4 (0.4%) complete atrioventricular block. Multivariable logistic analysis showed that implanted of asymmetrical occluder from foreign company was the risks factors for early onset severe conduct block, with longer procedure time. Placement of thin-waist-big-side occluder were risk factor for the late onset conduct block. Conclusions Heart conduct block after transcatheter closure VSD is common , light and recovery. The late onset severe conduct block is minor. Symmetrical occluder should be chosen in transcatheter closure VSD if possible.

Analysis on causes of failed procedure of transcatheter closure for ventricular septal defects in children / 中华实用儿科临床杂志

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.

Genetics and pedigree analysis of primary carnitine deficiency cardiomyopathy in 6 cases / 中华儿科杂志

<p><b>OBJECTIVE</b>To investigate the mutation and background of SLC22A5 in 6 patients with primary carnitine deficiency (PCD) who only presented as cardiomyopathy.</p><p><b>METHOD</b>Genomic DNA were abstracted from the blood of the patients and their parents. Using high-throughput sequencing to determine the mutation site.Using Sanger method to confirm the mutated alleles in PCD patients and detect the corresponding sequences in their patients. Using SIFT and PolyPhen to predict the function of protein for detected missense mutations.</p><p><b>RESULT</b>Three different mutations were identified, including 2 nonsense mutations (R254X and R289X), 1 missense mutation (C113Y), R254X was the most frequently seen mutation. Four patients had compound heterozygous mutations and 2 patients had homozygous mutations. Their parents were found to have heterozygous mutations in corresponding alleles.</p><p><b>CONCLUSION</b>R254X, R289X and C113Y might be associated with primary carnitine deficiency.</p>

Efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty (RVP-PBAV) for congenital aortic stenosis (AS).</p><p><b>METHOD</b>A total of sixteen children with AS accepted the treatment with RRVP-PBAV. The patients were at ages 6 months to 15 years, their median age was 5.4 years. Their body weight was between 8.5 and 59.0 kg, average (22.3 ± 16.5) kg. The gradient pressure across the aortic valve was measured for all the patients and aortic regurgitation was observed. The follow-up time ranged from 1 month to 5.5 years.</p><p><b>RESULT</b>All patients underwent RVP-PBAV successfully. The ratios of balloon/valve were 0.86 to 1.12. The gradient pressure varied from preoperative Δp = (96 ± 32) mmHg (1 mmHg = 0.133 kPa) to the immediate postoperative ΔP = (41 ± 26) mmHg, (P < 0.05). One case had postoperative restenosis, and 3 cases were complicated with bicuspid aortic valve deformity.</p><p><b>CONCLUSION</b>The treatment with RVP-PBAV for congenital aortic stenosis is safe and reliable. Rapid ventricular pacing is a safe procedure to stabilize the balloon during balloon aortic valvuloplasty and may decrease the incidence of aortic insufficiency.</p>

Experimental study of bioabsorbable iron-based pulmonary artery stent / 中华实用儿科临床杂志

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.

The clinical experience of childhood cardiomyopathy caused by inborn errors of metabolism in 11 cases / 临床儿科杂志

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.

Evaluation for the clinical effect of duct occluder in treatment of intracristal ventricular septal defect / 中华实用儿科临床杂志

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.

An analyze of medium and long term follow-up of arrhythmias after transcatheter closure of ventricular ;septal defect in children / 临床儿科杂志

Objective To observe and analyze the medium and long term follow-up data of arrhythmias after transcatheter closure of children with ventricular septal defect (VSD). Methods Retrospectively analyzed the clinical data of 1071 children with VSD, who successfully underwent transcatheter device closure, at l, 3, 6, 12 months and ev-ery year post procedure from March 2002 to December 2010. Results Of all 1071 children, 272 cases (25.4%) were ob-served of having arrhythmias within 1 month after intervention, mainly including atrioventricular block (AVB), branch block, junctional tachycardia, atrial and ventricular tachycardia, frequent contractions, etc. Among them, 22 cases (2.1%) had above II degree AVB, complete left bundle branch block (CLBBB) and other causes of serious arrhythmias. After treatment, all cases got better and no permanent pacemaker was necessary. After 1 to 107 months (2.8±1.7 years) fol-low-up, 161 cases (18.2%) were observed of having persistent abnormal ECG mainly caused by AVB and branch block, including 10 cases (1.1%) with serious arrhythmias. In 4 cases with late-onset AVB, 3 cases had already appeared AVB in early postoperative, 1 case had recurrence CLBBB, left ventricle enlarge, and died of heart failure during follow up. Four cases were implanted permanent pacemaker. Conclusion During follow-up, serious arrhythmias after VSD closure, such as AVB or CLBBB, have high risk of recurring. Conduction block arrhythmias may reappear or worsen, while arrhythmias like tachycardia and premature heart rhythm mostly return to normal.

A comparative study of right ventricular outflow tract pacing and right ventricular apical pacing in children / 实用医学杂志

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.