Rational Design of NiCo-borate/GO Heterojunction as a High-Performance Supercapacitor Electrode.

The bottleneck in the preparation of supercapacitors is how to develop high-energy and high-power-density devices by using appropriate materials. Herein, a novel NixCo3-x-B/GO heterostructure material was synthesized through a simple ultrasonic and precipitation method. The prepared NixCo3-x-B/GO heterostructure exhibits significant improvements in supercapacitor performance than NixCo3-x-B. The presence of GO effectively suppresses the excessive growth and accumulation of NixCo3-x-B; therefore, Ni2.7Co0.3-B/GO exhibits the best performance as an electrode material for supercapacitors: a high specific capacitance (Cm, 1789.72 F g-1@1 A g-1) and excellent rate performance. The asymmetric supercapacitor (ASC) device of Ni2.7Co0.3-B/GO//AC exhibits a Cm of 76.6 F g-1@1 A g-1, a large voltage window of 1.6 V, and a high energy density (ED) of 98.0 Wh kg-1. Furthermore, a flexible, all-solid-state supercapacitor assembled with Ni2.7Co0.3-B/GO as both the positive and negative electrodes demonstrates a Cm of 46.9 F g-1@1 A g-1. Even after multiple folding and bending at various angles, the device maintains excellent performance, showcasing remarkable stability. With a power density (PD) of 479.7 W kg-1, the device achieves a high ED of 60.0 Wh kg-1. This work provides valuable insights into the synergistic effects in electrochemical processes based on heterostructure materials.

Ivabradine monotherapy in pediatric patients with focal atrial tachycardia: a single-center study.

This study investigated the efficacy of ivabradine monotherapy in pediatric patients with focal atrial tachycardia (FAT). We prospectively enrolled 12 pediatric patients (7.5 ± 4.5 years; six girls) with FAT who were resistant to conventional antiarrhythmics and received ivabradine as monotherapy. Patients were classified as having tachycardia-induced cardiomyopathy (TIC) if they had a left ventricular ejection fraction (LVEF) of < 50% and a left ventricular end-diastolic dimension (LVDD) z-score of > 2 due to tachycardia. Oral ivabradine was initiated at 0.1 mg/kg every 12 h, increased to 0.2 mg/kg every 12 h if no restoration of stable sinus rhythm was observed after two doses, and discontinued after 48 h if neither rhythm nor heart rate control was observed. Of these patients, six (50%) had incessant atrial tachycardia, and 6 had frequent short episodes of FAT. Six patients were diagnosed with TIC, and their mean LVEF and mean LVDD z-score were 36.2 ± 8.7% (range, 27-48%) and 4.2 ± 1.7 (range, 2.2-7.3), respectively. Finally, six patients achieved either rhythm (n = 3) or heart rate control (n = 3) within 48 h of ivabradine monotherapy. One patient achieved rhythm/heart rate control with ivabradine at a dose of 0.1 mg/kg every 12 h, while the others achieved rhythm/heart rate control at a dose of 0.2 mg/kg every 12 h. Five patients received ivabradine monotherapy for chronic therapy, one (20%) of whom had FAT breakthrough 1 month after discharge, and metoprolol was added. Neither FAT recurrence nor adverse effect (with or without beta-blocker) was observed during a median follow-up of 5 months. CONCLUSION: Ivabradine is well-tolerated and may provide early heart rate control in pediatric FAT and can be considered early, especially in the presence of left ventricular dysfunction. Further investigations are deserved to confirm the optimal dose and long-term efficacy in this population. WHAT IS KNOWN: • Focal atrial tachycardia (FAT) is the most common arrhythmia associated with tachycardia-induced cardiomyopathy (TIC) in children, and the efficacy of conventional antiarrhythmic medications in the treatment of FAT is poor. • Ivabradine is currently the only selective hyperpolarization-activated cyclic nucleotide-gated (HCN) inhibitor, which can effectively low HR without negative effect on blood pressure or inotropy. WHAT IS NEW: • Ivabradine (0.1-0.2 mg/kg every 12 h) can effectively suppress focal atrial tachycardia in 50% of pediatric patients. • Ivabradine provides early control of heart rate and hemodynamic stabilization in children with severe left ventricular dysfunction due to atrial tachycardia within 48 h.

Stenting of branch pulmonary artery stenosis in children: initial experience and mid-term follow-up of the pul-stent.

Not all stents are suitable for children. For instance, premounted stents can be used in infants and small children but cannot dilate with age to accommodate adult-sized pulmonary arteries. Conversely, the Pul-Stent adapts to somatic growth. Thus, our hospital implemented the Pul-Stent in pediatric patients with branch pulmonary artery stenosis. This study summarizes our initial experience with Pul-Stents in this patient population, including the efficacy and safety. We implanted 37 Pul-Stents in 35 patients between August 2014 and June 2015. The patients' mean age and weight at stent implantation were 6.7 ± 3.0 years and 20.9 ± 8.7 kg, respectively. Bench testing revealed that axial shortening of the Pul-Stent was minimal with further dilation, and the radial strength did not change. The stents were successfully deployed in all cases, except two with minor malpositioning. Primarily, 8-12 mm mounting balloons were used for the initial implantation, and a long sheath (8-10 F) was used for delivery. After stent implantation, the minimal lumen diameter in the stenosed segment increased by 50% in 97% (34/35) of patients. Furthermore, the pressure gradient across the stenosed segment decreased by 50% in 77% (23/30) of biventricular patients. One stent fracture and one stent restenosis were noted during the follow-up visits (mean follow-up time: 4.6 ± 1.7 years). Eighteen patients (51%) underwent repeat catheterization; ten had successful redilation. No aneurysms or stent fractures were observed. Our initial results indicate that the Pul-Stent is safe and effective in pediatric patients and can be further dilated over time to accommodate somatic growth. Moreover, the Pul-Stent has good compliance and adequate radial strength to treat pulmonary artery stenosis effectively.

Treatment and prognosis of Scimitar syndrome: A retrospective analysis in a single center of East China.

Background: Scimitar syndrome is a rare congenital cardiovascular malformation; its optimal management remains controversial. This study aims to present the clinical experience of this disease in our center. Methods: We undertook a retrospective review of 34 patients with Scimitar syndrome documented at our institution between January 2013 and December 2018. The patients' clinical characteristics, management, and prognosis data were collected and analyzed. Results: Thirty-four patients, including 16 males and 18 females, were enrolled with a median age at diagnosis of 7 months and a follow-up period of 22.5 months. The infantile form of Scimitar syndrome presents more tendency for pulmonary hypertension (PH), pulmonary vein stenosis (PVS), and mortality than the adult form. Of the 15 patients who underwent surgical correction of the Scimitar vein, four had post-operation PVS. There was no significant difference in the stenosis incidence between baffle repair and Scimitar vein reimplantation groups. Eight patients received interventional catheter therapy, including occlusion of aortopulmonary collateral arteries (APCs) and other intracardiac malformations, without the following surgery. The overall mortality rate was 20.5% (7 of 34) over the study period. High-risk factors of death included age at diagnosis (p = 0.000), PH (p = 0.007) and PVS (p = 0.014). Conclusions: Infantile Scimitar syndrome needs intense suspicion for early diagnosis and multidisciplinary treatment. Interventional treatment of Scimitar syndrome alleviates pulmonary artery pressure and progression during infancy. Baffle repair and direct reimplantation of the Scimitar vein used in the surgical treatment of Scimitar syndrome are safe and have similar effects. Age at diagnosis, PH, and PVS are high-risk factors for death in Scimitar syndrome.

Clinical characteristics and survival of children with hypertrophic cardiomyopathy in China: A multicentre retrospective cohort study.

Background: Few data on paediatric hypertrophic cardiomyopathy (HCM) are available in developing countries. A multicentre, retrospective, cohort study was conducted to profile the clinical characteristics and survival of children with HCM in China. Methods: We collected longitudinal data on children with HCM aged 0-18 years at three participating institutions between January 1, 2010 and December 31, 2019. Patients were identified by searching for the diagnosis using ICD-10 codes from the electronic medical records database. HCM was diagnosed morphologically with echocardiography or cardiovascular magnetic resonance imaging. The exclusion criteria were secondary aetiologies of myocardial hypertrophy. The primary outcomes were all-cause death or heart transplantation. The Kaplan-Meier method was used to estimate the survival rate of different groups. Findings: A total of 564 children were recruited, with a median age at diagnosis of 1.0 year (interquartile range, IQR: 0.4-8.0 years), followed for a median of 2.6 years (1977 patient-years, IQR:0.5, 5.9 years). The underlying aetiology was sarcomeric (382, 67.7%), inborn errors of metabolism (IEMs) (108, 19.2%), and RASopathies (74, 13.1%). A total of 149 patients (26.4%) died and no patients underwent heart transplantation during follow-up. The survival probability was 71.1% (95% confidence interval [CI], 66.3%-75.3%) at 5 years. Patients with IEMs or those diagnosed during infancy had the poorest outcomes, with an estimated 5-year survival rate of 16.9% (95% CI, 7.7%-29.1%) and 56.0% (95% CI, 48.8%-62.5%), respectively. Heart failure was the leading cause of death in the cohort (90/149, 60.4%), while sudden cardiac death was the leading cause in patients with sarcomeric HCM (32/66, 48.5%). Interpretation: There is a high proportion of patients with IEM and a low proportion of patients with neuromuscular disease in children with HCM in China. Overall, mortality remains high in China, especially in patients with IEMs and those diagnosed during infancy. Funding: National Natural Science Fund of China (81770380, 81974029), China Project of Shanghai Municipal Science and Technology Commission (20MC1920400, 21Y31900301).

Drug-coated balloon angioplasty for coronary stenotic lesions in a paediatric patient after Kawasaki disease.

Percutaneous coronary intervention for stenosis of coronary artery after Kawasaki disease presents various challenges. The diameters of reference vessels and femoral artery in children are smaller, and the morphological changes are different from adults. Herein, we describe our successful experience with a severe coronary artery stenosis at the proximal portion of left anterior descending treated with drug-coated balloon dilation.

Rotating machinery fault diagnosis based on a novel lightweight convolutional neural network.

The advancement of Industry 4.0 and Industrial Internet of Things (IIoT) has laid more emphasis on reducing the parameter amount and storage space of the model in addition to the automatic and accurate fault diagnosis. In this case, this paper proposes a lightweight convolutional neural network (LCNN) method for intelligent fault diagnosis of rotating machinery, which can largely satisfy the need of less parameter amount and storage space as well as high accuracy. First, light-weight convolution blocks are constructed through basic elements such as spatial separable convolutions with the aim to effectively reduce model parameters. Secondly, the LCNN model for the intelligent fault diagnosis is constructed via lightweight convolution blocks instead of the tradi-tional convolution operation. Finally, to address the "black box" problem, the entire network is visualized through Tensorboard and t-distribution stochastic neighbor embedding. The results demonstrate that when the number of lightweight convolutional blocks reaches 6, the diagnosis accuracy of the LCNN model exceeds 99.9%. And the proposed model has become the most robust with parameters significantly decreasing. Furthermore, the proposed LCNN model has realized accurate, automatic, and robust fault diagnosis of rotating machinery, which makes it more suitable for deployment under the IIoT context.

GIS Partial Discharge Pattern Recognition Based on a Novel Convolutional Neural Networks and Long Short-Term Memory.

Distinguishing the types of partial discharge (PD) caused by different insulation defects in gas-insulated switchgear (GIS) is a great challenge in the power industry, and improving the recognition accuracy of the relevant models is one of the key problems. In this paper, a convolutional neural network and long short-term memory (CNN-LSTM) model is proposed, which can effectively extract and utilize the spatiotemporal characteristics of PD input signals. First, the spatial characteristics of higher-level PD signals can be obtained through the CNN network, but because CNN is a deep feedforward neural network, it does not have the ability to process time-series data. The PD voltage signal is related to the time dimension, so LSTM saves and analyzes the previous voltage signal information, realizes the modeling of the time dependence of the data, and improves the accuracy of the PD signal pattern recognition. Finally, the pattern recognition results based on CNN-LSTM are given and compared with those based on other traditional analysis methods. The results show that the pattern recognition rate of this method is the highest, with an average of 97.9%, and its overall accuracy is better than that of other traditional analysis methods. The CNN-LSTM model provides a reliable reference for GIS PD diagnosis.

Porcelain Insulator Crack Location and Surface States Pattern Recognition Based on Hyperspectral Technology.

A porcelain insulator is an important part to ensure that the insulation requirements of power equipment can be met. Under the influence of their structure, porcelain insulators are prone to mechanical damage and cracks, which will reduce their insulation performance. After a long-term operation, crack expansion will eventually lead to breakdown and safety hazards. Therefore, it is of great significance to detect insulator cracks to ensure the safe and reliable operation of a power grid. However, most traditional methods of insulator crack detection involve offline detection or contact measurement, which is not conducive to the online monitoring of equipment. Hyperspectral imaging technology is a noncontact detection technology containing three-dimensional (3D) spatial spectral information, whereby the data provide more information and the measuring method has a higher safety than electric detection methods. Therefore, a model of positioning and state classification of porcelain insulators based on hyperspectral technology is proposed. In this model, image data were used to extract edges to locate cracks, and spectral information was used to classify the surface states of porcelain insulators with EfficientNet. Lastly, crack extraction was realized, and the recognition accuracy of cracks and normal states was 96.9%. Through an analysis of the results, it is proven that the crack detection method of a porcelain insulator based on hyperspectral technology is an effective non-contact online monitoring approach, which has broad application prospects in the era of the Internet of Things with the rapid development of electric power.

Head-up tilt test induces T-wave alternans in long QT syndrome with KCNQ1 gene mutation: Case report CARE-compliant article.

INTRODUCTION: Long QT syndrome (LQTS) is a congenital disorder characterized by a prolongation of the QT interval on electrocardiograms (ECGs) and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest, or sudden death. T-wave alternans (TWA) refers to the periodic beat-to-beat alternation of T-wave shape, polarity and amplitude on surface ECG during regular heart rhythm. In this report, a case of long QT syndrome with KCNQ1 gene mutation induced TWA in the head-up tilt test (HUTT), which has not been reported yet. PATIENT CONCERNS: A 6-year-old boy presented with loss of consciousness twice, 5 months in duration. The boy's ECG showed prolonged QT interval (QTc = 600 ms, QTc = QT/RR). During HUTT test, QT interval was significantly prolonged (QTc = 716 ms) based on macroscopic TWA. DIAGNOSIS: The patient was diagnosed with 1. Long QT syndrome type 1(LQT1); 2. Vasovagal syncope (VVS) INTERVENTIONS:: Metoprolol 12.5 mg was given orally twice a day. The child was told avoid standing for a long time and strenuous exercises. OUTCOMES: There was no syncope or arrhythmia occurred during hospitalization and follow-up for 1 year. CONCLUSIONS: VVS may exist in patients with long QT syndrome. Increased sympathetic tone during the early stage of HUTT may induce macroscopic TWA in long QT syndrome with KCNQ1 gene mutation.

Clinical and Molecular Characterization of Infantile-Onset Pompe Disease in Mainland Chinese Patients: Identification of Two Common Mutations.

AIMS: We sought to understand the clinical course and molecular defects of infantile-onset Pompe disease (IOPD) among mainland Chinese patients. MATERIALS AND METHODS: Twenty-five Chinese patients with IOPD were enrolled and clinical data were retrospectively reviewed. The entire coding region of the GAA gene was amplified by polymerase chain reaction and analyzed by direct sequencing. RESULTS: The median age at symptom onset was 3.4 months (range: 1.0-7.1 months) and 4.9 months (range: 2.7-8.3 months) at diagnosis. Only one patient received enzyme replacement therapy (ERT) and this child survived beyond the age of 2 years. Of the 24 patients not receiving ERT, all, but one patient, died at a median age of 8.3 months (range: 4.0-12.2 months). Thirteen novel and two common GAA mutations were identified in this study. The allelic frequency of c.2662G > T (p.Glu888X) was 23.1% in northern Chinese patients and 4.2% in southern Chinese patients, whereas the allelic frequency of c.1935C > A (p.Asp645Glu) was 20.8% in southern and 3.8% in northern Chinese patients. CONCLUSIONS: We identified the most common mutations in southern and northern Chinese patients with IOPD.

Identification of TAZ mutations in pediatric patients with cardiomyopathy by targeted next-generation sequencing in a Chinese cohort.

BACKGROUND: Barth syndrome (BTHS) is a rare X-linked recessive disease characterized by cardiomyopathy, neutropenia, skeletal myopathy and growth delay. Early diagnosis and appropriate treatment may improve the prognosis of this disease. The purpose of this study is to determine the role of targeted next-generation sequencing (NGS) in the early diagnosis of BTHS in children with cardiomyopathy. METHODS: During the period between 2012 and 2015, a gene panel-based NGS approach was used to search for potentially disease-causing genetic variants in all patients referred to our institution with a clinical diagnosis of primary cardiomyopathy. NGS was performed using the Illumina sequencing system. RESULTS: A total of 180 Chinese pediatric patients (114 males and 66 females) diagnosed with primary cardiomyopathy were enrolled in this study. TAZ mutations were identified in four of the male index patients, including two novel mutations (c.527A > G, p.H176R and c.134_136delinsCC, p.H45PfsX38). All four probands and two additional affected male family members were born at full term with a median birth weight of 2350 g (range, 2000-2850 g). The median age at diagnosis of cardiomyopathy was 3.0 months (range, 1.0-20.0 months). The baseline echocardiography revealed prominent dilation and trabeculations of the left ventricle with impaired systolic function in the six patients, four of which fulfilled the diagnostic criteria of left ventricular noncompaction. Other aspects of their clinical presentations included hypotonia (6/6), growth delay (6/6), neutropenia (3/6) and 3-methylglutaconic aciduria (4/5). Five patients died at a median age of 7.5 months (range, 7.0-12.0 months). The cause of death was heart failure associated with infection in three patients and cardiac arrhythmia in two patients. The remaining one patient survived beyond infancy but had fallen into a persistent vegetative state after suffering from cardiac arrest. CONCLUSIONS: This is the first report of systematic mutation screening of TAZ in a large cohort of pediatric patients with primary cardiomyopathy using the NGS approach. TAZ mutations were found in 4/114 (3.5%) male patients with primary cardiomyopathy. Our findings indicate that the inclusion of TAZ gene testing in cardiomyopathy genetic testing panels may contribute to the early diagnosis of BTHS.

Identification of LAMP2 Mutations in Early-Onset Danon Disease With Hypertrophic Cardiomyopathy by Targeted Next-Generation Sequencing.

Danon disease is an X-linked disorder with the clinical triad of cardiomyopathy, skeletal myopathy, and mental retardation. Early diagnosis of this disease remains a challenge, especially in the pediatric population. In this study, we developed a targeted panel-based next generation sequencing pipeline to identify mutations by sequencing of selected candidate genes in 136 pediatric patients with either hypertrophic cardiomyopathy (HC) or idiopathic dilated cardiomyopathy (IDC). This led to the identification of lysosome-associated membrane protein 2 (LAMP2) mutations in 4 of the 64 (6%) probands with HC, including 3 novel nonsense mutations (p.Q240X, p.S250X, and p.G22X). No LAMP2 mutation was detected in the other 72 probands with IDC. All 4 probands and one additional affected family member (2 men and 3 women) had an early-onset age and presented either HC alone or combined with Wolff-Parkinson-White syndrome and skeletal myopathy. Immunofluorescence staining and Western blot analysis revealed absent LAMP2 expression in both cardiac and skeletal muscle samples of the first proband and severely decreased LAMP2 expression in the skeletal muscle samples of the second proband. In conclusion, cardiomyopathy in the patients with Danon disease may occur during early childhood and tend to be HC rather than IDC in both affected men and women. Therefore, Danon disease should be considered as one of the leading causes of unexplained ventricular hypertrophy in pediatric patients. The inclusion of LAMP2 gene in cardiomyopathy genetic screening panels may contribute to early diagnosis of Danon disease.

Clinical Experience of Transcatheter Closure for Residual Ventricular Septal Defect in Pediatric Patients.

BACKGROUND: Open cardiac surgery has traditionally been the gold standard for repair of ventricular septal defect (VSD). The inherent risks and complications associated with open surgery and the incidence of postoperative residual VSD are significant disadvantages of the open surgical approach. OBJECTIVE: To evaluate the methodology and efficacy of transcatheter closure of postoperative residual VSD in children. METHODS: Patients with postoperative VSD who underwent percutaneous device closure in the period from August 2010 and February 2014 in our center were analyzed retrospectively. Twelve children, including 8 males and 4 females, with mean age of 8.13 ± 5.39 years (range 0.67-17 years) and mean weight of 29.63 ± 20.86 kg (range 5.5-66 kg) were included in the study. RESULTS: Ten cases had residual shunts situated at the margin of the patches while the other two had residual shunts because of multiple muscular VSD. The mean VSD diameter was 3.84 ± 1.86 mm (range 2.3-8.7 mm). On cardiac catheterization, the reported mean pulmonary circulatory blood volume/systemic circulation volume (Qp/Qs) ratio was 1.86 ± 0.91 (range 1.1-4.25) and the mean pulmonary artery pressure was 25.92 ± 12.42 mm Hg (range 7-52 mm Hg). Successful transcatheter closure was obtained in all twelve patients with postsurgery residual VSD. Two cases with residual muscular VSD underwent retrograde catheterization via the femoral artery while one case with residual VSD located at the lower margin of the patch was accessed via the internal jugular vein. The mean procedure time was 81.25 ± 25.86 minutes (range 40-120 minutes). There was no evidence of residual VSD, and no instances of new-onset valvular regurgitation were reported, with the only two exceptions being patients who had multiple muscular VSDs where clinically insignificant residual shunts were detected. CONCLUSION: Our retrospective study highlights the potential safety and therapeutic efficacy of transcatheter approach for closing postoperative residual VSD in children. RECOMMENDATIONS: More robust studies with longer-term follow-up of outcomes are required to firmly establish the safety profile and respective indications for use of various occluder devices for treating different categories of residual VSD.

Congenital extrahepatic portosystemic shunt: an underdiagnosed but treatable cause of hepatopulmonary syndrome.

Congenital extrahepatic portosystemic shunt (CEPS) is a rare malformation of the mesenteric vasculature, which may lead to severe complications. In this report, we describe a case series of three children with type II CEPS (presenting as hypoxemia) and hepatopulmonary syndrome (HPS). The first patient was a 4-year-old male who did not receive any specific treatment and subsequently died of brain abscess 5 years after the diagnosis. The second patient was a 10-year-old female with a 5-year history of cyanosis and dyspnea on exertion. She had partial regression of hypoxemia and improved exercise tolerance at 8 months after a surgical shunt closure. The third patient was a 4-year-old male with a 3-year history of cyanosis and decreased exercise tolerance. He had full regression of hypoxemia at 3 months after a transcatheter shunt closure. CONCLUSION: These results indicate that CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences. Closure of portosystemic shunts may result in resolution of HPS in type II CEPS and the length of period for resolution varies depending on the severity of HPS. WHAT IS KNOWN: Congenital extrahepatic portosystemic shunt (CEPS) is a rare cause of hepatopulmonary syndrome (HPS). There have been few reports in the literature about the management and outcome of HPS in children with CEPS. WHAT IS NEW: CEPS may present in children with unexplained hypoxemia, which may lead to devastating clinical consequences. Closure of portosystemic shunts may result in resolution of HPS in type II CEPS.

[Initial experience with the new Pul-Stent in treating postoperative branch pulmonary artery stenosis].

OBJECTIVE: The Pul-stent is a new cobalt alloys stent, specially licensed for pulmonary artery stenosis. The aim of this study was to investigate the value of the stent implantation as the treatment of postoperative pulmonary artery stenosis. METHOD: Clinical practice was carried out to evaluate effectiveness, safety and maneuverability of Pul-stent in the defined clinical setting. Transthoracic echocardiography, chest roentgenography and electrocardiography were carried out as follow-up studies at 1 and 3 months after procedures. RESULT: Ten patients (7 males and 3 females) received Pul-stent implantation in left or right pulmonary arteries (9 stents in left and 1 stent in right). For 9 cases transcatheter stent implantation was performed and in I case hybrid procedure. The median age was 7. 9 years (range 3. 4 - 13. 7 years). The median weight was 22. 3 kg (range 13 - 32 kg). Among them 6 cases were post surgical repair of tetralogy of Fallot, 2 cases were after Fontan procedures, 1 patient was post surgical repair of pulmonary atresia with ventricular septal defect, and 1 patient had stenosis at Glenn pathway. The pressure gradient at the stenosis decreased from (31. 6 ± 10. 2) mmHg(1 mmHg =0. 133 kPa) to (7. 3 ± 10. 3) mmHg, and the diameter of the narrowest segment increased from (4. 0 ± 2. 3) mm to (9. 6 ± 2. 7) mm, the right ventricle to aortic pressure ratio decreased from 0. 54 to 0. 36, all of these improvements were statistically significant (t = 3. 9, -9. 7, 4. 5; P =0. 008, 0. 000, 0. 004). The total procedure time ranged from 55 to 220 min (median 117 min) , and the fluoroscopy time ranged from 9 to 67 min (median 26 min). There were 2 cases of post-stenting pneumorrhagia. No stent fracture, stent malposition and other severe complications were observed. Initial follow-up of 1 and 3-months showed good results with maintenance of improved caliber of the stented vessel, and the gradient across the stenosis area measured by echocardiography was (32. 0 ± 14. 6) mmHg after 3 months. Compared with before stentifng ((40.6 ± 15. 2) mmHg) and 1 month later ((30. 6 ± 13. 6) mmHg), the difference was not statistically significant (t =2. 2,1. 76; P =0. 07, 0. 10). CONCLUSION: Pul-Stent tracking and delivery was excellent, the initial experience has shown that Pul-stent implantation was effective and safe in treating post-operative branch pulmonary artery stenosis. Further follow-up study should be conducted to make sure whether those good results would be kept constant.

[Stent implantation for relief of pulmonary artery branch stenosis].

OBJECTIVE: Branch pulmonary artery stenosis is one of the common congenital heart disease. Stent implantation to relieve branch pulmonary artery stenosis (BPAS) is an alternative to failed surgical or balloon angioplasty. The aim of this study was to explore the indication, methods and complications of using balloon expandable stent placement to treat branch pulmonary artery stenosis, and evaluate the results of stent implantation in the treatment of branch pulmonary artery stenosis. METHOD: From August 2005 to December 2012, 19 patients underwent an attempt at stent implantation. The median age of those patients was 9.1 years (range 4.0-15.0 years). The median weight was 31.7 kg (range 17.0-60.5 kg); 14/19 patients underwent post surgical repair of tetralogy of Fallot, one patient received post surgical repair of pulmonary atresia with ventricular septal defect, one patient underwent post surgical repair of pulmonary atresia with intact septum, one with native left BPAS, and one was after surgical repair of aortopulmonary window and the other truncus arteriosus. CP stent and NuMED Balloon-in-Balloon catheter were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the inner balloon and outer balloon was inflated successively to expand the stent to desired diameter. Statistical analysis was performed with the unpaired Student t test. RESULT: A total of 26 stents were implanted successfully in 19 patients. The systolic gradient across the stenosis fell from a median of (36.0 ± 18.3) to (3.8 ± 3.4) mmHg (P < 0.01, 1 mmHg = 0.133 kPa) and the diameter of the narrowest segment improved from (6.0 ± 1.9) to (11.6 ± 3.1) mm (P < 0.01). The right ventricle to aortic pressure ratio fell from 0.68 to 0.49 (P < 0.01). Complications included the following: two stents were malpositioned in the right ventricular outflow tract and one balloon ruptured when dilated the hole of the stent. No other complications occurred. All patients were followed up for 6 months to 6.0 (2.5 ± 1.8) years. One patient underwent stent re-dilation in order to accommodate somatic growth two years later. CONCLUSION: Balloon expandable stents are safe and effective in relieving BPAS. Stent implantation should be considered the treatment of choice for most patients with BPAS. Stents placed into growing children will require further dilation to keep up with normal somatic growth. Intermediate and long-term follow up studies have shown excellent results after further dilation over time.

[Transcatheter closure of patent ductus arteriosus with the new Amplatzer Duct OccluderII].

OBJECTIVE: To evaluate the indications, methodology and results of the transcatheter closure of patent ductus arteriosus (PDA) with the new Amplatzer Duct Occluder II (ADO-II). METHOD: Totally 51 patients underwent transcatheter closure of PDA with the new ADO-II. The devices were delivered by 4F or 5F sheath through arterial or venous side respectively. The descending aorta angiography and transthoracic echocardiography was performed to evaluate the device position, residual shunt and complications caused by the device during and after implantation. RESULT: Forty-nine patients had successful transcatheter closure of the PDA without significant residual shunts and artery obstruction during the short-term follow-up. One patient received the ADO-II dislodgment and first generation ADO re-implantation for the obvious descending aortal obstruction caused by ADO-II. Another patient had the ADO-II dislodgment and left pulmonary artery shaping surgery, because the ADO-II implantation led to obstruction of the left pulmonary artery. Both the obstructions in these two patients were ameliorated afterwards. CONCLUSION: The transcatheter closure using the ADO-II is safe and effective for the non-window type PDA with a small size.

Nonsymptomatic myocardial injury after radiofrequency and cryoablation: a study of children and patients with congenital heart disease.

The aim of this study was to investigate the degree of myocardial injury following catheter radiofrequency (RF) ablation (RFA) or cryoablation and its clinical significance in children and patients with congenital heart disease. Cardiac troponin T (cTnT) or cardiac troponin I (cTnI), creatine kinase (CK), and its cardiac isoenzyme MB (CK-MB) were measured in 269 patients who underwent catheter ablation (216 RFA, 53 cryoablation) just before the procedure and again 6 hours after the end of the procedure. Follow-up studies included echocardiography and 12 lead electrocardiographics (ECGs). No clinical, ECG, nor ECG signs of ischemia were detected. Biomarkers were increased in 57.7-75.5 %. A linear regression analysis illustrated the ablation target site and the number of RF applications as a function of higher cTnI and cTnT levels, with the maximum increase due to ventricular ablation and higher numbers of RF applications. No significant difference in cTnT levels after RFA or cryoablation were observed for AV nodal reentrant tachycardia procedures and no significant differences were observed after nonirrigated tip or irrigated tip RFA in atrial wall or ventricular wall ablation. Elevations in both troponin T and troponin I levels were commonly observed after ablation, especially in ventricular wall ablation as well as with increasing numbers of radiofrequency applications. However, unlike in patients with acute coronary syndrome, these elevated levels had no specific significance. Reference values for each ablation target site were proposed in order to potentially detect additional subclinical injuries to the coronary arteries.

[Efficacy of the transcatheter closure of perimembranous and muscular ventricular septal defects with the Amplatzer duct occluder II].

OBJECTIVES: To evaluate the feasibility and efficacy of transcatheter closure of perimembranous ventricular septal defects (pmVSD) with aneurysmatic formation and muscular ventricular septal defects (mVSD) with Amplatzer duct occluder II. METHODS: This retrospective analysis included 48 cases received transcatheter closure of pmVSD aneurysmatic formation or mVSD from February 2011 to March 2012 in our hospital (42 pmVSD with aneurysmatic formation and 6 mVSD). Median age was 5.2 years (range: 1.8 - 15 years), and median weight was 20.2 kg (range: 12 - 44 kg). Amplatzer duct occluder II was selected depending on the condition of ventricular septal defect. The device was implanted by antegrade or retrograde approach. Complications such as residual shunt, valvular regurgitation and arrhythmia were evaluated by echocardiography or angiography. Median follow-up was 9.5 months (range: 1 - 13 months). RESULTS: The mean ratio of pulmonary (Qp) to systemic (Qs) blood flow was 1.35 ± 0.15 before transcatheter closure. The diameter of exit hole of ventricular septal defects was (2.46 ± 0.53) mm measured by transthoracic echocardiography, and (2.35 ± 0.40) mm by angiography. Successful implantation of the device was achieved in 46 patients (96%) and unsuccessful in two cases due to acute aortic insufficiency. Forty-two (92%) patients were closed successfully, and trivial residual leak was evidenced in four patients and remained unchanged during follow-up. One patient with mVSD still had trivial residual shunt at 6 months post procedure. New trivial tricuspid insufficiency was observed in 1 patient (2.1%) during follow-up. Two patients developed procedural related left anterior fascicular block and remained unchanged during follow-up. CONCLUSIONS: pmVSD with aneurysm and mVSD could be successfully treated with Amplatzer duct occluder II. However, the long waist and large disc of the device could interfere with tricuspid valve function and cause tricuspid insufficiency.