Using 2D speckle-tracking echocardiography to localize the accessory pathway and evaluate cardiac function and dyssynchrony in pediatric patients with Wolf-Parkinson-White syndrome.

Wolf-Parkinson-White (WPW) accessory pathway (AP) may be associated with reentry supraventricular tachycardia (SVT) in addition to ventricular dyssynchrony and cardiac dysfunction. Electrophysiological studies (EPS) are the gold standard for the localization of the AP; however, 2D speckle-tracking echocardiography (2D-STE) may help in the localization of the AP noninvasively. Our study aims to evaluate the capability of 2D-STE for AP localization and the identification of AP-related contractile abnormalities and dyssynchrony in pediatric patients with WPW syndrome. This prospective multicenter cohort study involved 18 pediatric patients with ventricular preexcitation from January 2021 to January 2023. Tissue Doppler imaging (TDI), conventional echocardiography, and 2D-STE were done. Myocardial velocities, myocardial performance index (MPI), the global and segmental longitudinal strain of the left ventricle (LV), and time-to-peak longitudinal strain (TPLS) were measured before and after ablation. The longitudinal strain of the LV segments supplied by the AP, or the nearby segments close to the AP, was significantly impaired and improved after ablation (P = 0.0001). The abnormal strain pattern in the affected segments could predict the location of the AP. The TPLS of the affected segments significantly increased after ablation (P = 0.0001), denoting improved dyssynchrony. The ejection time and the LV MPI measured at the basal septum improved significantly after ablation. CONCLUSIONS: 2D STE may be used for noninvasive localization of the AP and to evaluate cardiac function and dyssynchrony in patients with WPW. Further research on more patients is necessary to validate this method for AP localization. WHAT IS KNOWN: • Accessory pathways (AP) associated with the Wolf-Parkinson-White (WPW) syndrome have been linked to supraventricular tachycardia (SVT). Even without SVT, WPW can cause left ventricular dyssynchrony, contractile dysfunction, and cardiomyopathy. • Electrophysiology study is the gold standard for the localization of the AP in WPW syndrome. WHAT IS NEW: • The combination of 2D-speckle-tracking echocardiography (2D-STE) and the modified Arruda algorithm can precisely localize the AP associated with WPW syndrome. • 2D-STE can potentially assess cardiac function and dyssynchrony related to WPW syndrome. Additionally, 2D-STE can be utilized to evaluate the effectiveness of ablation in restoring cardiac function and dyssynchrony.

Cardiac repolarization abnormalities in children with familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever (FMF) is an autoinflammatory disease that can have conduction disturbances and cardiac rhythm disorders as manifestations of cardiac involvement. The aim of the study is to assess the susceptibility of children with FMF to cardiac repolarization abnormalities and therefore arrhythmia in children with FMF. METHODS: A cross sectional study conducted on 60 children had FMF and 40 age and sex matched healthy controls. Cardiac repolarization markers, cardiac dimensions and functions were assessed by electrocardiogram (ECG) and conventional echocardiography in patients and controls. RESULTS: The mean ± SD age of the patients was 10.43 ± 3.472 years, corrected QT (QTc) and the ratio of peak to end T wave (Tpe) over QTc interval (Tpe /QTc) increased significantly in FMF patients more than healthy control (p value 0.023 and 0.022 respectively). P wave dispersion (Pd) was significantly higher in FMF patients with amyloidosis (p value 0.030). No significant difference was found in cardiac dimensions and functions between the two groups. We found a statistically negative correlation between Pd and age of patients at time of study, age of disease onset and age at diagnosis. On the other hand, we found a statistically significant positive correlation between Pd with number of attacks per year and disease severity score. Furthermore, Tpe/QTc ratio correlated with FMF 50 score, QTc correlated with 24 hours proteinuria. QT, JT intervals correlated with fibrinogen. CONCLUSIONS: FMF Patients may have increased risk of arrhythmia and should be monitored on regular basis. Compliance to colchicine therapy and better disease control might play a role in decreasing this risk.

Margin between success and failure of PDA stenting for duct-dependent pulmonary circulation.

OBJECTIVES: Percutaneous patent ductus arteriosus (PDA) stenting is a therapeutic modality in patients with duct-dependent pulmonary circulation with reported success rates from 80-100%. The current study aims to assess the outcome and the indicators of success for PDA stenting in different ductal morphologies using various approaches. METHODS: A prospective cohort study from a single tertiary center presented from January 2018 to December 2019 that included 96 consecutive infants with ductal-dependent pulmonary circulation and palliated with PDA stenting. Patients were divided according to PDA origin into 4 groups: Group 1: PDA from proximal descending aorta, Group 2: from undersurface of aortic arch, Group 3: opposite the subclavian artery, Group 4: opposite the innominate/brachiocephalic artery. RESULTS: The median age of patients was 22 days and median weight was 3 kg. The procedure was successful in 78 patients (81.25%). PDA was tortuous in 70 out of 96 patients. Femoral artery was the preferred approach in Group 1 (63/67), while axillary artery access was preferred in the other groups (6/11 in Group 2, 11/17 in Group 3, 1/1 in Group 4, P <0.0001). The main cause of procedural failure was inadequate parked coronary wire inside one of the branch of pulmonary arteries (14 cases; 77.7%), while 2 cases (11.1%) were complicated by acute stent thrombosis, and another 2 cases with stent dislodgment. Other procedural complications comprised femoral artery thrombosis in 7 cases (7.2%). Patients with straight PDA, younger age at procedure and who had larger PDA at pulmonary end had higher odds for success (OR = 8.01, 2.94, 7.40, CI = 1.011-63.68, 0.960-0.99, 1.172-7.40,respectively, P = 0.048, 0.031,0.022 respectively). CONCLUSIONS: The approach for PDA stenting and hence the outcome is markedly determined by the PDA origin and morphology. Patients with straight PDA, younger age at procedure and those who had relatively larger PDA at the pulmonary end had better opportunity for successful procedure.

Early onset left ventricular remodeling in juvenile systemic lupus erythematosus; Insight from 3-dimensional speckle tracking echocardiography.

BACKGROUND: Early diagnosis and treatment of myocardial affection in patients with systemic lupus erythematosus (SLE) are crucial. OBJECTIVES: To evaluate the ventricular systolic function in juvenile-onset systemic lupus erythematosus (j-SLE) patients by 3-D speckle tracking echocardiography (3D-STE) and to determine the predictors of left ventricular (LV) dysfunction if present. METHODS: Twenty-six SLE patients without heart failure and 21 healthy controls were studied by standard echocardiogram and 3D-STE. Conventional parameters included LV ejection fraction (EF), fractional shortening (FS), and mitral annular plane systolic excursion (MAPSE). Global LV strain (GLS) and global area strain (GAS) were obtained by 3D-STE. Medical records, including diagnosis criteria, duration of disease, and SLE disease activity index (SLEDAI) were evaluated. RESULTS: The mean age was similar in patients and controls 11.42 vs 11.48 years p = 0.93. The mean duration of the disease was 1.87 ± 1.02 years and SLEDAI ranged from 0 to 9. By conventional and tissue Doppler imaging echocardiography, only MAPSE was significantly lower in SLE patients compared to controls (14.56 vs 18.46 mm, p < 0.001). By 3D speckle tracking echocardiography, GLS and GAS were significantly reduced in SLE patients compared to controls (-15.07 vs -19.9.4%, -34.6% vs -39.7%, respectively, p < 0.001). Multiple linear regression and ROC analyses indicated that the SLEDAI score was the only predictive factor for the left ventricular remodeling. CONCLUSIONS: These results indicate that early subclinical LV dysfunction occur in jSLE patients even with normal EF and SLE disease activity might be a potential driver for LV deformation.

Functional recovery of cardiomyopathy induced by atrial tachycardia in children: Insight from cardiac strain imaging.

AIM: To evaluate systolic and diastolic cardiac function in children who had cardiomyopathy induced by ectopic atrial tachycardia (EAT). METHODS: Twenty-two pediatric patients who had cardiomyopathy induced by EAT and 25 age-matched controls were recruited in this case-control study. The patients were examined after rhythm control and normalization of their left ventricular systolic function. Different echocardiographic modalities including tissue Doppler imaging and two-dimension speckle tracking echocardiography were utilized to assess the ventricular and atrial function. RESULTS: The patients' median age was 51 months (interquartile range: 28.5-84 months). The median time interval required for normalization of left ventricular ejection fraction (EF) among patients was 1.5 months (interquartile range: 1.5-2.12 months). Compared to controls, patients had a significantly higher median left ventricular myocardial performance index (MPI) at the interventricular septum (0.44 vs. 0.38, p = .001) and left ventricular lateral wall (0.46 vs. 0.32, p = .0001). The median right ventricular MPI of the patients' group was significantly higher when compared to the control group (0.34 vs. 0.26, p = .0001). The median right atrial (RA) reservoir function in patients was significantly reduced compared to controls (30% vs. 36.63%, p = .007). CONCLUSIONS: Shortly after rhythm normalization and restoration of left ventricular EF, using tissue Doppler imaging and two-dimension speckle tracking echocardiography, children who had cardiomyopathy induced by EAT continue to have left ventricular diastolic dysfunction, right ventricular dysfunction, and reduced RA reservoir function.

Transcatheter Closure of Perimembranous Ventricular Septal Defects Using Different Generations of Amplatzer Devices: Multicenter Experience.

OBJECTIVES: To demonstrate safety and efficacy of using different generations of softer Amplatzer™ devices for ventricular septal defect (VSD) closure to avoid serious complications at follow-up. BACKGROUND: Transcatheter closure of perimembranous ventricular septal defects (PmVSD) is a well-established procedure; however, it is associated with unacceptable incidence of complete heart block. Great advantages have been achieved by using softer devices for VSD transcatheter closure. The first and second generation of Amplatzer™ occluders (AVP II, ADO, and ADO II) seem to offer a safe and attractive alternative for this procedure. These devices can be delivered using either an arterial (retrograde) or venous (prograde) approach. METHODS AND RESULTS: Patients with congenital PmVSD who underwent transcatheter closure using ADO, ADO II, and AVP II devices were included. Primary end point was to determine efficacy and safety of these generations of devices and to determine the incidence of complications at follow-up (complete AV block and aortic/tricuspid/mitral regurgitation). One hundred and nineteen patients underwent VSD closure at a median age of 5 years (8 months-54 years). During the catheterization, there were only minor complications and at follow-up of 36 ± 25.7 months (up to 99 months), the closure rate was high of 98.3% and freedom from AV block was 100%. CONCLUSIONS: The use of softer Amplatzer™ devices is a good alternative to achieve PmVSD closure safely with no risk of AVB during the procedure or at midterm follow-up.

Evolution of ventricular function in children with permanent right ventricular pacing after tetralogy of Fallot repair: A midterm follow-up.

OBJECTIVE: We aim to evaluate the midterm effect of chronic apical right ventricular (RV) pacing on right and left ventricular (LV) function using different modalities of echocardiography including conventional echocardiography, tissue Doppler imaging and two-dimension speckle tracking echocardiography. METHODS: This case-control study enrolled 49 patients divided into two groups: a paced group and a nonpaced group. The paced group included 23 patients that underwent tetralogy of Fallot (TOF) repair and required permanent pacemaker insertion for postoperative complete heart block. The nonpaced group included 26 patients that had TOF repair at the same period. RESULTS: The median age for the paced and nonpaced groups was 6 and 8 years, respectively (P = .169). The follow-up duration after TOF surgical repair was 4 years for the paced patients and 5 years for nonpaced patients (P = .411). In the nonpaced group, the QRS duration increased and LV global longitudinal strain (GLS) decreased significantly with increasing duration after TOF repair, P value was .006 and .042, respectively. In the paced group, tricuspid annular systolic plane excursion (TAPSE) was significantly correlated with age (r = .578; P = .004) and duration following TOF correction (r = .724; P < .001). CONCLUSION: Chronic RV apical pacing in children after TOF repair was associated with better clinical status, preservation of RV systolic function, and prevention of progressive QRS prolongation. RV pacing was not associated with progressive deterioration of LV systolic function with increasing the time interval following TOF repair. Therefore, RV pacing can be beneficial in corrected TOF patients presenting with RV failure.

Echocardiographic findings in children with chronic kidney disease.

Cardiovascular diseases (CVD) are considered major cause of morbidity and mortality among children with chronic kidney disease (CKD). This study aims to determine the incidence of CVD in children with CKD, to analyze risk factors and early predictors for late onset atherosclerosis. Thirty-five CKD children [25 on regular hemodialysis (HD) and 10 on conservative management] were evaluated clinically. Left ventricular (LV) functions and carotid artery intima-media thickness (c-IMT) were assessed using conventional echocardiography, pulsed wave Doppler (PWD) and tissue Doppler imaging (TDI). There was decreased E/A ratio and increased E/E' ratio in 66% and 77% of patients, respectively signifying diastolic cardiac dysfunction. There was a significant correlation between increased A' value (peak late diastolic annular velocity) and both increased serum cholesterol and anemia (P = 0.009, 0.004 respectively). Serum high density lipoprotein (HDL) significantly correlated negatively with inter-ventricular septal thickness and LV end-diastolic dimensions (P = 0.05, 0.02, respectively) and positively with E' value (peak early diastolic annular velocity) (P = 0.04). Abnormal c-IMT correlated significantly with HD duration (correlation coefficient = 0.428, P = 0.01) and with both increased serum cholesterol and decreased serum HDL (P = 0.021, 0.031, respectively). Diastolic dysfunction and abnormal LV dimensions are present in patients with CKD even those on conservative management. TDI appears to be more impressive than PWD in assessing early myocardial dysfunction. Increased c-IMT and dyslipidemia are prevalent in patients with CKD and more prevalent in patients on HD.

Is sofosbuvir/ledipasvir safe for the hearts of children with hepatitis C virus?

BACKGROUND: Symptomatic bradycardia has been reported in adults treated for chronic hepatitis C using sofosbuvir based regimens. AIM: We studied the cardiac safety of sofosbuvir/ledipasvir in Egyptian children, treated for chronic hepatitis C. METHODS: The study included 40 hepatitis C virus infected children and adolescents 12-17 years old, using the combination of sofosbuvir (400 mg)/ledipasvir (90 mg) in a single oral tablet (Harvoni) taken daily for 12 weeks. All subjects underwent a baseline standard 12-lead surface Electrocardiography that was repeated at 4 and 12 weeks of therapy. Electrocardiography parameters (Heart Rate, RR interval, PR interval, QRS, QT interval, corrected QT interval, QT dispersion, JT interval, corrected JT interval, JT dispersion, Tpeak-Tend interval) were compared at the 3 different time points during antiviral therapy. RESULTS: No symptoms related to the cardiovascular system were reported during treatment. There were no cases of symptomatic bradycardia/syncope. Heart rate was noted to be significantly lower and RR and QT intervals were significantly longer in the baseline electrocardiography. Heart rate was significantly lower and RR interval was significantly longer in patients with higher viral load. CONCLUSION: No adverse cardiovascular events were observed in this group of HCV infected children and adolescents treated with sofosbuvir/ledipasvir. None of the patients developed bradyarrhythmias during treatment.

Off-label use of duct occluder devices to close hemodynamically significant perimembranous ventricular septal defects: A multicenter experience.

OBJECTIVE: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc. BACKGROUND: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique. METHODS: From 2010 to 2016, 222 patients (female 47.7%) were identified from databases of five participating institutions in whom pmVSD closure was attempted using an Amplatzer Duct Occluder I or Lifetech duct occluder device. RESULTS: Patients ranged in age from 0.7 to 52 years (median, 7.0 years) and in weight from 4.0 to 70 kg (median, 18.0 kg). The mean size of the VSD was 6.8 ± 2.2 mm. A large defect (> 6 mm) was present in 137 patients (61.7%). Device closure was successful in 218 patients (98.2%). The 10/8 mm device was used in most patients (n = 85, 38.3%), and the vascular approach was from the femoral vein in 169 patients (76.1%). There were 18 early complications in 17/218 patients (7.8%). Three patients (1.4%) developed complete heart block (transient n = 2; requiring permanent pacing n = 1). Median follow-up was 6 months (6 months-6 years). A mild residual shunt was seen in 10 patients at 6 months follow-up. CONCLUSIONS: The immediate results of transcatheter pmVSD closure using a duct occluder device with a single retention disc are promising. It is an effective technique with a lower rate of complications than for other currently available devices.

Multicenter Off-Label Use of Nit-Occlud Coil in Retrograde Closure of Small Patent Ductus Arteriosus.

We studied the safety and efficacy of closing patent ductus arteriosus by Nit-Occlud coils via retrograde approach. This is a retrospective study of 46 attempts to close ducts by this method in two hospitals in Egypt and Iran. Ductus arteriosus was crossed by left or right Judkins or endhole catheters. The coil was delivered via the same catheter or the provided endhole catheter after exchange. The procedure was successful in 42 out of 46 attempts. Fluoroscopy and procedural times were significantly shorter when the catheter was not exchanged. This method is effective and safe for the closure of small ducts. Crossing the duct and delivering the coil by a left Judkins catheter is the easiest and fastest way to perform this method.

The prevalence of congenital heart defects in infants with cholestatic disorders of infancy: a single-centre study.

BACKGROUND: There is deficiency of data about congenital heart defects (CHDs) in cholestatic disorders of infancy other than Alagille syndrome (AGS). We aimed to define the prevalence and types of CHDs in infants with various causes of cholestatic disorders of infancy. METHODS: This cross-sectional study was conducted on 139 infants presenting with cholestasis whether surgical or non-surgical. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt. Full examination and investigations were done in an attempt to reach an aetiologic diagnosis for cholestasis, in addition to a comprehensive echocardiographic study. RESULTS: The age at the onset of cholestasis ranged from 1 day to 7 months. Males constituted 61.2%. Biliary atresia (BA) was diagnosed in 39 patients (28%), AGS in 16 patients (11.5%), 27 patients had miscellaneous diagnoses and 57 cases had indeterminate aetiology. CHDs were detected in 55 patients (39.5%). Shunt lesions were detected in 24 patients (43.6%), pulmonary stenosis in 18 patients (32.7%) and combined lesions in 9 patients (16.4%). Three patients (5.5%) had abnormal cardiac situs. Only seven patients had clinical presentation suggestive of CHD. CHDs were detected in 14 patients with BA (35.9%), 15 patients with AGS (93.7%) and 26 patients in the remaining group (30.9%). CONCLUSION: CHDs are not uncommon among cholestatic infants and are mostly asymptomatic. Echocardiographic examination of cholestatic infants is recommended particularly for patients with BA before undergoing hepatic portoenterostomy as presence of CHD may impact the anaesthetic planning and affect the outcome of hepatobiliary surgery.

Skeletal and cardiac muscle involvement in children with glycogen storage disease type III.

UNLABELLED: Glycogen storage disease type III (GSD III) may present with hepatic disease or may involve both skeletal and cardiac muscles as well. To assess the prevalence of neuromuscular and cardiac involvement in a group of children with GSD III, 28 children with GSD III, diagnosed by enzymatic assay, were enrolled in the study after an informed consent was obtained from their parents/guardians and after the study protocol was approved by our institutional ethical committee. Their mean age was 6.6 + 3.1 years. All cases were assessed neurologically by clinical examination, electromyography (EMG), and nerve conduction velocity. The heart was examined clinically by electrocardiogram and echocardiography. Seventeen patients (61 %) had myopathic changes by EMG, three of them had associated neuropathic changes. Creatine phosphokinase (CPK) was elevated in all myopathic cases except one. Children with myopathic changes were significantly older (p = 0.02), and CPK was significantly higher (p < 0.0001). Nine cases had left ventricular (LV) hypertrophy, seven of them had myopathic changes by EMG. CONCLUSION: Myopathic changes are not uncommon in children with GSD III. Myopathic changes tend to occur in older age and are associated with higher CPK level. Cardiac muscle involvement is less common in this age group and may, on occasion, occur alone without skeletal muscle involvement. Despite mild degrees of affection in this age group, it is recommended to perform prospective annual screening using EMG and echocardiography in order to augment dietary therapy regimen to prevent progression to life threatening complications.

Permanent cardiac pacing in pediatric patients.

Pediatric pacemaker (PM) implants comprise less than 1 % of all PM implants. This study aimed to investigate permanent cardiac pacing among the pediatric population, identifying different indications and complications of pediatric cardiac pacing, especially focusing on the effect of the pacing sites, the PM lead type, and the indications for pacing. The current work is a cross-sectional study of 103 procedures for permanent PM insertion in pediatric patients between January 2001 and December 2010. The patients were followed up 1, 3, and 6 months after implantation, then every 6 months or as needed. Evaluation included routine clinical examination, electrocardiography, chest X-ray, echocardiography, and a full analysis of the pacing system measurements. The ages of the patients ranged from 0.09 to 12 years (median, 2.3 years). The most common indication for pacing was postoperative complete heart bock, noted in 54 patients (52.4 %). Transvenous endocardial PM insertion was performed in 92 procedures (89.3 %), whereas transthoracic epicardial insertion was performed in 11 procedures (10.7 %). The most common site of pacing was the right ventricular apex (n = 64, 62 %), followed by the right ventricular outflow tract (n = 25, 24.3 %). Transthoracic epicardial PM insertion was associated with a significantly higher percentage and greater severity of complications. In this study, 65 % of the patients with left ventricle (LV) dilation before pacing showed a significant improvement in LV dimensions and function after pacing. This was noted only in those with endocardially inserted PM leads in both the congenital and the postoperative groups regardless of the pacing site. Endocardial PM insertion in children is a safe procedure with fewer complications and a lower ventricular threshold than the epicardial route. Permanent single-chamber right ventricle pacing is safe and can lead to significant improvement in LV function and dimensions. However, long-term follow-up assessment is needed for further evaluation.