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Introduction: The people worldwide have been affected by severe acute respiratory syndrome-coronavirus 2 (SARS-CoV-2) infection since its appearance in December, 2019. Kawasaki disease-like hyperinflammatory shock associated with SARS-CoV-2 infection in previously healthy children has been reported in the literature, which is now referred to as a multisystem inflammatory syndrome in children (MIS-C). Some aspects of MIS-C are similar to those of Kawasaki disease, toxic shock syndrome, secondary hemophagocytic syndrome, and macrophage activation syndrome. Case Presentation: This study reported an 11-year-old boy with MIS-C presented with periorbital and peripheral edema, abdominal pain, elevated liver enzymes, severe right pleural effusion, moderate ascites, and severe failure of right and left ventricles. Conclusions: Due to the increasing number of reported cases of critically ill patients afflicted with MIS-C and its life-threatening complications, it was recommended that further studies should be carried out in order to provide screening tests for myocardial dysfunction. Adopting a multidisciplinary approach was found inevitable.

Objectives: This study compared the success rate of atrial septal defect device closure in children with and without balloon sizing during the procedure. Methods: This four-year, combined retrospective and prospective study was performed at a single center. All participants underwent transcatheter closure under transthoracic echocardiography and the success rate was analyzed considering the effect of balloon sizing. Results: Eighty-eight patients with a mean age of 6.48 ± 3.32 years were enrolled. Balloon sizing was performedor in 39 (44%) patients. It didn’t change the success rate compared to 49 (56%) patients without balloon sizing. Likewise, age and sex had no effect on thesuccess rate. However; the failure rate was higher in patients with larger defects. Conclusions: The study demonstrated that balloon sizing did not influence the success rate of atrial septal defect device closure, and a precise measurement by echocardiography can be as successful as balloon sizing.

Treadmill exercise testing is a crucial diagnostic tool for evaluating congenital and acquired heart disease in the pediatric population. This study aimed to perform a comprehensive evaluation of exercise-induced electrocardiographic (ECG) changes in children. Although there are numerous studies on exercise testing in various cardiac pathologies, studies on exercise-induced ECG changes in normal children with coverage of all ECG parameters of atrial and ventricular depolarization and repolarization are very scant, if any.This study aimed to investigate the exercise-induced ECG changes in healthy children and evaluate the effects of gender and four different formulas of heart rate correction of Bazett, Fridericia, Framingham and Hodges on ventricular repolarization parameters pre-and post-exercise.Between April 2019 and April 2020, all children with normal electrocardiogram, echocardiogram and exercise test, high-quality ECG tracings and consent for participation were enrolled in this prospective study. Twenty electrocardiographic parameters were measured and 25 indices were calculated.Seventy-four healthy children were studied. Amplitudes of P, S, and T waves increased significantly after the exercise. All durations, except P wave time to peak and T peak -T end /QT (Tp-e/QT) interval decreased significantly with exercise. Generally, the parameters of ventricular repolarization were not statistically significant between males and females. There were significant differences among the heart-rate corrected values of intervals of QTc, QoTc, JTc, J point to peak T and Tp-e/QTc by various formulas. There was no U wave either at pre-exercise or post-exercise. QT interval was shortened by 24.6 % ± 12.1 % with exercise. The ECG-derived estimated duration of mechanical systole and diastole decreased with exercise. The percentage of decrease in diastole was more than systole (43.79 %± 13.31% versus 33.74% ±15.79 %, respectively,Diastolic time decreased more than systolic time with exercise and systolic time to diastolic time increased with exercise. Hodges' and Fridericia's formulas resulted in the longest and shortest QT and QoT, JT, and JTP, respectively. Thus, using a single value as the cut-off for long QT syndrome can lead to under or over-diagnosis. Nomograms incorporating data on age, heart rate, and heart rate correction formula are indispensable for accurate long QT diagnosis. Furthermore, gender differences in ventricular repolarization parameters are not generally present in 5 to 14-year-old healthy children. The lack of U wave in this study may implicate the need for more careful investigation in the presence of U wave in the treadmill exercise testing of healthy children.

COVID-19 as a viral infection is usually asymptomatic in children, but complication of this disease in children is not rare and may be fatal Hyper inflammation of COVID-19 is a potential fatal in undiagnosed children It is very similar to Kawasaki disease but with higher mortality and morbidity Multisystem inflammatory syndrome in children (MIS-C) and multi-organ involvement was reported in hyper inflammation syndrome following COVID19 Herein we report our algorithmic approach to Kawasaki-like syndromes due to COVID-19 in our center Based-on this approach we had no mortality during the last 5 months [ABSTRACT FROM AUTHOR] Copyright of Iranian Journal of Pediatrics is the property of Kowsar Corporation and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission However, users may print, download, or email articles for individual use This abstract may be abridged No warranty is given about the accuracy of the copy Users should refer to the original published version of the material for the full abstract (Copyright applies to all Abstracts )

Objective: Previous studies have demonstrated that both children and adult patients with a history of congenital heart disease (CHD) are at high risk for coronavirus disease 2019 (COVID-19) infection. This study investigates the status of COVID-19 infection among children undergoing surgical repair within the past 2 years.Methods: All alive patients operated on in a tertiary referral center between March 2018 and March 2020 were recruited in the present study. Detailed demographics, past medical and surgical history, and physical examination were reviewed for each patient. During the COVID-19 pandemic, data regarding the patient's status were collected by telephone survey from April 15 to April 30, 2020.Results: A total number of 210 patients are analyzed in this study. Participants' median age was 21.59 months [interquartile range (IQR) = 12–54.67], and 125 (59.5%) were female. The median interval between surgery and COVID-19 assessment was 305 days (IQR = 215–400). In addition, 67 (32%) patients used angiotensin receptor blocker (ARB)/angiotensin-converting enzyme (ACE) inhibitor (spironolactone and/or captopril). Sixteen patients (7.6%) were symptomatic and had positive chest CT results and/or RT-PCR compared to the previously reported prevalence of COVID-19 among the pediatric population (2.4% of children with p = 0.00012). Two patients were admitted to the intensive care unit (ICU); one patient was discharged 2 weeks later with acceptable status, and one patient died 2 days after ICU admission due to cardiac and respiratory arrest and myocarditis. The complexity of the underlying cardiac disorders was not different between patients with low risk (p = 0.522), suspicious patients (p = 0.920), and patients positive for COVID-19 (p = 0.234). The ARB/ACE inhibitor consumption was not associated with the COVID-19 infection [p = 0.527, crude odds ratio (OR) = 1.407, 95% CI = 0.489–4.052].Conclusion: Children with a history of previous CHD surgery are more susceptible to infections, especially those infections with pulmonary involvements, as the lung involvement could cause worsening of the patient's condition by aggravating pulmonary hypertension. The results of the current study indicate that these patients are more prone to COVID-19 infection compared to the healthy children population.

Background: A large proportion of infants with congenital heart defects (CHD) suffer from malnutrition. The aim of this study was to assess the impact of using energy- and nutrient-dense formula on weight gain in malnourished infants with CHD before surgery. Methods: This was a one arm, open label intervention study, conducted in the pediatric cardiology department of children’s medical center, Teheran, Iran. 49 infants with moderate/high risk cardiac defects who were undernourished (WFA z-score ≤ - 2) and were candidates for surgical repair were enrolled in this study. Infants on regular infant formula were switched completely to energy- and nutrient-dense formula. The infants were evaluated for growth parameters (weight and length) at enrolment and every 3 weeks thereafter at the center for a period of 12 weeks. Results: The average head circumference was 37.1+2.8 cm at baseline and 39.0+3.46 cm at the final assessment (p=0.079). The mean MUAC increased from 11.25+1.39 cm at baseline to 14.75+2.06 cm at the final assessment (p=0.001). The average intake of energy- and nutrient-dense formula was 148.24+214.737 Kcal/day (36.23+ 52.49 Kcal/kg/day) at study start and 455.4+177.21 Kcal/day (98.61 ± 38.27 Kcal/kg/day) at the end of the study. The WFA z-score improved significantly on each visit during the follow-up interval (pConclusion: The results of the current study highlight the efficacy of preoperative feeding with energy- and nutrient dense formula in improving weight gain of malnourished infants with CHD.reference number: IR.TUMS.VCR.REC.1396.3543

Recent achievements in the genetic diagnosis of Dilated Cardiomyopathy (DCM) have disclosed rare variants in numerous genes encoding different types of myocardial proteins. However, the causative gene underlying the pathogenesis of about 60% of familial cases with DCM has not been identified. One novel gene introduced in 2016 for cardiac-restricted DCM is FLNC. In this study, we applied Whole Exome Sequencing (WES) and bioinformatics-based methods to a member of an extended non-consanguineous family with DCM history accompanied with fatal arrhythmia in at least four consecutive generations. We found a novel splice-site mutation in FLNC gene (c.2389+1G>A) which cosegregated with all symptomatic individuals in the family. Computational prediction software tools as well as RT-PCR method were used to evaluate the impact of the FLNC splice site mutation. This substitution leads to exon 15th donor-site disruption and exon skipping, which would result in a premature stop codon three aminocids downstream of the mutation site. The aberrantly mRNA transcript can induce nonsense-mediated mRNA decay. Although carrier individuals show remarkable variable expression regarding the severity of DCM as well as the disease age of onset, a highly penetrant fatal arrhythmia was found to be shared between them. We strongly suggest that the involvement of FLNC gene, due to haploinsufficiency, should be considered in familial cases with DCM, especially if accompanied with arrhythmia and increased incidence of sudden cardiac death.

Background: To normally grow and develop, children with congenital heart diseases (CHD) in the hypermetabolic state need 50% more energy than healthy ones. Objectives: This study aimed to investigate the prevalence of malnutrition and its association with other factors among the non-syndromic acyanotic patients with CHD undergoing palliative or corrective surgery. Methods: A retrospective study of medical records of 812 children with acyanotic congenital heart disease undergoing palliative or corrective surgery was performed from March 2011 to March 2017. Collected data consisted of the age at the time of the surgery, gender, weight at the time of the surgery, height at the time of the surgery, birth weight, length of ICU stay, and 1-year mortality. The WAZ, WLZ, LAZ, and BMI Z-scores were calculated based on World Health Organization (WHO) standards. We classified the Z-scores into 4 groups, including normal (Z-scores > -1), mild (-2 to > -1), moderate (-3 to > -2), and severe (< -3) malnutrition groups. Results: Total amount of 812 patients, 375 (46.2%) were female and 437 (53.8%) male, participated in this study. The average age, weight, and height at the time of the surgery were 23.52 + 29.38 months, 9.086 + 6.71 kilograms, and 76.93 + 23.37 centimeters, respectively. Moreover, 68.7% and 66.4% of the patients suffered from mild to severe forms of malnutrition based on WAZ and WLZ, respectively (P value < 0.0001, 0.025). There was no significant difference between males and females in WAZ, WLZ, LAZ and, BMIZ (P value: 0.154, 0.265, 0.965, and 0.667, respectively). Generally, in acyanotic defects, we found that more severe malnutrition causes an increment in length of ICU stay (P value = 0.001) and lesser birth weight has a significant relationship with the severity of growth failure in infancy or childhood (P value < 0.0001). Conclusions: In our study, the high prevalence of the malnutrition and related complications during and after surgical repair shows the importance of nutritional support and interventions in patients with CHD.

Objectives: To evaluate fetal cardiac changes in gestational diabetic mothers, compared to healthy ones by means of different indices and to determine which index can first represent the alterations. Methods: The study was conducted as an observational cross-sectional study, including 25 pregnant women with gestational diabetes as the cases and 50 healthy pregnant women as the controls. The preload index, left and right side myocardial performance index (MPI), Interventricular septal hypertrophy, the left and right side cardiac output were assessed in all the patients. Results: The gestational ages were 31.65 ± 8.02 and 31.64 ± 5.37 weeks in case and control group respectively, without any significant difference. Both of the left and right ventricular MPI did not differ statistically between the case and controls. The cases had a greater Interventricular septal hypertrophy but the cardiac output was similar. The preload index was higher in the fetuses of the gestational diabetic mothers. Conclusions: In our study, the MPI did not show any difference between the fetuses of the gestational diabetic mothers and non-diabetic ones; but, fetuses of gestational diabetic mothers had a greater value of PLI, representing early diastolic function changes in right heart even before the overt heart failure occurred. This could be a sign of vasculopathy in gestational diabetic mothers.

We report on a novel mutation in a two-year-old child with autosomal recessive cutis laxa with severe generalized laxity of the skin, prematurely aged appearance, conjunctival chalasia, episodes of severe rectal prolapse, isolated right ventricular non-compaction, (RVNC), significant pulmonary hypertension at the systemic arterial pressure level, severe tricuspid regurgitation, corpulmonale secondary to recurrent pulmonary infections, and mixed pulmonary fibrosis and emphysema. Next generation sequencing of cutis laxa genes identified a novel homozygous mutation in the FBLN5 gene (homozygous sequence alteration of c.907C > T [p. Gin303*] FBLN5 [ENST00000342058]). Despite severe and generalized disorder in the patient’s connective tissues, she had no primary valvar or vascular abnormalities in the heart. Complete speckle-tracking strain imaging (SI) by two-dimensional echocardiography showed decreased systolic longitudinal and transverse strain in the involved segment of the right ventricle (RV).

Background: Myocardial damage is a common complication in patients with Duchenne muscular dystrophy (DMD) that occurs due to myocardial replacement by fat and fibrosis. In recent years, efforts have been made toward finding new pharmacological agents with fewer complications which can be used as prophylactic before the symptoms. Coenzyme Q10 plays a central role in production of bioenergy in heart muscle and antioxidant in reperfusion condition of myocardial damaged muscle and leads to membrane stability and prevents cell death. Objective: This study aimed at comparing the Effectiveness of coenzyme Q10 on echocardiographic parameters of pediatric patients with Duchenne muscular dystrophy. Methods: This randomized clinical trial study (RCT) was carried out on 25 pediatric patients with pre-diagnosed DMD who attended the Children’s Medical Center (CMC), Tehran, Iran from February 2013 to 2015. The patients were randomly divided into two groups. Group-1; (n=12) was treated with coenzyme Q10 for six months and group-2 ;(n=13) received placebo for the same time. The primary aim was to compare the myocardial performance index (MPI), between the two groups at the end of six months. Data were analyzed by SPSS software (ver-16) and using T-Test. Results: Twenty-five patients under study were divided into two groups of (Q10=12) and (placebo=13). Mean ages were 8.9±1.7 and 8.6±1.4 in Q10 and placebo groups (P=0.66). No significant difference was detected in MPI at all three views of mitral and tricuspid and septum respectively in two groups after the end of treatment (0.41±0.13, and 0.43±0.6; P=0.59), (0.45±0.12, and 0.46±0.1; P=0.05), and (0.45±0.06, and 0.45±0.1; P=0.31). Conclusion: According to the results obtained from this study, coenzyme Q10 had no significant effect on improving the performance of echocardiographic parameters in patients with DMD. Trial registration: The trial is registered at the Iranian Clinical Trial Registry (IRCT.ir) with the IRCT identification number IRCT2015070223018N1. Funding: This research has been financially supported by the Research Council of Tehran University of Medical Sciences.

Relatively high prevalence of dilated cardiomyopathy in children, unfavorable response to traditional drug therapy, and limitations in heart transplantation call for new therapeutic options. Stem cell therapy can be promising in children suffering from this disease. The presented case documents that intracoronary injection of autologous bone marrow-derived mesenchymal stem cells in a boy with progressive dilated cardiomyopathy is feasible and safe. Furthermore, it may positively influence functional class, quality of life, and echocardiographic indices of cardiac function.

The Immediate-Release Patch is the newest version of the Transcatheter Patch, which is a bioabsorbable device for the closure of cardiac defects. Closure of 12 atrial septal defects and 1 fenestration was attempted using this device. Of the 13 devices used, 12 were implanted successfully (including that for the fenestration), whereas 1 device moved from the original position and was retrieved percutaneously. No other major adverse events occurred. After a median follow-up period of 11 months, trivial (≤ 2-mm) residual shunts remained in two patients, and a significant residual defect (7 mm) remained in 1 patient. The major advantages of this device include its wirelessness, its bioabsorbablity, potential application for defects up to 30 mm using only three sizes, its easier retrieval, and its ability to occlude defects with a deficient rim and some ostium primum and sinus venosus type defects. In contrast to the metal devices, it is bulkier and has a different application method requiring operator familiarity, and it sometimes leaves residual shunts.

A male infant with high-output heart failure who had been found to have a hepatic arteriovenous malformation by ultrasound imaging was referred to our center (Department of Diagnostic and Interventional Radiology, Imam Khomeini Hospital, Tehran University of Medical Sciences, Islamic Republic of Iran) for further evaluation. Computed tomography angiography revealed a large hepatic arteriovenous malformation with feeders originating from enlarged hepatic arteries and draining to enlarged hepatic veins. We performed a transcatheter embolization of the anomaly using ethylene vinyl alcohol copolymer (Onyx) during a single session. The cardiac function of the infant rapidly improved after the procedure. Over the 19 months of follow-up, his cardiac output remained stable and within the normal limits, and no complications were detected.

Objective Atrial septal defect (ASD) device closure is routinely done under the guide of transesophageal or intracardiac echocardiography which are expensive techniques and not easily affordable in developing countries. Methods Using metallic devices, we attempted 32 ASD device closures under transthoracic echocardiography. Findings Of those, 30 procedures were successful (94 %). In two patients with relatively large ASD we encountered difficulty in positioning the device. These patients were referred for surgical closure. Conclusion ASD device closure can be carried out successfully in most patients under transthoracic echocardiography in situations where transesophageal or intravenous echocardiographies are not available or affordable.

Closure of perimembranous ventricular septal defects in patients with Down syndrome, either surgically or by Amplatzer occluders, carries a high risk of complete heart block. We report 5 closures using the transcatheter patch, a wireless bioabsorbable device without any reported heart block to date. The median defect size was 11 mm. Small devices were used in 4 patients and a medium size in 1. The patch was released after 48 hours in 3 patients and immediately in the other 2. Of the 5 patients, 3 were followed up for >5 years and 2 for 1 year. None of these patients had atrioventricular block during their follow-up. In conclusion, the transcatheter patch might be superior in terms of cardiac conduction system protection in patients with Down syndrome after ventricular septal defect closure.