Prevalence of electrocardiographic abnormalities among Iranian children and adolescents and associations with blood pressure and obesity: findings from the SHED LIGHT study.

BACKGROUND: There are few studies for detecting rhythm abnormalities among healthy children and adolescents. The aim of the study was to investigate the prevalence of abnormal electrocardiographic findings in the young Iranian population and its association with blood pressure and obesity. METHODS: A total of 15084 children and adolescents were examined in a randomly selected population of Tehran city, Iran, between October 2017 and December 2018. Anthropometric values and blood pressure measurements were also assessed. A standard 12-lead electrocardiogram was recorded by a unique recorder, and those were examined by electrophysiologists. RESULTS: All students mean age was 12.3 ± 3.1 years (6-18 years), and 52% were boys. A total of 2900 students (192.2/1000 persons; 95% confidence interval 186-198.6) had electrocardiographic abnormalities. The rate of electrocardiographic abnormalities was higher in boys than girls (p < 0.001). Electrocardiographic abnormalities were significantly higher in thin than obese students (p < 0.001), and there was a trend towards hypertensive individuals to have more electrocardiographic abnormalities compared to normotensive individuals (p = 0.063). Based on the multivariable analysis, individuals with electrocardiographic abnormalities were less likely to be girls (odds ratio 0.745, 95% confidence interval 0.682-0.814) and had a lower body mass index (odds ratio 0.961, 95% confidence interval 0.944-0.979). CONCLUSIONS: In this large-scale study, there was a high prevalence of electrocardiographic abnormalities among young population. In addition, electrocardiographic findings were significantly influenced by increasing age, sex, obesity, and blood pressure levels. This community-based study revealed the implications of electrocardiographic screening to improve the care delivery by early detection.

Reference percentiles for tri-ponderal mass index and its association with general and abdominal obesity among Iranian children and adolescents: A report from the SHED LIGHT study.

BACKGROUND: The body mass index (BMI) has some limitations in identifying obesity, particularly among children and adolescents. OBJECTIVES: We sought to determine the reference percentiles of tri-ponderal mass index (TMI) for Iranian population, and its relation to BMI, waist circumference (WC), and waist circumference-to-height ratio (WHtR). METHODS: Baseline characteristics for 14 641 children and adolescents were applied. The area under the characteristics (AUC) curve and optimal cut-off points for TMI were used to define the accuracy of TMI for discriminating general and central obesity compared to other anthropometric measurements. RESULTS: TMI levels remained stable from 6 to 18 years with a small range of difference. The highest AUCs for discriminating obesity according to BMI and WHtR ≥0.5 from non-obese subjects were related to TMI (0.980) and TMI (0.912), respectively, and these findings were similar for both sexes. Moreover, the AUC for TMI to discriminate central obesity by WHtR was higher than that for BMI (0.912 vs. 0.833, p < .001), and this difference was similar among sex and age groups. The optimal value of TMI for discriminating general obesity, obesity by WC, and obesity by WHtR were 15.2 (sensitivity 96.2 and specificity 89.8), 15 (sensitivity 86.9 and specificity 79.9), and 13.9 (sensitivity 85.1 and specificity 81.5), respectively. CONCLUSIONS: TMI discriminated central obesity by WHtR more accurately than obesity by BMI among Iranian children and adolescents irrespective of sex. Besides it remained relatively constant across age- and sex-specific groups providing a single optimal cut-off point for screening obesity during childhood and adolescence.

Ventricular tachycardia ablation in children.

INTRODUCTION: The ablation of ventricular tachycardia, including premature ventricular contractions, is an approved, albeit infrequent procedure in pediatric patients. Data are scarce regarding the outcomes of this procedure. The purpose of this study was to share a high-volume center experience and patient outcomes for catheter ablation of ventricular ectopy and ventricular tachycardia in pediatric population. METHODS: Data were retrieved from the institutional data bank. Outcomes over time were evaluated, and procedural details were compared. RESULTS: A total of 116 procedures were performed on 102 pediatric patients between July 2009 and May 2021 at the Rajaie Cardiovascular Medical and Research Center in Tehran, Iran. Ablation was not performed in 4 procedures (3.4%) due to high-risk substrates. Of the remaining 112 ablations performed, 99 (88.4%) were successful. However, one patient died due to a coronary complication. There were no significant differences observed in early ablation results based on patients' age, sex, cardiac anatomy, or ablation substrates (P > 0.05). Follow-up records were available for 80 procedures, and 13 (16.3%) of those experienced recurrence. During long-term follow-up, none of the variables mentioned above were statistically different between patients with or without arrhythmia recurrence. CONCLUSION: The overall success rate of pediatric ventricular arrhythmia ablation is favorable. We found no significant predictor for the procedural success rate concerning acute and late outcomes. Larger multicenter studies are needed to elucidate the predictors and outcomes of the procedure.

Association Between Obesity and Blood Pressure Among Iranian Children and Adolescents: A Sub-analysis from the SHED LIGHT Study.

Childhood obesity has become a major non-communicable disease worldwide. It is associated with an increased risk of cardiometabolic factors, including diabetes and hypertension (HTN). The purpose of this study was to evaluate the association between obesity and HTN among Iranian children and adolescents. Cross-sectional data from the SHED LIGHT study performed in Tehran urban area were used in this report. The anthropometric values and blood pressure were analyzed. The obesity status was identified based on body mass index (BMI), waist circumference (WC), and waist-to-height ratio (WHtR). The blood pressure status was defined using percentiles for height, age, and sex. A total of 14,641 children with a mean age of 12.28 ± 3.1 years (6-18) were assessed, and 52.8% of them were boys. The prevalence of HTN was higher among obese compared to healthy weight subjects (p < 0.001). HTN had the strongest association with the central obesity by WC (odds ratio [OR] 4.098, 95% confidence interval [CI] 3.549-4.732), generalized obesity by BMI (OR 3.000, 95% CI 2.749-3.274), and central obesity by WHtR (OR 2.683, 95% CI 2.451-2.936). Moreover, parental university education, having studied in private schools, and the smaller number of household children increased the risk of obesity. The rate of HTN was high among children and adolescents with generalized and central obesities. HTN, elevated blood pressure, boy gender, and socioeconomic status were associated with obesity, emphasizing on the importance of screening and implementing lifestyle changes to decrease future risk of cardiovascular diseases.

Pocket-sized echocardiography for screening structural heart disease: diagnostic accuracy and cost-effectiveness for population-based studies.

BACKGROUND: The standard transthoracic echocardiography has some limitations in emergent and community-based situations. The emergence of pocket-sized ultrasound has led to influential advancements. METHODS: In this prospective study, in the hospital-based phase, children with suspected structural heart diseases were enrolled. In the school-based phase, healthy children were randomly selected from six schools. All individuals were examined by experienced operators using both the standard and the pocket-sized echocardiography. RESULTS: A total of 73 individuals with a mean age of 9.9 ± 3.2 years in the hospital-based cohort and 143 individuals with a mean age of 12.8 ± 2.9 years in the school-based cohort were examined. The agreements between the standard and the pocket-sized echocardiography were good or excellent for major CHDs in both cohorts (κ statistics > 0.61). Among valvular pathologies, agreements for tricuspid and pulmonary valves' regurgitation were moderate among school-based cohorts (0.56 [95% confidence interval 0.12-1] and 0.6 [95% confidence interval 0.28-0.91], respectively). The agreements for tricuspid and pulmonary valves' regurgitation were excellent (>0.9) among hospital-based population. Other values for valvular findings were good or excellent. The overall sensitivity and specificity were 87.5% (95% confidence interval 47.3-99.7) and 93.8% (95% confidence interval 85-98.3) among the hospital-based individuals, respectively, and those were 88% (95% confidence interval 77.8-94.7) and 68.4% (95% confidence interval 56.7-78.6) among the school-based individuals, respectively. The cost of examination was reduced by approximately 70% for an individual using the pocket-sized device. CONCLUSIONS: When interpreted by experienced operators, the pocket-sized echocardiography can be used as screening tool among school-aged population.

Accuracy of Doppler-derived indices in predicting pulmonary vascular resistance in children with pulmonary hypertension secondary to congenital heart disease with left-to-right shunting.

This study aimed to evaluate the accuracy of Doppler echocardiography-derived indices in children with pulmonary hypertension secondary to congenital heart disease with left-to-right shunting. Doppler-derived indices including the acceleration time corrected (AcTc), deceleration time corrected, deceleration index, peak velocity, heart-rate-corrected inflection time (InTc), and a new index (the acceleration slope [Acc = peak flow velocity/AcTc]) were measured from the pulmonary artery (PA) systolic flow curve before and after 100 % oxygen administration in the main, left, and right PAs of 33 children. The acquired data were compared between low and high pulmonary vascular resistance (PVR) groups and between responders and nonresponders to the vasoreactivity test. The AcTc values differed significantly between the low and high PVR groups before and after oxygen administration in the main (P = 0.032 and <0.001, respectively), right (P = 0.011 and <0.001, respectively), and left (P < 0.001 and <0.001, respectively) PAs. The AcTc cutoff point in the main PA was 3.44 before oxygen administration (81% sensitivity and 91% specificity). The InTc in the main PA and its changes differed significantly between the low and high PVR groups before and after oxygen administration and between the responders and nonresponders (P = 0.016, 0.046, and 0.021, respectively). The velocity changes of the PA in the main PA differed significantly between the responders and nonresponders to oxygen administration (P < 0.001). The Acc and its changes differed significantly between the low and high PVR groups after oxygen administration and between the responders and nonresponders to oxygen administration (P = 0.044 and 0.006, respectively). Doppler echocardiographic examination using PA systolic flow indices in addition to PA reactivity testing is a promising technique for assessing PVR in children with congenital heart disease.

Ventricular Septal Defect and Mitral Regurgitation Due to Penetrating Cardiac Trauma; a Case Report and Review of Literature.

Penetrating cardiac trauma is a fatal condition and can result in the injury of various parts of the heart. Ventricular Septal Defect (VSD) following these traumas occurs only in 1-5% of cases. The patients' conditions depend on location, size, and concomitant injuries. One of the uncommon coincidences with the VSD is Mitral Regurgitation (MR) due to injury to sub-valvular structures. In this study, we report a case of concomitant traumatic-induced VSD and MR in a 14-year-old boy following a stab wound to his chest. The patient was a teenage boy coming to the Rajaei Cardiology Hospital emergency room following a stab wound to the anterior and left part of his chest. Despite primary urgent surgery, his breathlessness had continued for three more months. Evaluations with Transthoracic Echocardiography (TTE) revealed VSD with concomitant MR, but there was no papillary muscle rupture. Cardiac Magnetic Resonance Imaging (MRI) and angiographic evaluation confirmed the provisional diagnosis. The Amplatzer VSD occluder repaired the VSD, and the patient was discharged following the resolution of his symptoms. Although the MR has been present in the follow-up echocardiography, the patient has been asymptomatic. Since the initial presenting symptoms and signs of VSD and MR might be subtle or delayed, imaging modalities such as TTE and Transesophageal Echocardiogram (TEE) are beneficial in determining the diagnosis and the optimal treatment.

Whole-exome sequencing reveals a likely pathogenic LMNA variant causing hypertrophic cardiomyopathy.

OBJECTIVE: We studied the clinical and molecular features of a family with hypertrophic cardiomyopathy (HCM). BACKGROUND: A very heterogeneous disease affecting the heart muscle, HCM is mostly caused by variants in the proteins of sarcomeres. The detection of HCM pathogenic variants can affect the handling of patients and their families. METHODS: Whole-exome sequencing (WES) was performed to assess the genetic cause(s) of HCM in a consanguineous Iranian family. RESULTS: Missense likely pathogenic variant c.1279C>T (p.Arg427Cys) within exon 7 of the LMNA gene (NM_170707) was found. The segregations were confirmed by polymerase chain reaction-based Sanger sequencing. CONCLUSIONS: Variant c.1279C>T (p.Arg427Cys) in the LMNA gene seemed to have been the cause of HCM in the family. A few LMNA gene variants related to HCM phenotypes have been recognized so far. Identifying HCM genetic basis confers significant opportunities to understand how the disease can develop and, by extension, how this progression can be arrested. Our study supports WES effectiveness for first-tier variant screening of HCM in a clinical setting.

Long-term outcome of interventional approaches for treatment of coronary artery fistulas: a retrospective cohort study in a great referral center.

BACKGROUND: Despite the spontaneous regression of many cases of coronary artery fistulas (CAFs), symptomatic patients or cases with severe shunting may require therapeutic interventions. In the present study, we aimed to assess the outcome of treatment of CAFs using interventional approaches. METHODS: This retrospective cohort study was performed on 29 patients with CAFs that were referred to our tertiary center between 2009 and 2019. Baseline characteristics were collected by reviewing the hospital files, and the patients were followed up to assess long-term outcomes with a mean follow-up of 3.3 years. RESULTS: Overall, in 29 patients in our cohort study, 82.9% suffered from isolated CAFs and in the remaining cases, concurrent congenital abnormalities did exist. For treatment, coils (Cook, Pfm, Ev3) were used in 79.3%, ADO II(AGA) in 18.3%, vascular plug (AGA) in 3.4%, and a combination of coil/ vascular plug/amplatzer in 3.4%. Postoperative complications were reported in 4 patients as external iliac artery thrombosis, transient PSVT, ST-T wave changes and mild pericardial effusion that were all managed successfully with no adverse sequels. No coronary artery injury, device dislocation, dissection, ischemia or coronary dilatation occurred, and there was no death. As larger fistulas were treated by a retrograde approach through the right side of the heart, there was significant correlation between residual shunts and the mode of closure approach; the majority of the residual shunts occurred in patients in the retrograde approach group. CONCLUSIONS: Trans-catheter approach for treating CAFs leads to appropriate long-term outcome with minimal potential side effects.

Comparison of Machine Learning Algorithms Using Manual/Automated Features on 12-Lead Signal Electrocardiogram Classification: A Large Cohort Study on Students Aged Between 6 to 18 Years Old.

PROPOSE: An electrocardiogram (ECG) has been extensively used to detect rhythm disturbances. We sought to determine the accuracy of different machine learning in distinguishing abnormal ECGs from normal ones in children who were examined using a resting 12-Lead ECG machine, and we also compared the manual and automated measurement using the modular ECG Analysis System (MEANS) algorithm of ECG features. METHODS: Altogether, 10745 ECGs were recorded for students aged 6 to 18. Manual and automatic ECG features were extracted for each participant. Features were normalized using Z-score normalization and went through the student's t-test and chi-squared test to measure their relevance. We applied the Boruta algorithm for feature selection and then implemented eight classifier algorithms. The dataset was split into training (80%) and test (20%) partitions. The performance of the classifiers was evaluated on the test data (unseen data) by 1000 bootstrap, and sensitivity (SEN), specificity (SPE), AUC, and accuracy (ACC) were reported. RESULTS: In univariate analysis, the highest performance was heart rate and RR interval in the manual dataset and heart rate in an automated dataset with AUC of 0.72 and 0.71, respectively. The best classifiers in the manual dataset were random forest (RF) and quadratic-discriminant-analysis (QDA) with AUC, ACC, SEN, and SPE equal to 0.93, 0.98, 0.69, 0.99, and 0.90, 0.95, 0.75, 0.96, respectively. In the automated dataset, QDA (AUC: 0.89, ACC:0.92, SEN:0.71, SPE:0.93) and stack learning (SL) (AUC:0.89, ACC:0.96, SEN:0.61, SPE:0.99) reached best performances. CONCLUSION: This study demonstrated that the manual measurement of 12-Lead ECG features had better performance than the automated measurement (MEANS algorithm), but some classifiers had promising results in discriminating between normal and abnormal cases. Further studies can help us evaluate the applicability and efficacy of machine-learning approaches for distinguishing abnormal ECGs in community-based investigations in both adults and children.

A novel stop-gain pathogenic variant in FLT4 and a nonsynonymous pathogenic variant in PTPN11 associated with congenital heart defects.

BACKGROUND: Congenital heart defects (CHDs) are the most common congenital malformations, including structural malformations in the heart and great vessels. CHD complications such as low birth weight, prematurity, pregnancy termination, mortality, and morbidity depend on the type of defect. METHODS: In the present research, genetic analyses via whole-exome sequencing (WES) was performed on 3 unrelated pedigrees with CHDs. The candidate variants were confirmed, segregated by PCR-based Sanger sequencing, and evaluated by bioinformatics analysis. RESULTS: A novel stop-gain c.C244T:p.R82X variant in the FLT4 gene, as well as a nonsynonymous c.C1403T:p.T468M variant in the PTPN11 gene, was reported by WES. FLT4 encodes a receptor tyrosine kinase involved in lymphatic development and is known as vascular endothelial growth factor 3. CONCLUSIONS: We are the first to report a novel c.C244T variant in the FLT4 gene associated with CHDs. Using WES, we also identified a nonsynonymous variant affecting protein-tyrosine phosphatase, the non-receptor type 11 (PTPN11) gene. The clinical implementation of WES can determine gene variants in diseases with high genetic and phenotypic heterogeneity like CHDs.

Impact of donor-to-recipient weight ratio on the hospital outcomes of pediatric heart transplantation.

BACKGROUND: Identifying the factors that can influence the prognosis and final outcomes of pediatric heart transplantation is important and makes it possible to prevent complications and improve outcomes. Coordination of donor characteristics with the recipient in terms of sex, weight, body mass index (BMI), and body surface area (BSA) is an important factor that can influence the outcome of the transplantation. There is still no consensus regarding the role of discrepancy in anthropometrics between donors and recipients. The aim of this study was to investigate the relationship between donor and recipient weight mismatch on the early outcomes of pediatric heart transplantation. In this historical cohort study, 80 children who had underwent heart transplantation for the first time between 2014 and 2019 in Shahid Rajaie Cardiovascular Medical and Research Center in Tehran, Iran, were enrolled and divided into three groups according to donor-to-recipient weight ratio (0.8 < D/RW ≤ 1.5, 1.5 < D/RW ≤ 2.5, and 2.5 < D/RW). The early outcomes of transplantation, during the first post-transplant month, including right heart failure, renal failure, graft rejection, inotrope dependency, duration of intubation, length of ICU stay, death and requiring extracorporeal membrane oxygenation, were recorded through reviewing patient records. RESULTS: Median donor-to-recipient BSA ratio was directly associated with higher vasoactive-inotropic score (P = 0.038), while no significant association was found between donor-to-recipient weight ratio and vasoactive-inotropic score (P = 0.07). No significant relationship was found between other outcomes and donor-to-recipient weight ratio or donor-to-recipient BSA ratio. CONCLUSIONS: Patients who require heart transplantation may also benefit from mismatch donors, especially in those with significant cardiomegaly.

Prevalence of Structural Heart Diseases Detected by Handheld Echocardiographic Device in School-Age Children in Iran: The SHED LIGHT Study.

Background: Structural heart disease (SHD) has great impacts on healthcare systems, creating further public health concerns. Proper data are scant regarding the magnitude of the affected population by SHD. Objectives: This study aimed to determine the prevalence of SHD among children and adolescents in an Iranian population. Methods: In this population-based study, a multistage cluster-random sampling was used to choose schools from the Tehran urban area. All students were examined using a handheld Vscan device by echocardiographer, and the results were concurrently supervised and interpreted by cardiologists. All the major findings were reevaluated in hospital clinics. Results: Of 15,130 students (6-18 years, 52.2% boys) who were examined, the prevalence of individuals with congenital heart disease (CHD) and cardiomyopathy was 152 (10.046 per 1,000 persons) and 9 (0.595 per 1,000 persons), respectively. The prevalence of definite and borderline rheumatic heart disease (RHD) was 30 (2 per 1,000 persons) and 113 (7.5 per 1,000 persons), correspondingly. Non-rheumatic valvular heart disease (VHD) was also detected in 465 (30.7 per 1,000 persons) students. Of all the pathologies, only 39 (25.6%) cases with CHD and 1 (0.007%) cases with RHD had already been diagnosed. Parental consanguinity was the strongest predictor of CHD and SHD (odds ratio [OR]: 1.907, 95% CI, 1.358 to 2.680; P < 0.001 and OR, 1.855, 95% CI, 1.334 to 2.579; P < 0.001, respectively). The female sex (OR, 1.262, 95% CI, 1.013 to 1.573; P = 0.038) and fathers' low literacy (OR, 1.872, 95% CI, 1.068 to 3.281; P = 0.029) were the strongest predictors of non-rheumatic VHD and RHD, correspondingly. Conclusions: The implementation of echocardiographic examinations for detecting SHD among young population is feasible which detected SHD prevalence in our population comparable to previous reports. Further studies are required to delineate its economic aspects for community-based screening.

Predictors of Prolonged Mechanical Ventilation in Pediatric Patients After Cardiac Surgery for Congenital Heart Disease.

BACKGROUND: The duration of mechanical ventilation (MV) is one of the most important clinical factors which predict outcomes in pediatric cardiac surgery. The prolonged mechanical ventilation (PMV) following cardiac surgery is a multifactorial phenomenon and there are conflicts regarding its predictors in pediatric population between different centers. OBJECTIVES: The current study aimed to describe PMV predictors in patients undergoing cardiac surgery for congenital heart disease in a tertiary center for pediatric cardiovascular diseases in Iran. PATIENTS AND METHODS: From May to December 2014, all pediatric patients (less than a month - 15 years old) admitted to pediatric Intensive Care Unit (PICU) after congenital heart surgeries were consecutively included. The PMV was defined as mechanical ventilation duration more than 72 hours as medium PMV and more than seven days as extended PMV. The demographic data and variables probably related to PMV were recorded during the PICU stay. RESULTS: A total of 300 patients, 56.7% male, were enrolled in this study. Their mean age was 32 ± 40 months .The median duration (IQR) of MV was 18 hours (8.6 - 48 hours). The incidence of PMV more than 72 hours and seven days was 20% and 10.7%, respectively. Younger age, lower weight, heart failure, higher doses of inotropes, pulmonary hypertension, respiratory infections and delayed sternal closure were independent predictors of PMV in multivariate analyses. CONCLUSIONS: The results of this study indicated that PMV predictors could be specific for each center and a good administration program is needed for each pediatric cardiac surgery center for the preoperative management of patients undergoing congenital heart surgeries.

Enzyme Polymorphism in Warfarin Dose Management After Pediatric Cardiac Surgery.

BACKGROUND: Warfarin is an anticoagulant and is widely used for the prevention of thromboembolic events. Genetic variants of the enzymes that metabolize warfarin, i.e. cytochrome P450 2C9 (CYP2C9) and vitamin K epoxide reductase (VKORC1), contribute to differences in patients' responses to various warfarin doses. There is, however, a dearth of data on the role of these variants during initial anticoagulation in pediatric patients. OBJECTIVES: We aimed to evaluate the role of genetic variants of warfarin metabolizing enzymes in anticoagulation in a pediatric population. PATIENTS AND METHODS: In this prospective cohort study, 200 pediatric patients, who required warfarin therapy after cardiac surgery, were enrolled and divided into two groups. For 50 cases, warfarin was prescribed based on their genotyping (group 1) and for the remaining 150 cases, warfarin was prescribed based on our institute routine warfarin dosing (group 2). The study endpoints were comprised of time to reach the first therapeutic international normalization ratio (INR), time to reach a stable warfarin maintenance dose, time with over-anticoagulation, bleeding episodes, hospital stay days and stable warfarin maintenance dose. RESULTS: There was no significant difference concerning the demographic data between the two groups. The time to stable warfarin maintenance dose and hospital stay days were significantly lower in group 1 (P <0.001). However, there was no statistically significant difference in time to reach the first therapeutic INR, time with over-anticoagulation and bleeding episodes, between the two groups. CONCLUSIONS: The determination of warfarin dose, based on genotyping, might reduce the time to achieve stable anticoagulation of warfarin dose and length of hospital stay.

Early and Mid-Term Outcome of Pediatric Congenital Mitral Valve Surgery.

BACKGROUND: Congenital lesions of the mitral valve are relatively rare and are associated with a wide spectrum of cardiac malformations. The surgical management of congenital mitral valve malformations has been a great challenge. OBJECTIVES: The aim of this study was to evaluate the early and intermediate-term outcome of congenital mitral valve (MV) surgery in children and to identify the predictors for poor postoperative outcomes and death. PATIENTS AND METHODS: In this retrospective study, 100 consecutive patients with congenital MV disease undergoing mitral valve surgery were reviewed in 60-month follow-up (mean, 42.4 ± 16.4 months) during 2008 - 2013. Twenty-six patients (26%) were under one-year old. The mean age and weight of the patients were 41.63 ± 38.18 months and 11.92 ± 6.12 kg, respectively. The predominant lesion of the mitral valve was MV stenosis (MS group) seen in 21% and MR (MR group) seen in 79% of the patients. All patients underwent preoperative two-dimensional echocardiography and then every six months after surgery. RESULTS: Significant improvement in degree of MR was noted in all patients with MR during postoperative and follow-up period in both patients with or without atrioventricular septal defect (AVSD) (P = 0.045 in patients with AVSD and P = 0.008 in patients without AVSD). Decreasing trend of mean gradient (MG) in MS group was statistically significant (P = 0.005). In patients with MR, the mean pulmonary artery pressure (PAP) had improved postoperatively (P < 0.001). Although PAP in patients with MV stenosis was reduced, this reduction was not statistically significant (P = 0.17). In-hospital mortality was 7%. Multivariate analysis demonstrated that age (P < 0.001), weight (P < 0.001), and pulmonary stenosis (P = 0.03) are strong predictors for mortality. Based on the echocardiography report at the day of discharge from hospital, surgical results were optimal (up to moderate degree for MR group and up to mild degree for MS group) in 85.7% of patients with MS and in 76.6% of patients with MR. Age (P = 0.002) and weight (P = 0.003) of patients are strong predictors for surgical success in multivariate analysis. CONCLUSIONS: Surgical repair of the congenital MV disease yields acceptable early and intermediate-term satisfactory valve function and good survival at intermediate-term follow-up. Strong predictors for poor surgical outcome and death were age smaller than 1 year, weight smaller or equal than 6 kg, and associated cardiac anomalies such as pulmonary stenosis.

Single Dose Corticosteroid Therapy After Surgical Repair of Fallot's Tetralogy; A Randomized Controlled Clinical Trial.

BACKGROUND: Inflammatory reaction can produce several complications after cardiac surgery. Many attempts have been made to reduce these complications; perioperative corticosteroid therapy is one of the simplest methods. OBJECTIVES: We conducted a randomized study to evaluate the efficacy of single dose methylprednisolone, prescribed after surgery, for reducing the complications. Repair of Tetralogy of Fallot was chosen as a homogenous large group for the study. PATIENTS AND METHODS: One hundred children who underwent total repair of Tetralogy of Fallot were enrolled in this study. After the surgery, all patients were transferred to pediatric ICU and were randomized (in a double-blind fashion) in 2 groups (A and B); a single dose of methylprednisolone (30 mg/kg of body weight) was injected to participants of group "A" just at the time of ICU entrance. Group "B" received no drug. Then, clinical outcomes and laboratory data were compared between the two groups. RESULTS: The only significant differences were lower incidence of bacteremia and higher incidence of hyperglycemia in the group who were used methylprednisolone. CONCLUSIONS: Using a single postsurgical dose of methylprednisolone does not significantly alter the clinical outcome after repairing Tetralogy of Fallot.

Non-thyroidal illness syndrome and cardiopulmonary bypass in children with congenital heart disease.

BACKGROUND: The thyroid hormones influence on all metabolic pathways. After heart surgery using cardiopulmonary bypass (CPB), serum T3 decreases and remains low for at least 24 hours. Several studies on pediatric have reported reduction of thyroid hormones after heart surgery. This study aimed to investigate the status of thyroid function tests in children with CPB surgery. METHODS: This study was carried out based on the available data on 132 children aged less than 15 years suffering from CHD. The patients underwent open heart surgery in Rajaie Center in Iran from January to November 2010. The thyroid hormone levels were measured shortly after admission, and postoperatively in Intensive Care Unit (ICU) and thereafter at 12, 24 and 48-hour intervals. The patients' gender, age, weight, body mass index, heart disease details, previous cardiac surgeries, and cardiac surgery-related data such as pump time, aortic clamping time, hypothermia duration, postoperative hemodynamic status and postoperative use of inotropic drugs were recorded and analyzed Results: All patients showed a decrease in T3, T4 and TSH and an increase in T3-resin uptake after surgery. Eventually, 3 (3.2%) patients died. Preoperatively, there was a significant association between the reduction in the thyroid hormone levels and inotropic drugs as well as the type of the heart disease (p<0.05). CONCLUSION: There is an association between post-operative inotropic drugs administration and reduction thyroid hormones levels in patients undergoing congenital heart disease cardiopulmonary bypass surgery.

Cardiac malformations in fetuses of gestational and pre gestational diabetic mothers.

OBJECTIVE: In this study we aimed to determine the prevalence of cardiac malformations in fetuses of Iranian diabetic mothers with pre-gestational and gestational diabetes mellitus (GDM) and to find the patterns of different cardiac malformations. METHODS: One-hundred and seventy diabetic pregnant women (68 preGDM and 102 GDM) (mean age: 32.17±4.8 years) and 85 healthy controls (mean age: 31.35±4.55 years) were recruited from September 2008 to July 2012. Fetal echocardiography was performed to assess cardiac malformation. In order to study major factors that may affect the results, a complete history was obtained. FINDINGS: Fetal echocardiography was performed at mean gestational age of 24.7±5.4 and 20.27±3.9 weeks in diabetic patients and control group, respectively. Fifteen (8.8%) fetuses of diabetic mothers were detected to have cardiac malformations compared with 1 (1.17%) fetus in control group (OR: 8.13, 95%CI: 1.1-62.61, P-value=0.02). Hypertrophic cardiomyopathy noted as the most common cardiac malformation occurred in 6 out of 15 (40%) fetuses, and was found significantly more common in pre-GDM compared to GDM group (7.4% vs 1%, P-value =0.04). Despite the higher incidence of cardiac malformation in pre-GDM compared to GDM group, the difference was not significant. Further, no significant association was observed between the variables including; parity, diabetic regimen, parents' consanguinity, maternal history of hypertension or hypothyroidism and occurring cardiac malformations (P-value>0.05). CONCLUSION: In this study we detected cardiac malformations in 8.8% of our diabetic referrals. The result of the present study shows that screening diabetic mothers for fetal cardiac malformations could be beneficial.