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Background: Improving surgical and interventional modalities for treating congenital heart disease has increased these children’s life expectancy, leading to increased cognitive and behavioral problems. Objectives: This study compared executive function between surgically and interventionally treated congenital heart disease children and their age-matched controls. Methods: In this retrospective cross-sectional study, we enrolled 30 children treated with surgery, 30 interventionally treated patients, and 30 healthy controls. These participants were compared in the executive function domains by the Stroop test, trail making test, cancellation test, visual and auditory memory span test, and behavioral problems using the parental form of strengths and difficulties questionnaire. Results: Executive function in memory span and sustained attention was lower in the surgical group than in the intervention group (P < 0.05). Both groups had lower flexibility, memory recall, simple and complex working memory, visual attention, and simple and complex selective attention performance (P < 0.05). The effect of behavioral problems on daily life was higher in surgical patients than in the intervention group (P < 0.0001). Both groups of patients had higher emotional, conduct, and communication problems than the control group (P < 0.0001). Conclusions: Interventionally treated patients had better performance than surgically treated patients. Regardless of disease severity or treatment, patients with congenital heart disease had poor performance than normal subjects and suffered from behavioral disorders affecting their daily lives. Therefore, it is essential to include diagnostic and therapeutic interventions for treating these patients’ executive function and behavioral problems.

Background: Patent ductus arteriosus (PDA) stenting was invented particularly for congenital heart diseases with ductal-dependent pulmonary blood flow, and newer techniques are constantly being introduced. Objectives: In this study, we investigate the one-year results of this procedure and describe some of our periprocedural considerations. Methods: The neonates with pulmonary atresia and ventricular septal defect (VSD) who underwent PDA stenting in our center from February 2016 to December 2019 were included in this retrospective study. Spo2 and McGoon ratios were recorded before and a year after stenting and compared to each other. Results: In 26 neonates, PDA stenting was performed; 20 procedures (77%) were successful, and six procedures (23%) were unsuccessful. Two of these unsuccessful cases died during the procedure, and two of them died as a result of neonatal sepsis following the procedure. A total of 18 PDAs (70%) were accessed via the axillary artery, 6 (23%) via the femoral artery, and 2 (7%) via the antegrade inferior vena cava route. The comparison between the results before and one year after stenting revealed the increase of O2 saturation from 53.78 ± 7.35 to 84.33 ± 6.5 (P-value = 0.032) and McGoon ratio from 1.16 ± 0.25 to 1.65 ± 0.34 (P-value = 0.041). Additionally, we discussed some technical considerations of PDA stenting, including the access point, management of pulmonary artery bifurcation stenosis, stent diameter, stenting of long and tortuous PDAs, thrombosis, and anticoagulation. Conclusions: Ductal stenting increases SpO2 and McGoon ratio and could be a less-invasive option for securing pulmonary blood circulation. However, further studies are required to advance PDA stenting techniques for specific anatomical features.

Background: Perimembranous ventricular septal defect (VSD) is the most common congenital heart defect. There is a trend for percutaneous VSD closure. However, little evidence is available for the effect of this method on ventricular remodeling. Objectives: This study aimed to investigate the effect of percutaneous closure of perimembranous VSD on cardiac function and ventricular recovery. Methods: A total of 46 pediatric patients (32 males vs. 14 females) who underwent transcatheter closure of perimembranous VSD from 2010 to 2020 were randomly included in the study. Data regarding the demographic profile, angiographic records, and follow-up echocardiography were extracted from their files and recorded in questionnaire templates. The echocardiographic parameters were recorded and compared with published Z-scores for the corresponding age groups. Results: The mean duration of follow-up was 15.76 ± 12.20 months. In M-mode echocardiography, 84.6% had interventricular septum diastolic diameter Z-score ≥ 2; 23.8% had interventricular septum systolic diameter Z-score ≥ 2; 38.5% had left ventricular internal diameter in diastole Z-score ≥ 2; 34.6% had left ventricular internal diameter in systole Z-score ≥ 2; and 65.4% had left ventricular posterior wall in diastole Z-score ≥ 2. In the evaluation of Doppler and tissue Doppler, 36.4% of the patients had a Z-score ≥ 2 for E/Ea of tricuspid. Also, VSD size had a positive correlation with interventricular septal diameter in systole Z-score (P = 0.015, r = 0.537). Conclusions: In the midterm follow-up after percutaneous perimembranous VSD closure, left ventricular dilation and hypertrophy persisted in a significant number of patients. However, early closure of the VSD, especially in patients with lower weight could affect ventricular hemodynamics and remodeling.

Background: Atrial septal defect and its closure can lead to changes in the right and left cardiac cavities' function and size. In this study, Z-scores of the cardiac chambers and the heart function were assessed, and the important complications were mentioned. Methods: This interventional cross-sectional study was done on patients who had atrial septal defect closure aged younger than 18 years. All patients were recruited for transthoracic echocardiography. About half of the patients were randomly selected. The information of angiography and its side effects belong to all patients, but the echocardiographic parameters and Z-scores belong only to the selected group. Results: A total of 370 patients underwent the atrial septal defect closure, of whom 150 patients participated in the study. The patients' average age and weight were 9.25 ± 3.44 years and 15.12 ± 11.83 kg, respectively, and the mean follow-up time was 2.56 years. Z-scores of the interventricular septal dimension in diastole, the left ventricular posterior wall dimension in diastole, the left ventricular internal dimension in systole, and Z-scores of the size of the right atrium, right ventricle, pulmonary valve annulus, and the main pulmonary artery were more than Z-scores of the normal population. Furthermore, Z-scores of the E/A and the Eat/Aat of the tricuspid valve were less than their peers. Besides, the correlation between Z-scores and the atrial septal defect size and weight of the patients was assessed, which was statistically significant, and patients who underwent atrial special defect closure at the age of fewer than three years and less than 15 kg had more normal cardiac Z-scores. Conclusions: Z-scores of the cardiac chambers and pulmonary artery were more than normal after successful closure of the atrial septal defect in the mid-term follow-up.

Background: The definite treatment of tetralogy of Fallot (TOF) as the most common cyanotic congenital heart disease is open heart surgery and the operation technique depends on the coronary artery anatomy. Objectives: We aimed to evaluate the sensitivity and specificity of various root Aortogram views to detect abnormal coronary artery configuration and course in these patients. Methods: In this retrospective study, the reports of coronary anatomy in angiography and operation notes of TOF patients since 2005 to 2018 were evaluated. The sensitivity and specificity of the two common root Aortogram views including the left anterior oblique/cranial (LAO/CRA) and left anterior oblique/caudal (LAO/CAU) were determined. Results: Four hundred and fifty one patients with the median age of 28 months (3 - 432 months) were included. All patients had LAO/CRA views and 199 patients had both LAO/CRA and LAO/CAU views. The rate of coronary abnormalities reported by surgery was 8%, and abnormal origin of the left anterior descending artery from the right coronary artery was the most common reported anomaly (4.4%). The sensitivity of the LAO/CAU view was 100%, but LAO/CRA view was about 75% in detecting abnormal coronary configuration. Both of them had excellent specificity (~100%). No significant sex difference was seen between different coronary anatomy abnormalities. Conclusions: The Caudal root angiogram is an essential view to evaluate coronary artery anatomy in TOF patients and it seems that there is no need for other views like lateral view, but relying on LAO/CRA view alone may lead to significant misdiagnosis of the coronary arteries, crossing the right ventricular outflow tract (RVOT).

Background: Ordinary pressure dressing for hemostasis after cardiac catheterization is time consuming and might cause some problems, such as pain or loss of blood, which has to be controlled, particularly in pediatric patient. Using Celox® (chitosan) powder dressing might cause quicker initial hemostasis. Methods: In this prospective study, we assessed Celox® powder among patients in hospitals affiliated with Shiraz University of Medical Sciences, from November 2017 to February 2018. The patients were stratified in two groups. The case group included patients for whom Celox® powder was used along with sterile gauze pressure at the puncture site to achieve hemostasis, and the control group those in whom hemostasis was achieved by standard sterile gauze pressure method. Results: Sixty patients under 16 years of age with congenital heart diseases were evaluated and underwent cardiac catheterization. We stratified the patients in two groups called case (30 patients) and control group (30 patients). Considering both arterial and venous initial hemostasis, in the case group, the minimum and maximum, median and mean coagulation time were less than those in the control group; however, the initial hemostasis was statistically significant only in venipuncture site. Also, the venous coagulation time was shorter among the patients weighing less than 10 kilograms in comparison to those with higher weight. In the case group, using Celox® stirred hemostasis toward the lower percentiles, but based on 50th percentile, the distribution in each group was identical. Conclusions: Celox® powder dressing in children led to reduced coagulation time in venipuncture site, and we might recommend utilizing this type of dressing for venous hemostasis in children after venipuncture.

Objectives: This prospective study was designed with the aim to evaluate the prevalence and risk factors of arterial and venous complications among children and adolescents during cardiac catheterization. Methods: All patients younger than 18 years who underwent cardiac catheterization from April 2016 to April 2017 were consecutively enrolled in this study. Results: A total of 179 vascular accesses were performed, and most (70%) cases were interventional procedures. Totally 17 (14%) arterial and 16 (13%) venous events occurred, while 4% and 5% of them, respectively, were more serious. Half of the patients who underwent axillary artery access developed major or minor trauma, and these subtracted from total events of artery accesses, only 9% of femoral arteries developed minor or major complications. The more serious arterial complications were dissection, pseudo-aneurysm and fistula whereas in venous access they were pseudo-aneurysm and thrombosis. Serious and non-serious complications considered together, the mean age of those without complication was 1.9 ± 3.6 years and in complicated group it was 1.7 ± 2.6 years (P = 0.33), and the mean weight of non-complicated cases was 9.27 ± 7.9 kg and 8.51 ± 8.12 kg in the complicated group with no significant difference (P = 0.41), whereas the incidence of more serious vascular complications was highest among patients younger than 1 year of age, and less than 9 kilograms. 23% of the cases who underwent more than 3 tries for arterial and 100% for venous access showed the complications. Also few arterial tries resulted in unintentional venous complications and vice versa. None of the cases needed surgical or interventional therapy. Conclusions: Our study showed that venous complications are as high as arterial complications, although most of them are self-limited.

Background: Benefits of stem cell therapy on remodelling and cardiac function have been described in adults with dilated cardiomyopathy and acute myocardial infarction. Objectives: We investigated the effect of this treatment modality amongst children with severe dilated cardiomyopathy. Methods: Intracoronary injection of autologous bone marrow mononuclear stem cells was performed in our centers for 8 severely ill children during 2015 - 2016. The mean age of the patients was 10.1 years (5 girls, 3 boys). They were followed by longitudinal speckle tracking echocardiography (STE) and conventional echocardiography for 6 months. Results: Heart functional class improved in 62% of patients. M-mode echocardiography showed significant improvement in ejection fraction (mean 24.8 ± 8.3 vs. 37.4 ± 10.5) and in STE, the mean global longitudinal strain improved (GLS: -2.8 ± 1.9 vs. -5.2 ± 3.9). None of the patients had serious complications. Conclusions: Intracoronary injection of autologous mononuclear stem cells might improve the ventricular function and cardiac remodelling in pediatric patients with dilated cardiomyopathy and could be considered in critically ill patients.

Background: Speckle tracking echocardiography has great value in evaluation of regional and global myocardial function. Objectives: The goal of this study was an assessment of cardiac function and remodeling in children with thalassemia major after infusion of a high dose of deferoxamine during a short course by speckle echocardiography and evaluate the efficacy of this imaging modalities in the early recognition of recovering of myocardial dysfunction. Methods: In a prospective study between Feb 2014 till 2017 conventional 2 dimensional and speckle tracking echocardiography were done consecutively on 21 patients with beta-thalassemia major before intravenous infusion of high dose of deferoxamine (50 mg/kg) for 5 days and then after 3 months echocardiographic measurements repeated for assessment of efficacy of deferoxamine infusion on ventricular function and cardiac remodeling of our study population. Results: Serum ferritin of all patients reduced significantly (P < 0.001). Ejection fraction was improved after the therapy (P < 0.001). Mitral E/A velocity ratio after therapy increased significantly (P < 0.001). Strain imaging measures showed an increase in apical lateral, mid-lateral, basal lateral, mid-septal, basal septal left ventricular longitudinal wall strain three months after the use of high dose deferoxamine (P < 0.001). Apico-septal wall strain measurements of the left ventricle did not change significantly after high dose deferoxamine (P = 0.144). Conclusions: Intravenous infusion of high dose of deferoxamine after chelating of iron results in reduction of serum ferritin which may cause washout of cardiac deposit of iron with consequent improvement of cardiac function and remodeling.

Background: Neonatal jaundice resulting from raised blood bilirubin levels is one of the most common clinical conditions that needs medical attention. To initiate appropriate management that can both prevent and treat severe neonatal jaundice, screening methods that measure bilirubin level are warranted. Methods: In this study, we present an Android OS-based application for detecting neonatal jaundice. We used the application to detect jaundice in 113 neonates. Our smartphone-based estimation of bilirubin levels depends on a smartphone, a color calibration card, and a 100X zoom microscope clip. Our application was designed to acquire images of the newborn’s forehead skin in a standardized manner, estimate the average R, G, B scores of the images that have been taken from the forehead skin and calibration card, and then convert them to hue, saturation, intensity (HSI) parameters. All these are performed offline; in this application, we used offline machine learning and regression techniques for analysis Results: Our smartphone-based estimation of bilirubin levels had a sensitivity of 68% and specificity of 92.3% for estimating the bilirubin levels of less than 10 mg/dL and sensitivity of 82.1% and specificity of 100% for estimating the bilirubin levels of less than 15 mg/dL. Our application-based estimation of bilirubin levels had the correlation of 0.479 with the total serum bilirubin values. Conclusions: Our results suggest that our smartphone-based application can serve as a promising screening tool for neonatal jaundice, and it can aid in determining neonates requiring a blood draw for measuring total serum bilirubin level.

Background: The aim of this study was to determine if there is any correlation between patent ductus arteriosus (PDA) dimensions measured by two dimensional echocardiography (2DE) and three dimensional echocardiography (3DE) in comparison with angiographic data as the gold standard technique. Such correlation may help us to immediately detect ductal spasm and select the proper device according to echocardiographic assessments. Methods: In this comparative study, we successively selected 26 pediatric patients with isolated PDA, who referred for elective percutaneous PDA closure at Nemazee Hospital, affiliated to Shiraz University of Medical Sciences (SUMS) since January 2016 till March 2017. All patients underwent full 2DE and 3DE before device closure at the day of angiography (less than 5 hours before catheterization). We emphasized the dimension of pulmonic and aortic end diameter and length of PDA by these modalities and comparison of our data. Results: The study population had a mean age of 28.7 months and a mean weight of 10.67 Kg; the majority of our patients were female (84.6%). The difference in pulmonic end of PDA was not significant statistically by all modalities. The difference in aortic end of PDA was significant and there was no correlation between 2DE and 3DE with angiographic data. Comparison of data obtained from 2DE and 3DE revealed that the length of PDA in 2DE, 3DE angiography well correlated with each other. One case developed ductal spasm during angiography. Conclusions: The ductus pulmonic end and length dimensions measured by echocardiography and angiography well correlated with each other and were interchangeable. Such findings may be helpful during percutaneous transcatheter occlusion if any ductal spasm happens. Thus, we may recommend the use of a suitable device according to the maximum size of PDA that was measured either by echocardiography or angiographic studies.

Isolated major aortopulmonary collaterals are rare in healthy term neonates or infants without congenital heart disease; only a few cases have been reported with heart failure or recurrent lower respiratory tract infection in an otherwise structurally normal heart and there is no consensus about their treatment. We report a case with repeated pneumonia, failure to thrive, pulmonary hypertension, and shift of heart to right which is successfully managed by stage coil closure of collaterals.

Background: Tissue Doppler imaging evaluates the ventricular function in both systolic and diastolic assessments. Objectives: This study aimed to determine the level of cardiac function in children with Down syndrome without structural cardiac lesions using conventional and tissue Doppler echocardiography. Methods: From September 2013 to August 2014 in Mashhad, Iran, a group of 36 children with Down syndrome aged between 4 months and 19 years without anatomic heart disease had their ventricular function assessed by both 2D echocardiography and tissue Doppler imaging. The following were measured: velocity of systolic waves (S), traditional Doppler imaging of early diastolic waves (E), traditional Doppler imaging of late diastolic waves (A), tissue Doppler imaging early diastolic waves (E’), and tissue Doppler imaging late diastolic waves (A’) at the mitral and tricuspid valve levels. Other data, such as fractional shortening, heart rate, and ejection fraction, were also measured. Results: At the time of the examination, the mean age of the participants was 6.4 ± 5.5 years. An association was found between children’s age and the tricuspid A-wave. The A-wave velocity of the tricuspid annulus increased when the body surface area decreased. In addition, compared with the normal subjects, the large age group presented with considerable differences in wave speed, including E, A, and E/E’, at the mitral and tricuspid annulus levels. Conclusions: Despite the Down syndrome children having an apparently normally structured heart, tissue Doppler imaging reveals different findings than those of normal children. The findings of this modality can be used to explain the reason behind the limited physical functioning of children with Down syndrome and may be considered as predictive factors for future cardiac events in these children. © 2016, Growth & Development Research Center.

Background: Transcatheter patent ductus arteriosus (PDA) closure is an established procedure. Objectives: The aim of the study was to assess midterm follow up of the Nit-Occlud coil and the amplatzer ductal occluder (ADO) closure of PDA. Patients and Methods: In this cohort study, we collected the longitudinal data of patients who underwent percutaneous closure using coil or ADO from November 2005 to November 2013. A total of 404 patients with PDA closure by devices were included during the study period. Coil occlusion was performed in 220 patients and 184 patients underwent catheterization using ADO. Follow-up evaluations were performed with echocardiography at two weeks, two months, six months, and during the study period (in average 4.8 ± 3.8 years). Results: The patients’ mean age was 24 months (range: 1 - 312). The catheterization was successful in 393 (97.2%) patients and unsuccessful in 11 (2.7%). Immediate complete occlusion was seen in 290 (73.7 %) patients. The occlusion rates at two weeks, two months, six months, and during the study period were 73.7%, 84%, 93.6%, 98.7%, and 99.5%, respectively. Complications occurred in 23 (5.8%) patients during or immediately after the catheterization, and device embolization with 2.7% was the most common complication. Most complications occurred in a patient with pulmonary hypertension who was less than one year old and was undergoing the first year of experience with devices. Conclusions: Our findings showed that transcatheter occlusion of the PDA is an effective and safe intervention by coil or Amplatzer with excellent early and one-year outcomes. Pulmonary hypertension, age of less than 12 months and experience of less than one year may increase the complications of device closure.

Objective The purpose of this study was to examine the relationship between T-peak-to-T-end interval and its dispersion in children with syncope to detect possible repolarization abnormalities in these patients. Methods We enrolled 19 patients with a positive tilt test for syncope (7 boys, 12 girls) and 35 participants with normal results on the test. Findings Mean age was 11.4±3.1 years in patients and 10.0±5.1 years in controls (P =0.27). The T-peak-to-T-end interval in lead V1 was significantly longer in patients with a positive tilt test (0.36±0.062 vs. 0.32±0.071, P=0.007). T-peak-to-T-end interval dispersion was significantly greater in the group of patients (0.15±0.07 vs. 0.11±0.04, P±0.003). Conclusion The T-peak-to-T-end interval in lead V1 and T-peak-to-T-end dispersion were significantly larger in patients with a positive tilt test. Our findings suggest a depolarization abnormality in children with syncope.

Introduction: Truncus arteriosus with intact ventricular septum is a rare and unique variant of persistent truncus arteriosus (PTA) which usually presents with central cyanosis and congestive heart failure in neonate and early infancy. Associated cardiac and non-cardiac anomalies may affect morbidity and mortality of these patients. Case Presentation: We describe clinical presentation, echocardiography and angiographic features of a 7-month old boy with PTA and intact ventricular septum who underwent surgical repair of the anomaly at our institution. Operative findings, surgical procedure and short-term outcome are reported. Conclusions: While our patient had systemic pulmonary arterial pressure at the time of complete surgical repair, it was improved after surgery.

Background: Patent ductus arteriosus (PDA) is an important risk for heart failure due to left to right shunt in term neonates. Objectives: In this study, we evaluated the effect of high dose ibuprofen in closure of PDA in term neonates. Patients and Methods: We used double dose ibuprofen (20 mg/kg, 10 mg/kg, and 10 mg/kg) for 3 - 30 day old term neonates with PDA who were admitted in the neonatal wards of Shiraz University of Medical Sciences. The results of this study were compared to the data of the previous study in our center which used the low dose of ibuprofen (10 mg/kg, 5 mg/kg, and 5 mg/kg). Results: 29 full term neonates received high-dose ibuprofen, in 18 neonates, PDA was closed after 4 days (62.1% versus 43.3% for the standard dose and 4.7% for the control group in the previous study) (P = 0.001). The results showed no significant correlation between the closure rate and gestational age, postnatal age, sex, and weight. In the 4 th day of treatment, size of the pulmonic end of ductus arteriosus decreased from 2.09 mm to 0.77 mm compared to 1.68 mm to 0.81 mm in the standard dose of oral ibuprofen and 2.1 mm to 1.4 mm in the control group (P = 0.046). Conclusions: This study indicated that high-dose oral ibuprofen was more effective in closing or decreasing the size of PDA.

Dear Editor, Takayasu arteritis (TA) is a rare granulomatous pan-arteritis that involves large vessels predominantly the thoracoabdominal aorta and its branches (1). Angiography, particularly subtraction angiography is the procedure of choice for diagnosis of patients with TA and provides an appropriate guide for endovascular procedure (2, 3). A ten-year-old Afghan girl was referred to the pediatric cardiology ward of Namazee Hospital, Shiraz, Iran due to a history of fever, vascular claudication, and easy fatigability in both lower extremities. In physical examination, pulseless upper extremities and carotid artery bruit were detected. One year prior to this admission, the patient was labeled as a case of Henoch-Schonlein purpura due to her first presentation with rash and fever. In spite of this management, erythrocyte sedimentation rate and C reactive protein levels had not returned to the normal range. Color Doppler sonography of both upper extremities showed the significant thickening of intima and media, diffuse lumen narrowing of both subclavian arteries, subclavian and axillary arteritis. Bringing together all the aforementioned findings, the patient’s impression was changed to the TA; therefore, prednisolone and infliximab was started. The patient was followed at the outpatient clinic. Four months later, she was admitted in the hospital due to decreased level of consciousness following left sided paralysis in both upper and lower extremities and status epilepticus. In her physical exam, motor power of the left upper and lower extremities was 1/5. The brain computerized tomography showed a small size intra-cerebral hemorrhage in the frontal lobe. Brain magnetic resonance angiography showed stenosis in the posterior, middle and anterior cerebral artery. Echocardiography showed fair left ventricular function, hypertrophic left ventricle with dyskinesia of the ventricular septum. She received plasmaphresis, rituximab, prednisolone, anticonvulsants (phenobarbital and phenytoin) and antihypertensive drugs. Multislice computerized tomography showed stenosis of both carotids and subclavian arteries (Figure 1). Figure 1. Multislice Computerize Tomography Shows Stenosis of Both Carotids And Subclavian Arteries Due to repeated convulsions and poor general condition of the patient angiography for possible stenting the stenotic cranial arteries was done. The right innominate and subclavian artery was severely stenotic and totally occluded just proximal to the origin of the vertebral artery (Figure 2). Blood flow to the right cerebral hemisphere was supplied by the collateral channel, mainly from the vertebrobasilar system. The left common carotid and subclavian arteries were also stenotic. Figure 2. A, Arch Aortogram Shows Deterioration of All the Supra-Aortic Lesions; B, Transbrachial Angiogram Reveals That the Right Subclavian Artery Was Stenotic (Arrow) Proximal to the Orifice of the Vertebral Artery. Right common carotid artery was not visualized ... Under conscious sedation anesthesia and systemic heparinization, a five-French guiding catheter was placed in the aortic arch via the femoral route and a hippocampus stent 6 × 20 mm stent was deployed at the stenotic lesion of the right subclavian artery but the stenosis could not be relieved by multiple inflation of balloon (Figure 3). Subsequently, another stent (hippocampus stent 6 × 20 mm) was placed in the stenotic part, proximal to the previous stent, which markedly improved the antegrade flow of the right common carotid artery (Figure 4). Figure 3. A and B, A Hippocampus Stent 6 × 20 mm Stent Was Deployed at the Stenotic Lesion of the Right Subclavian Artery but the Stenosis Could not Be Relieved by Multiple Inflations of Balloon; C, Another Stent (Hippocampus Stent 6 × 20 mm) Was ... Figure 4. Final Result Showed Significant Improvement of Antegrade Flow to Right Hemisphere The symptoms disappeared immediately after the first treatment, and the patient returned to her daily life activities. Neurologic examination showed no changes during one year follow up and Doppler sonography revealed both carotid stenosis and good flow through the stent. In cases of TA, stenting for these supra-aortic vessels, especially for the carotid lesions, has rarely been presented (4-6). Several authors have reported using subclavian stenting in cases of adult TA (7, 8). In our case, we think that the right vertebral artery stenting was reasonable because both carotid arteries were occluded and the right vertebral artery was functioning as the main collateral pathway to the right cerebral hemisphere, which must be preserved throughout life. To our knowledge, this is the first reported case of stenting of stenotic lesions in the supra-aortic vessels in children in Iran and also there are few reports of stenting in children in the literature.

Objective: This study aimed to evaluate the prevalence of celiac disease (CD) in the patients with dilated cardiomyopathy (DCM). Simultaneous presentation of these two diseases has been recently reported in some studies; however, few researches have been done on children. The sooner CD is diagnosed, the better the prognosis will be, especially in the patients with a chronic disease like DCM. Methods: In this study, 82 cases were screened for CD by measuring the level of anti-body against transglutaminase (anti tTG). These cases included 41 patients with DCM labeled according to clinical evaluation and echocardiography and 41 healthy children who had been referred for routine checkup. All the patients were between 1 and 18 years old. The expired patients and those with previous diagnosis of CD were excluded from the study. Besides, the patients with positive antibody results underwent intestinal biopsy to match the serology findings with histopathology of CD in the intestine. Finally, the data were analyzed by the SPSS statistical software (v. 16) and through t-test and Pearson correlation coefficient. Findings: According to the findings, 1/41 (2.5%) DCM cases had positive tTG antibody level and negative intestinal biopsy which is classified as potential CD in the children with DCM. In addition, 7/41 (17%) patients had borderline anti body level. A direct correlation was observed between age and anti tTG level. Conclusion: It is beneficial to assess CD in DCM children with unknown cause.

Objective Dilated cardiomyopathy is the end result of chronic iron overload in patients with beta thalassemia major. The objective of the present study was to evaluate the safety and efficacy of Carvedilol in patients with beta thalassemia major and dilated cardiomyopathy. Methods During a six-month period, fourteen patients with beta-thalassemia major and heart failure without diabetes mellitus referred to pediatric cardiology clinic enrolled in this double blind, randomly assigned study. All patients were on anti failure therapy with Digoxin, Captopril and Furosemide. Carvedilol was started at a dosage of 3.12 mg bid and for patients who had a systolic blood pressure >100 mmHg, heart rate >60/min and no signs of low cardiac output the dosage was increased every two weeks to a maximum of 25 mg bid. Clinical signs and symptoms, systolic and diastolic echocardiographic indexes and Tissue Doppler Imaging (TDI) data were collected from each patient. Findings Eight patients received Carvedilol (Group 1) and six received placebo (Group 2). The mean age of patients in Group1 and 2 were 16±0.7 years and 17±3 years respectively. Only one patent in Group 1 tolerated increasing Carvedilol dosage to more than 6.25 mg bid. Changes in New York Heart Association (NYHA) classification, Ejection fraction, End diastolic dimension changes, TDI systolic(S), early (Ea) and late (Aa) diastolic waves were not statistically significant in these two Groups (P>0.05). Pulse Doppler E/A wave ratio of mitral valve in Group1 and Group 2 changed from 1.1±0.37 m/s to 1.8±0.40 m/s and from 1.34±0.30 m/s to 2.6±0.23m/s respectively (P=0.04). Conclusion Patients with thalassemia and dilated cardiomyopathy have poor tolerance to increasing Carvedilol dosage and develop decreased systolic blood pressure during advancement of the drug dosage. Carvedilol can be effective in prevention of progression of diastolic dysfunction in these patients.