hamid amoozgar

Inadequate pulmonary blood flow in tetralogy of Fallot (TOF) can lead to the development of major aortopulmonary collateral arteries (MAPCA), which interferes with surgical repair. The present study evaluated the features of MAPCAs among patients with TOF and their treatment approaches. Besides, perioperative parameters and mortality rates of our TOF patients with and without MAPCA were compared.  This retrospective case-control study was conducted from 2011 to 2020 at Namazi and Shahid Faghihi Hospitals, affiliated with Shiraz University of Medical Sciences, Shiraz, Iran. The significant aspects of MAPCAs, including their quantity, the presence of dual or single supply lung segments, and the employed devices for closure were evaluated. The patients were divided into three groups: TOF patients without MAPCAs as the control group, those with preoperative percutaneous MAPCA closure (Closed MAPCA), and those with small MAPCAs deemed unsuitable for percutaneous closure (Open MAPCA). A comparative analysis, encompassing hospital and surgical data, such as the presence of MAPCA, blood transfusion volume, intubation time, ICU stay, and mortality rates during and post-surgery, was performed among the aforementioned groups. The Chi square, Mann-Whitney, and Kruskal-Wallis tests were used to analyze the data. 59 patients were enrolled, with a mean age of 27.98±24.19 months. The control group included 34 patients with no collaterals, the closed MAPCA group had 12 patients with occluded collaterals, and the open MAPCA group had 13 patients with small collaterals unsuitable for closure. Blood transfusion volume and intensive care unit (ICU) stay were significantly higher in the open MAPCA group than the control group (P=0.01 and P=0.04, respectively). The highest mortality rate was seen in the Iranian Journal of Medical Sciences group (P<0.001).  In TOF patients, percutaneous MAPCA closure prior to surgical repair was recommended. This approach could potentially decrease the occurrence of complications both during and post-surgery.

Tetralogy of Fallot (TOF) is the most prevalent cyanotic congenital heart defect. Pulmonary regurgitation (PR) is a common sequela following most surgical repairs for TOF. Tadalafil might reduce pulmonary vascular resistance after Tetralogy of Fallot total correction (TOFTC).

This study evaluated the efficacy of tadalafil in reducing PR volume and improving heart function among TOFTC children with severe PR, using transthoracic echocardiography, particularly focusing on changes in N-terminal pro-b-type natriuretic peptide (NT-proBNP).

The present study was conducted on TOFTC patients consecutively between September 2019 and August 2020 at Shiraz University of Medical Sciences, Shiraz, Iran. M-mode and two-dimensional (2D) Doppler echocardiography were performed, and NT-proBNP levels were measured in 20 patients before and one month after tadalafil administration. SPSS version 23.0 was utilized to analyze all results.

The patients' ages ranged from 25 to 128 months. The mean age and weight of the patients were 67.9 ± 34.5 months and 21.1 ± 6.9 kg, respectively. Tadalafil administration did not significantly improve Doppler and tissue Doppler parameters; however, it increased the pulmonary valve pressure gradient and velocity-time integral. Additionally, tadalafil had no significant effect on improving NT-proBNP levels. The Spearman correlation test did not show any significant correlation between the pulmonary valve pressure gradient and velocity-time integral with age, weight, and NT-proBNP.

Tadalafil increased the pulmonary valve pressure gradient and velocity-time integral in TOFTC patients with severe PR; however, it did not affect NT-proBNP levels or tissue Doppler parameters.

Branch pulmonary artery stenosis presents as either an isolated disease or in association with congenital heart diseases (in 2 - 3% of cases). Balloon angioplasty (BA) and stent implantation (SI) are two percutaneous methods that are commonly used to treat this condition. We compared the outcomes of these 2 methods in children and adolescents with isolated branch pulmonary artery stenosis.

Pediatric patients with severe branch pulmonary artery stenosis who were referred to our center and underwent transcatheter insertion via either BA or SI between 2010 and 2019 were studied. Patients’ demographic data, including age, gender, and body weight, were recorded. Standard procedures for BA and SI were applied. Post-procedure angiography and echocardiographic parameters were determined and compared between the 2 groups. Patients who were followed up for at least 2 years were selected for this study, and comparative evaluations were performed during the follow-up.

Forty patients with a median age of 2.1 years and a median weight of 10 kg were enrolled in the study, of whom 25 and 15 underwent BA and SI, respectively. The trans-stenotic pulmonary artery pressure gradient significantly reduced immediately after both procedures, but there was no significant difference between the BA and SI groups in terms of pulmonary artery pressure gradient early after the procedures (P-value = 0.014). There was a significant decline in the peak right ventricular pressure after both procedures. Restenosis occurred more frequently in the BA group. In the BA group, patients under one year old and weight lower than 10 kg had a lower restenosis recurrence rate, evidenced by echocardiographic measurements, compared to those with a weight over 10 kg. Three patients (12%) in the BA group needed balloon reinsertion.

Immediate therapeutic success was similar between BA and SI in patients with branch pulmonary artery stenosis. Re-stenosis occurred more often in the BA group; however, the chance of re-stenosis was lower among patients weighing less than 10 kg over a 2-year follow-up.

Conduction disturbance (CD) is a major complication of percutaneous closure of the perimembranous ventricular septal defect (pmVSD).

This study aimed to investigate the incidence, predisposing factors, and outcomes of sustained CD following percutaneous closure of these defects.

All patients whose pmVSD was closed successfully with percutaneous methods within April 2016 to April 2021 were enrolled in this cohort study. The defects’ size, septal aneurysms, and distance to the aortic valve annulus were determined with transthoracic echocardiography and catheterization. Continuous heart monitoring was performed during the procedures and one hour after, and standard 12-lead electrocardiograms were obtained regularly to determine any CD and arrhythmias.

A total of 260 patients who had successful pmVSD closure were enrolled in the study. In this study, 135 (52%) and 125 (48%) patients were male and female, respectively. The mean age of the patients was 75.66 ± 68.89 months. The patients’ median follow-upwas36months(range: 9-210 months). Thirty-onepatients (11.9%) developed sustainedCD,and25 cases recovered to normal conduction. Additionally, 7 patients (2.7%) had several permanent conduction abnormalities, including four right bundle branch blocks, two left bundle branch blocks, and one bi-fascicular block. No one had a permanent atrioventricular block. Closure of non-aneurysmal defects and more prolonged procedures were independent risk factors of CD. Using Amplatzer duct occluder type II was associated with less incidence of conduction abnormalities.

The incidence of sustained CD after transcatheter closure was relatively high; however, most cases recovered to normal conduction. The use of softer devices and the placement of devices into the septal aneurysms might lower the risk of CD.

Diabetic ketoacidosis (DKA) can cause several complications. The myocardial damage in children with DKA was not evaluated clearly. This study aimed to investigate the troponin level as a biochemical marker of myocardial damage in children with DKA during the disease and after recovery.

This cross-sectional study was conducted in the pediatric endocrinology department of Namazi Hospital, Shiraz, Iran, from May to December 2019. Fifty diabetic children aged 1 to 18 years old with DKA by census sampling method. All patients were examed, and electrocardiography (ECG) was carried out. Troponin level was measured at admission and 48 hours after recovery. Finally, data were analysed using SPSS software 20.

At the time of admission, the mean troponin level was 11.56±5.35 ng/mL and it became 2.15±1.21 ng/mL after recovery. Although the initial troponin level during DKA in three patients was higher than the expected normal reference range, their echocardiography and ECG were normal. Moreover, there was no statistical relationship between troponin level with age, diabetes duration, and DKA severity.

The troponin level was remarkably different during DKA and after recovery. However, in merely 6% of DKA patients, the troponin level was higher than the expected value. We recommended that these patients be followed for future cardiovascular complications.

 Improving surgical and interventional modalities for treating congenital heart disease has increased these children’s life expectancy, leading to increased cognitive and behavioral problems.

 This study compared executive function between surgically and interventionally treated congenital heart disease children and their age-matched controls.

 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.

 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).

 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.

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.

In this study, we investigate the one-year results of this procedure and describe some of our periprocedural considerations.

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.

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.

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.

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.

This study aimed to investigate the effect of percutaneous closure of perimembranous VSD on cardiac function and ventricular recovery.

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.

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).

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.

Wilson’s cardiac involvement causes cardiomyopathy, arrhythmia, autonomic nervous system dysfunction, and sudden cardiac death. This study aimed to evaluate cardiac dysfunction in pre-liver transplant patients suffering from Wilson’s disease and to classify their risk of arrhythmia and sudden cardiac death. 

This case-control study was performed in the Transplant Coordination Center at Namazi Hospital, Shiraz University of Medical Sciences, between 2012 and 2014. The cardiac function was evaluated with 12-lead electrocardiography and echocardiography (M-mode, color Doppler, and tissue Doppler imaging). P-wave dispersion, QT dispersion, and T peak to T end-dispersion were measured in the patient group, and these values were compared with those in the control group. 

Totally, 23 patients with Wilson’s disease and 47 healthy individuals were included in this study. P-wave dispersion and QT dispersion were significantly increased in the patients with Wilson’s disease (P<0.05). Pulsed Doppler echocardiographic findings showed significantly increased E and A peak velocities of the mitral and tricuspid annuli in the patient group (P<0.05). Tissue Doppler imaging was in favor of a significant increase in systolic and early and late diastolic velocities of the mitral and tricuspid annuli. 

The prolongation of P-wave dispersion and QT dispersion renders patients with Wilson’s disease susceptible to atrial and ventricular arrhythmias and sudden cardiac death. The evaluation of the cardiac function of such patients should include color Doppler and tissue Doppler imaging to assess diastolic dysfunction as one of the initial cardiac involvements. (Iranian Heart Journal 2022; 23(1): 118-128)

Coronary sinus dimension is an important factor for diagnosing some types of cyanosis as well as congenital heart diseases and insertion of some devices into the coronary sinus if required. This study was designed to access the diameter and Z-score of the coronary sinus among children under 18 years of age.

In this cross-sectional study on 95 individuals, the coronary sinus diameter was measured by transthoracic echocardiography in the four-chamber view at the connection to the right atrium, middle part, and distal end. The linear regression equation was utilized to determine age-adjust reference values, Z-scores, and the relationship between the individuals’ coronary sinus diameter age, height, and body surface area. The study was conducted from March to July 2020 in Namazi hospital clinic of Shiraz University of Medical Sciences, Shiraz, Iran.

The mean age of the persons who entered this study was 5.87±4.25 years. The mean coronary sinus diameter was 4.91±1.29 mm at the site of connection to the right atrium, 4.50±1.44 mm at the middle part, and 3.74±1.32 mm at the distal end. Coronary sinus diameter correlates positively with the participants’ age, weight, height, and body surface area (P<0.001).

Coronary sinus diameter significantly correlates with the age, height, and body surface area of the cases. These features are useful in diagnosing some congenital heart diseases and insertion of suitable devices through it.

Pacemaker implantation is an effective life-long treatment in patients with atrioventricular block to generate a reliable heartbeat. Choosing between epicardial and endocardial (trans-venous) techniques in children is based on the cardiac center experience and each technique has some benefits and risks.

In this observational cross-sectional study, we reviewed file-records of 186 under 18- year-old patients who underwent cardiac pacemaker implantation due to atrioventricular block. All of endocardial implantations had been performed by experienced pediatric cardiologists and all epicardial pacemakers by experienced cardiac surgeon from 2006 to 2018 in Namazi and Faghihi hospitals in Shiraz, Iran.

One hundred and five patients had epicardial pacemaker and 81 patients had endocardial pacemaker. One hundred and seventy-eight patients had postoperative complete heart block after correction of congenital cardiac abnormality due to the destruction of conductive pathway. Eight patients were born with complete heart block. Four (2.15%) patients in the endocardial group developed pacemaker related infection. Two (1%) patients had sudden cardiac death after pacemaker insertion in the follow-up; Medtronic single chamber pacemaker was inserted for one patient who had complete heart block after surgical ventricular septal defect closure. However, a month later she expired due to sudden cardiac arrest during exercise and one patient after correction of complete atrio-ventricular septal defect had pacemaker insertion and sudden death, 3 months after pace insertion (1.12%); none of them had history of palpitation, syncope, arrhythmia in their post-operation electrocardiography, or tachycardia in their pacemaker analysis.

In Conclusion, epicardial pacemaker has a noticeable battery longevity in comparison to endocardial pacemakers and fewer valvular complications and endocarditis cases. Also, it appears that increasing size and vessel stiffness followed by aging can prime better vascular access and less lead malfunction in older pediatrics in endocardial approach; however, the site of ventricular pacing is still a puzzle because of the effect of pacing site on left ventricle synchrony and ejection fraction.

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.

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.

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.

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.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Most studies have focused on Right Ventricular (RV) dysfunction, while the left ventricle has received less attention in patients with TOF.

This study aimed to investigate the Left Ventricular (LV) function after surgical Pulmonary Valve Replacement (sPVR) in patients with repaired TOF (rTOF) by Speckle Tracking Echocardiography (STE).

This single-center, observational, cross-sectional study was conducted on 58 volunteers (age: 15 - 31 years) divided into three groups as follows: 22 PVR patients (mean age: 18.96 ± 7 year), 16 patients with rTOF, and 20 healthy controls who were matched regarding the PVR age range. 2D echocardiography (including Doppler and M-Mode indices of the right and left ventricles) and Speckle Tracking Echocardiography (STE) (Global Longitudinal Strains (GLS) and 18 segment analyses) were performed for all patients. All analyses were done using the SPSS software and P < 0.05 was considered to be statistically significant.

2D echocardiography showed normal LV Ejection Fraction (LVEF) in all study groups (64% in sPVR, 60% in rTOF (P = 0.127), and 62.5% in the control group). However, the mean GLS of the left ventricle significantly reduced in both sPVR (-17.5 ± 2.5%) and rTOF (-17.1 ± 4.7%) patients in comparison to the control group (-20.2 ± 0.7%) (P = 0.003). Yet, no significant difference was observed between the rTOF and sPVR groups regarding the GLS (P = 0.9). Segmental analysis of the Longitudinal Strain (LS) indicated a significant decrease in the sPVR and rTOF groups in basal anterior, basal septal, basal anterolateral, mid-anterior, and anterolateral segments. Except for the lower LS in the apical-anteroseptal segment, this level was mostly spared in both sPVR and rTOF patients.

LVEF was within the normal range among the sPVR patients, but the pattern of impaired segmental LS and GLS did not change compared to the rTOF group. In conclusion, sPVR might not have a significant effect on the improvement of LV function assessed by STE in patients with rTOF. LV damage occurring during the surgical correction of TOF might have a permanent deteriorating effect on LV function.

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.

We aimed to evaluate the sensitivity and specificity of various root Aortogram views to detect abnormal coronary artery configuration and course in these patients.

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.

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.

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).

 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.

 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.

 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.

 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.

Cardiac catheterization and angiography are diagnostic and therapeutic imaging modalities that produce the highest X-ray radiation, which might impose lifelong risks to patients. This study aimed to evaluate radiation burden among children and adolescents with congenital heart diseases, who underwent cardiac catheterization and angiography. Then the results were compared with other imaging modalities.

In this prospective study, children and adolescents with congenital heart diseases were consecutively evaluated from April 2017 till November 2017. This research was designed in Namazi and Faghihi teaching hospitals affiliated to Shiraz University of Medical Sciences, Shiraz, Iran. In addition to the basic data and total radiation time, effective dose and dose area product were assessed for each patient.

We enrolled 148 consecutive patients under 18 years old, who underwent 36 diagnostic and 112 interventional cardiac catheterizations. Effective dose was 50.23 mGy in the diagnostic catheterizations, and 48.39 mGy in the interventional angiographies. Also the dose area product was 427/28 mGy.cm2 in the diagnostic catheterizations, and 476.62 mGy.cm2 in the interventional angiography. The most time-consuming cine fluoroscopy pertained to ventricular septal defect closure and (11.67seconds) and the most effective dose and dose area product pertained to the same procedure, as well (68,514 mGy and 623,843 mGy.cm2 respectively).

Effective dose and dose area product in cardiac catheterization were desirable, and some
previous studies revealed that carcinogenic effect of X-ray radiation is more prominent when effective dose is more than 70-100 mGy. Thus the effective dose might not cause hazardous outcomes if other x ray
modalities are not frequently requested for them in future.

Kawasaki disease (KD) is the most frequent cause of coronary artery aneurysm (CAA) in children. This study tried to evaluate the accuracy of different KD scores developed for prediction of CAA, in an Iranian population.

This is a cross-sectional retrospective investigation on pediatric patients with a diagnosis of KD. Clinical manifestations, laboratory, and echocardiographic data were recorded. Five Kawasaki scores, including Kobayashi, Egami, Sano, Nakano, and Harada, were assessed and analyzed in relation to CAA and intravenous immunoglobulin (IVIG) resistance.

During five years, we recruited 121 cases of KD under 13 years of age. The rates of CAA and IVIG resistance were 16.5%, and 13.2% respectively. The IVIG resistance group was significantly younger than responder patients. All five scores had low sensitivity in predicting CAA or IVIG resistant cases; the highest sensitivity pertained to the Harada score with 50% sensitivity and 59% specificity (the area under the curve: 0.545, with a 95% confidence interval: 0.423 to 0.667) in predicting CAA, which is lower than the usual acceptable criteria for a screening test. The specificity of all other scores were more than 85% in predicting CAA or IVIG resistance. Gender, fever before therapy and laboratory data showed no significant difference between the groups.

The Kobayashi, Egami, Sano, Nakano and Harada scores have limited usefulness in the Iranian population to predict high risk patients for coronary artery involvement or IVIG resistance; in our study, age under one year was a risk factor for IVIG resistance.

Type I Diabetes Mellitus (T1DM) is one of the most common endocrine diseases in the world. It is also the dominant type of diabetes mellitus among children and adolescents that, if left uncontrolled, becomes a major risk factor for cardiovascular disorders and may cause clinical and subclinical cardiac dysfunction.

This study aimed to compare uncontrolled pediatric and adolescent patients and similar healthy people regarding cardiac function according to echocardiography and B-type Natriuretic Peptide (BNP).

This case-control study was carried out in the Outpatient Department of Nemazi tertiary hospital in Shiraz, Iran between June and September 2016. The study included 29 consecutive outpatient cases aged 5 - 18 years who had been diagnosed with T1DM for more than three years and had normal ejection fraction in M-mode echocardiography. The control group consisted of 29 age- and gender-matched healthy non-diabetic volunteers. Both cases and controls were selected by simple random sampling. The participants underwent clinical evaluation, including BNP assay and echocardiographic examination composed of two-dimensional echocardiography, M-mode echocardiography, pulsed Doppler imaging, and Tissue Doppler Imaging (TDI). P-value ≤ 0.05 was considered to be statistically significant.

The results revealed a decline in cardiac function in TDI, which was related to the HbA1C level and duration of the disease. A significant difference was found between the cases and the controls regarding the left ventricular posterior wall, interventricular septal diameters in diastole, and E/A ratio of tricuspid valve (P = 0.047, P = 0.001, and P = 0.021, respectively). A significant difference was also observed between the two groups in terms of septal S´ and E´ parameters (P = 0.004 and P = 0.002, respectively). Moreover, diminished E/A ratio of tricuspid valve annulus was related to the increased duration of the disease. Even though left ventricular hypertrophy occurred in the patients, no significant increase was observed in BNP values.

The results indicated that M-mode, two-dimensional, pulsed Doppler, and TDI echocardiography might efficiently evaluate cardiac function in patients with preserved ejection fraction. However, BNP measurement might not be useful for screening of cardiac function.

 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.

 All patients younger than 18 years who underwent cardiac catheterization from April 2016 to April 2017 were consecutively enrolled in this study.

 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.

 Our study showed that venous complications are as high as arterial complications, although most of them are self-limited.

Benefits of stem cell therapy on remodelling and cardiac function have been described in adults with dilated cardiomyopathy and acute myocardial infarction. We investigated the effect of this treatment modality amongst children with severe dilated cardiomyopathy. 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. 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. 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.

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. 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 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. 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.

Speckle tracking echocardiography has great value in evaluation of regional and global myocardial function. 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. 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. 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). 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.

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.

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. 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. 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. 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

Congenital Aortic Stenosis (AS) is a common problem among congenital heart diseases and balloon valvuloplasty is known as the procedure of choice for treatment.
This study aimed at evaluation of the function and remodeling of the heart after a successful balloon AS valvuloplasty.
Patients and From September 2012 to 2016, 33 patients with congenital AS who had successful balloon valvuloplasty were enrolled into this study. All patients were evaluated by 2D, M-mode, Doppler, and tissue Doppler echocardiography before and after the procedure. The data were analyzed using the SPSS statistical software.
The median duration of follow-up was 2 years (range = 6 months to 7.4 years). Besides, the mean of immediate peak-to-peak gradient reduction was 41.50 ± 17.36 mmHg. Moderate to severe aortic insufficiency occurred in less than 28% of the patients. Additionally, M-mode echocardiography showed residual left ventricular hypertrophy in 50% of the patients. Moreover, E∕A ratio was less than -2 Z-scores in 73% of the patients. E/Ea ratio was also more than 2 Z-scores in 50% of the patients, which was an indicator of diastolic dysfunction. The most common associated lesion was bicuspid aortic valve in 48% of the cases.
Balloon aortic valvuloplasty effectively reduced peak systolic pressure gradient across the aortic valve in patients with congenital AS although left ventricular hypertrophy and diastolic dysfunction persisted in a significant number of patients.