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Several surgical techniques have been proposed to repair right partial anomalous pulmonary venous connection (PAPVC) along with sinus venosus defect (SVD). This study aimed to compare the perioperative data and outcomes of double-patch repair using a minimally invasive approach versus conventional sternotomy in pediatric and adult patients.This retrospective study was conducted on 48 minimally invasive cases and 35 sternotomy cases, undergoing surgery by a single surgeon between July 2002 and August 2020. For all patients, repair was performed using the double-patch technique. In the minimally invasive approach, right mini-thoracotomy was performed with central cannulation for children and with peripheral cannulation for adults. The patients were classified into two pediatric and adult groups, and each group was categorized into minimally invasive and sternotomy approaches. They were followed-up by transthoracic echocardiography and electrocardiography before and early after surgery, 3 and 6 months after surgery, and then annually. The relative data were compared between the two approaches in terms of perioperative findings, postoperative pulmonary vein or superior vena cava (SVC) stenosis, and sinus node dysfunction.This study included 25 minimally invasive cases and 19 sternotomy cases in the pediatric group (mean age, 4.99 ± 4.28 and 6.10 ± 4.39 years, respectively) and 23 minimally invasive cases and 16 sternotomy cases in the adult group (mean age, 35.73 ± 8.06 and 32.62 ± 9.80 years, respectively). The mean and median follow-ups were 6.31 ± 4.92 years and 6 years (range: 6 month-18 year) in the pediatric group and 6.15 ± 4.53 years and 5 years (range: 6 month-18 year) in the adult group, respectively. The mean chest tube drainage was significantly lower in the minimally invasive pediatric group (p = .03), and the mean blood transfusion volume was significantly lower in the minimally invasive adult group compared to the other groups (p = .03). No stenosis occurred in the pulmonary veins. Mild SVC stenosis occurred in one patient in the minimally invasive pediatric group, with no need for reintervention. All patients had a normal sinus rhythm, except for the mentioned case with a transient, first-degree atrioventricular block, which spontaneously reverted to the normal sinus rhythm.The minimally invasive approach can be a safe and practical alternative for the double-patch repair of PAPVC and SVD. It ensures a repair with comparable quality to sternotomy, but with better cosmetic and psychological outcomes.

Background: 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. Methods: 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. Results: 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

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.

We reinvestigated the causes, symptoms, and management of childhood pericardial effusion (PE) and its gradual changes during recent years in a referral pediatric cardiology center in the south of Iran.We retrospectively analyzed the profile of PE patients who were under 18 years old from 2015 to 2020. The patient's demographic, clinical, and paraclinical information was extracted and analyzed using SPSS software.In general, 150 out of 63,736 admitted patients (0.23% of the total pediatric admissions) were diagnosed with PE (male/female 1:1.17). The median age was 3.25 years (range:\ 2 days to 18 years; interquartile range: 9.5), and 50% of them were under 3 years of age. 32.6% had moderate to severe PE. Most patients presented with acute symptoms (68%) and respiratory problems, as the most common symptoms (30.6%). Tamponade signs were presented in 2% (Childhood PE is mostly a result of renal failure and noninfectious causes. True pericarditis cases are not common, except in severe cases. It is more common in less than 3-year-old patients, and chronicity is rare. Severe cases had a high chance of pericardiocentesis, but other cases were mainly managed by treatment of the underlying causes.

OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.

Objective Spontaneous closure of the ductus arteriosus often fails to occur in premature newborns and this condition can be associated with increased morbidity and mortality. The initial treatment to achieve closure of the opening is pharmacological, and various nonsteroidal anti-inflammatory drugs may be used. The aim of this study was to determine whether combining acetaminophen with ibuprofen is more effective than the individual use of these drugs to treat patent ductus arteriosus (PDA). Methods The present randomized, controlled trial study included 154 premature newborns with PDA. The patients were randomized into 3 groups: the acetaminophen group (n=67), ibuprofen group (n=68), and combination drug group (n=19). Echocardiography was performed before initiating the medication and after completing a first and second course of treatment. Blood markers were measured to assess the safety of the 3 types of therapy. Results After the first course of treatment, PDA closure was seen in 76.1% of the infants in the acetaminophen group, 76.4% of those in the ibuprofen group, and 78.9% of the combination therapy group (p=0.97). The closure rate after a second course of treatment was 43.7% in the acetaminophen group, 62.5% in the ibuprofen group, and 100% in the combination group. There were no complications attributed to the 3 methods of treatment used. Conclusion Concomitant use of acetaminophen and ibuprofen can be an effective option for closure of PDA. Other studies with a larger sample size are recommended in order to confirm these results.

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 Improving surgical and interventional modalities for treating congenital heart disease increased these children's living expectancy, a leading cause of cognitive and behavioral problems. This study compared executive functions among surgically, interventionally treated congenital heart disease, and aged match control. Methods This was a retrospective cross-sectional study. We enrolled thirty children treated with surgery, 30 interventionally treated patients and 30 healthy controls. We compared these participants in the executive functions domains by Stroop test, trail making test, cancellation test, visual and auditory memory span test, and behavioral problems using the parent form strength and difficulty questionnaire. Executive performance in memory span and sustained attention was lower in the surgical group than in the intervention group (P˂0.05). Results Both groups of patients had lower flexibility, memory recall, simple and complex working memory, visual attention, and simple and complex selective attention performance (P

Background Coronary artery fistula (CAF) is a rare congenital anomaly with a challenging scenario in children. This study reports our experience in transcatheter closure of CAF with Nit-Occlude PDA coil and midterm clinical and imaging follow-up. Methods Twelve children with congenital CAF between 2009 and 2019, mean age 2.05 ± 2.05 years (4 days to 7.2 years), mean weight 8.8 ± 4.83 (2.8–17 kg), who underwent transcatheter closure with PFM coil at the Namazi hospital, Shiraz, Iran, were reported. Echocardiography and electrocardiogram were done before and after the procedure (early, 3, and 6 months after), and Multi-slice computerized tomography or conventional coronary angiography was performed at least one year after closure. Results In a median follow-up of 5.5 years (range 13 months to 8 years), retrogradely closed fistula had no residual, and the fistula tract was wholly occluded, but in most anterogradely closed fistula, had a small residual, which made the fistula tract open and need additional coil closure. Conclusions Transcatheter closure of CAF with PFM coil is feasible and effective with low mortality and morbidity, although antegrade closure with this device may be accompanied by residual shunt and need for multiple coil insertion.

Background Repair of the absence of the whole or major parts of pulmonary arteries is a challenge, and the choice of conduit material to reconstruct the pulmonary arteries is under dispute. We used the autologous innominate vein to construct pulmonary arteries. Case presentation l We present a novel technique using the autologous innominate vein as a free graft in a 6-month-old infant with pulmonary atresia and absence of central pulmonary arteries. Double ductus arteriosus were the only source of perfusion of the lungs. The innominate vein was substituted for the central pulmonary artery between the two lung hila. Total repair by using Contegra graft was performed 9 months later. The patient has been followed for 5 years. Conclusions The autologous innominate vein could be used as inter-hilar pulmonary arteries with no calcification and fibrosis in 5-year follow-up.

Introduction The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Material and methods This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery. Results The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients. Conclusion The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs on its own or with some congenital cardiac disorders, particularly tetralogy of Fallot (TOF), while the hidden pulmonary artery might originate from a closed ductus arteriosus (DA) that can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of our nine TOF patients who had the absence of the left pulmonary artery before the operation. The patients were stratified in three groups: group one, whose closed DA were found and stented successfully to the hidden pulmonary artery; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of the other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgically correction, while the pulmonary vein angiography of the group two patients showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of the group three patients, aged 12, 38, 60, and 63 months. Moreover, evaluation of the other six previously corrected patients who were operated with a right pulmonary artery revealed pulmonary artery hypertension of the entire patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

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 The aim of this study was to collect consistent data on the efficacy and safety and evaluation hepatotoxicity of intravenous acetaminophen for the treatment of PDA in preterm infants. Methods This is an observational longitudinal prospective study on 46 preterm infants with PDA who treated with high dose of acetaminophen and evaluated with echocardiography and serum liver enzymes at Hafez and Zeinabiyeh hospitals from January 2016 to December 2019. Result Forty-six preterm infants with PDA treated with intravenous acetaminophen. Rate of closure of PDA was 82.6. There was no significant difference after treatment regarding AST, ALT, Albumin, total and direct bilirubin (P value > 0.05) and no adverse side effects were observed in association with intravenous acetaminophen. Conclusion High dose of acetaminophen is not more effective than that with standard doses although without hepatotoxic side effect for PDA closure.

Objectives: TOF is the most common cyanotic CHD. We investigated left ventricular (LV) function after surgical pulmonary valve replacement (sPVR) in patients with repaired Tetralogy of Fallot (rTOF) by Speckle Tracking Echocardiography (STE).Methods: 58 volunteers participated in this study who divided into 3 groups including 22 PVR patients (mean age 18.96±7 year), 16 repaired Tetralogy of Fallot and 20 healthy age match control. For all patients, we performed 2D echocardiography and STE.Results: 2D echocardiography in all groups showed normal LV ejection fraction without a significant statistical difference (64% sPVR, 60% in repaired Tetralogy of Fallot and 62.5% in the control group). However, the mean global longitudinal strains (GLS) of LV were significantly reduced in both sPVR (-17.5±2.5%) and repaired Tetralogy of Fallot (-17.1±4.7%) patients rather than control group (-20.2±0.7%) (P = 0.003). But GLS had no statistically significant difference between repaired Tetralogy of Fallot and sPVR patients (P=0.9). Segmental analysis of longitudinal strain (LS) showed a significant decrease in sPVR patients and repaired Tetralogy of Fallot group in basal anterior, basal septal, basal anterolateral segments, mid-anterior and anterolateral segments. Except for lower LS in the apical-anteroseptal segment, this level was mostly spared in both sPVR and repaired Tetralogy of Fallot patients.Conclusion: LVEF was within normal range after sPVR patients, but the pattern of impaired segmental LS and GLS did not change as compared with rTOF. Surgical PVR in patients with repaired TOF may not have a significant effect on the improvement of LV function assessed by STE. LV damage which happens during surgical correction of TOF may have a permanent deteriorating effect on LV function.

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 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.Methods In this retrospective study, the reports of coronary anatomy in angiography and operation notes of TOF patients since 2005 to 2017 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 451 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.Conclusion 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).

Pulmonary arterial hypertension (PAH) related to left-to-right shunt can progress to Eisenmenger syndrome, a serious and fatal disease that is not yet curable. This pilot study considered stem cell injection as a new treatment modality in persistent pulmonary hypertension after the correction of a congenital heart defect. Three patients with persistent pulmonary hypertension after ventricular septal defect repair were included in this pilot study for a clinical trial. Mononuclear stem cells derived from patients’ bone marrow specimens were injected into the right and left pulmonary arteries via cardiac catheterization. The patients were followed over a 6-month period, with six-minute walk test, echocardiography and repeated angiography performed in the sixth month after treatment. The results of the study showed improvement of 40 m, 280 m and 100 m in 6-minute walk distance in patients 1 to 3, respectively. The peak PR gradient decreased 2, 5 and 9 mmHg by echocardiography, and mean PA pressure decreased 21, 22 and 9 mmHg by catheterization in patients 1 to 3, respectively. Pulmonary artery resistance decreased 4, 4.5 and 1.3 Wood units after 6 months of stem cell therapy in the three patients. No short-term complications were detected in this pilot trial, and all patients tolerated the procedure without any complications. Intrapulmonary artery injection of stem cells may have a role in the treatment of persistent PAH secondary to congenital heart disease. This procedure is feasible, with no significant complications, and this study can be considered as a platform for larger studies.

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.

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.

The goal of this study was to compare heart function and arrhythmia in clinically asymptomatic patients with beta thalassemia intermedia and beta thalassemia major.In this cross-sectional study, 60 patients with beta thalassemia major and 60 patients with beta thalassemia intermedia who had clinically no symptoms of arrhythmia and clinically normal heart function were evaluated using 24-hour ambulatory electrocardiogram monitoring and echocardiography. For data analysis SPSS ver.20 software was used. A P-value of less than 0.05 was considered statistically significant.The mean age of the beta thalassemia intermedia patients was 24.18 ± 7.9 years and the mean age in beta thalassemia major was 24.38 ± 7.7 years (P0.05). Premature atrial contractions (PACs) were observed in 14 (23.3%) patients with beta thalassemia intermedia and in 22 (36.6%) beta thalassemia major patients. Premature ventricular contractions (PVCs) were detected in 8 (13.3%) patients in the beta thalassemia intermediate group and 16 (26.6) patients in the beta thalassemia major group, respectively. The left ventricular diastolic dimension, end-diastolic volume, and stroke volume were significantly higher in beta thalassemia intermedia group (P0.05). Pulmonary acceleration time as an indicator of pulmonary pressure was lower in beta thalassemia intermedia group.Both atrial and ventricular arrhythmias were more common in the beta thalassemia major group. Higher end-diastolic volume and stroke volume were detected in the beta thalassemia intermedia group. Pulmonary acceleration time was lower in the beta thalassemia intermedia group, which can be an indicator of higher pulmonary pressure.

Background: Syncope is a common problem among children. Objectives: This cross-sectional study aimed to determine the epidemiological characteristics of children with syncope and clarify the outcome of managements in Imam Reza Pediatrics Cardiology Clinic affiliated to Shiraz University of Medical Sciences, the main referral center for pediatric patients with syncope in south of Iran. Patents and Methods: This cross-sectional, descriptive–analytical study was conducted on all the children referred with transient loss of consciousness and positive head up tilt test. A total of 243 children (132 girls and 111 boys) were recruited from April 2007 to April 2013. The patients’ treatment outcomes were determined through telephone interviews. All the statistical analyses were performed using the SPSS statistical software (version 16.0). Besides, P 3 seconds during syncope. Conclusions: This study indicated that similar to other populations, syncope was more prevalent among girls in our area. Additionally, the most common cause was mixed type of vasovagal syncope. Medical therapy along with offering the patients to increase water and salt intake and do counter pressure physical maneuvers were effective in reduction of the recurrence of syncope.

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.

FH is a genetic disorder characterized by an increase in serum LDL and total cholesterol values. The afflicted patients are at increased risk of premature atherosclerosis and myocardial infarction. Different treatment modalities are present, including pharmacological agents and surgical procedures. The most effective method of therapy in refractive cases is liver transplantation. Herein, we report our experience on 36 cases of patients with FH undergoing liver transplantation in our center, the main referral center of liver transplantation in Iran. The clinical findings, hospital courses, post-operative complications, and patient follow-up are also described.