mohammad borzouee

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.

 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.

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.

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

Syncope is a common problem among children.. 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 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 < 0.05 was considered to be statistically significant.. The majority of the patients were female (54.3% vs. 45.7%). In addition, the mean age at the first episode of syncope was 13.02 ± 3.9 years and 14.8% of the patients had positive family history of syncope. The mean duration of follow-up was 4.2 ± 3.41 years. Besides, breath holding spell history was positive in 13.6% of the patients. Medical treatment was done for 76.5% of the patients (96.7% propranolol and 3.3% atenolol). Increase of salt and fluid intake and physical maneuvers were performed for management of syncope in 75.3% and 30.9% of the patients, respectively. During follow-up, 66.7% of the patients did not experience any syncope episodes at all. Among the patients, 33.3% were referred by neurologists, while 66.7% had self-referred or referred by pediatricians or family practitioners. Moreover, 80.2%, 8.6%, and 6.2% of the syncope cases were mixed type, inodeppressive, and choronodeppressive, respectively. Furthermore, 35.8% of the tilt tests were positive with nitroglycerin consumption. Also, 11.1% of the patients had asystole > 3 seconds during syncope.. 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..

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. 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. While our patient had systemic pulmonary arterial pressure at the time of complete surgical repair, it was improved after surgery.

Endothelium is an essential organ for maintaining an adequate vascular tone and preventing the pathological process of atherosclerosis. Heart-lung machine is a mechanical support for maintenance of blood circulation during open heart surgery. It has been shown that flow of blood through this circuit can induce complement activation, endotoxines production, and release of many inflammatory mediators.. Cardiopulmonary Bypass (CPB) has some detrimental effects on endothelial function. Flow Mediated Dilation (FMD) is also a noninvasive method for evaluation of endothelial function..Patients and This study was conducted on 22 patients who were admitted in cardiac surgery ward for open heart operation and met the inclusion criteria of the study. Brachial artery FMD was measured the day before and 2 days after CPB.. The mean duration of CPB was 62.95 minutes. The mean percent of FMD changes was measured and compared before and after CPB (4.29 and 0.03 vs. 1.38 and 1.99, < 0.001). The results showed a significant relationship between CPB and bronchial endothelial function.. This study revealed the influence of CPB on endothelial function. Yet, more studies are necessary to confirm this important issue, and decline in use of CPB is appreciated..

Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency disease, predisposing the patients to various tissue involvement and organ damage. Here a 16-year-old boy is presented who was referred to our center with cough, dyspnea, cyanosis, and history of recurrent pneumonia. The diagnosis of CVID was made according to reduction all serum immunoglobulin levels, normal numbers of T, B and NK lymphocyte subpopulations, poor antibodies responses. Considering abnormality in heart examination and chest X-ray, echocardiography and computed tomography angiography were performed which showed large thoraco-abdominal aortic aneurysm in this patient. Although there are some reports of cardiovascular disease associated with primary antibody deficiencies, this is the first time that such large thoraco-abdominal aortic aneurysm is reported in CVID. This may be secondary to recurrent pulmonary infections or an unknown mutation process. Cardiovascular abnormalities are an entity that should be kept in mind in patients with primary immunodeficiency diseases.

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. 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. 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). 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.

The most accepted method of secondary prophylaxis in rheumatic fever (RF) has been an injection of 1,200,000 units of benzathine penicillin G (BPG) every 4 weeks, but recurrences have been reported in some patients despite such a prophylaxis program. The WHO recommended BPG injections every 3 weeks in high risk patients and situations due to some published data in favor of inadequacy of once every 4 weeks injection of BPG. Our study was designed to determine serum penicillin levels (SPL) during the 4 weeks after an intramuscular injection of 1,200,000 units of BPG, in order to find the appropriate interval between BPG injections and also to assess the effects of weight and sex on this level. We included 42 RF patients (mean±SD=14.8±11.9 years) in our study. SPL was determined by disk agar diffusion method. In this study the minimum accepted SPL to be effective against group A β-hemolytic streptococci was 0.02 µg/mL. In 46% of the patients the mean SPL decreased to <0.02 µg/mL at the end of the third week (mean ±SD=2.35 ± 1.3 weeks). The mean SPLs were significantly higher in patients who weighed <45 kg (mean±SD=38.6±4.3 kg) in comparison with those who weighed 2:45 kg (mean±SD=54.25±4.87 kg), with a p value <0.0001. There was no significant differences in mean SPL between boys and girls (p =0.145). Although in this study the mean SPL was <0.02 µg/mL in 46% of patients at the end of the third week, we could not recommend every 3 weeks injections of BPG in all patients, except in high risk patients and situations as recommended by the WHO and also in those patients who weighed 2:45 kg.