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Background and Aims: Syncope is due to brain dysfunction and is a common compliant among children. It accounts for almost 3% of all emergency departments (ED) visits. The aim of this study was to evaluate the QT dispersion in children with syncope in Shahid Beheshti Hospital of Kashan in 2014.Methods: This was a descriptive cross-sectional study performed among 50 children with syncope attacks (case group) referred to the Clinic of Shahid Beheshti Hospital in Kashan during 2014 and 50 healthy children (control group) referred to the center for causes such as a cold or developmental examination. ECG was taken and QT, QTd (QT dispersion), QTc (Corrected QT interval) and QTcd (QTmin-QTmax =QT dispersion) intervals were studied. After proper explanation to the parents, data was obtained from the case and control group and was analyzed using Chisquare, Mann-Whitney, Kolmogorov–Smirnov, Levene›s, Kruskal-Wallis statistical tests.Results: Total, there were 100 children in the study, the participants were divided into two groups of case (n=50) and control (n=50). Among the studied children, a total of 46 of them (46%) were boys. The mean age range of children in the case and control groups were 7.73± 2.33 and 8.09± 2.31 years, respectively (P-value 0.440). The mean QTmax, QTcmax, QTd and QTcd in the case group were 348.80 ± 46.93, 464.94±48.71, 47.80±19.72, 68.36±24.59 ms (millisecond) respectively and 305.28 ±35.52, 395.70 ±50.05, 29.68±13.45, and 45.16±24.46 ms respectively in the control group. There was a significant difference in terms of each of the 4 parameters (P

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.

The aim of this study was to determine the left ventricular myocardial deformation and segmental myocardial dysfunction by speckle tracking echocardiography and tissue Doppler imaging among the operated patients with anomalous origin of the left coronary artery from the pulmonary artery. The study was conducted on 12 patients diagnosed with anomalous origin of the left coronary artery from the pulmonary artery, who had been operated upon between 2001 and 2013 at the medical centres of Shiraz University of Medical Sciences, Shiraz, Iran. The mean age of the patients at the time of surgical correction was 12.6 years ranging from 6 months to 43 years, and the duration of postoperative follow-up was between 1 and 12 years. Comparison of the strain rate between the patients with acceptable ejection fraction and the control group by tissue Doppler imaging showed significant differences between the two groups regarding the lateral wall (p

Background Endothelin-1 is a potent endogenous vasoconstrictor and an important remodeling factor in the pathogenesis of pulmonary arterial hypertension (PAH). Bosentan, a nonselective and active dual endothelin receptor antagonist, is used as a vasodilator in treatment of such patients. This study aimed to evaluate acute response to a single oral dose of bosentan as a vasodilator agent in comparison with nasal oxygen (O2) in patients with PAH related to congenital heart disease (CHD). Materials and Methods We enrolled 20 patients with PAH-CHD, with a mean age of 5.45 ± 4.5 years. Hemodynamic variables were measured at baseline state, after administration of nasal O2 (5 L/min) for 20 minutes, and then when hemodynamic variables returned to the baseline, the measurements were repeated for the third time 3 hours after administration of a single oral dose of bosentan (2 mg/kg). Results Mean pulmonary vascular resistance was 9.92 ± 2.97 Wood units·m2 at baseline and was lowered by O2 to 6.17 ± 2.71 Wood units·m2 (P = .001) and by bosentan to 5.90 ± 2.69 Wood units·m2 (P = .0001). Mean pulmonary artery pressure was 71.2 ± 15.4 mm Hg at baseline and was reduced to 62.6 ± 15.2 mm Hg (P = .001) by O2 and to 61.6 ± 14.8 mm Hg (P = .0003) by bosentan. Conclusion A single oral dose of bosentan has the same acute vasodilatory effect on the pulmonary vascular bed as nasal O2 in patients with PAH related to CHD. Such patients may benefit from long-term therapy with this novel medication.

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

Endothelin-1 is a potent endogenous vasoconstrictor and an important remodeling factor in the pathogenesis of pulmonary arterial hypertension (PAH). Bosentan, a nonselective and active dual endothelin receptor antagonist, is used as a vasodilator in treatment of such patients. This study aimed to evaluate acute response to a single oral dose of bosentan as a vasodilator agent in comparison with nasal oxygen (O2) in patients with PAH related to congenital heart disease (CHD).We enrolled 20 patients with PAH-CHD, with a mean age of 5.45 ± 4.5 years. Hemodynamic variables were measured at baseline state, after administration of nasal O2 (5 L/min) for 20 minutes, and then when hemodynamic variables returned to the baseline, the measurements were repeated for the third time 3 hours after administration of a single oral dose of bosentan (2 mg/kg).Mean pulmonary vascular resistance was 9.92 ± 2.97 Wood units · m(2) at baseline and was lowered by O2 to 6.17 ± 2.71 Wood units · m(2) (P = .001) and by bosentan to 5.90 ± 2.69 Wood units · m(2) (P = .0001). Mean pulmonary artery pressure was 71.2 ± 15.4 mm Hg at baseline and was reduced to 62.6 ± 15.2 mm Hg (P = .001) by O2 and to 61.6 ± 14.8 mm Hg (P = .0003) by bosentan.A single oral dose of bosentan has the same acute vasodilatory effect on the pulmonary vascular bed as nasal O2 in patients with PAH related to CHD. Such patients may benefit from long-term therapy with this novel medication.

The aim of this study was to evaluate the left ventricular systolic and diastolic function before and after transcatheter percutaneous patent ductus arteriosus (PDA) closure. 21 children (age6 months old) diagnosed with hemodynamically significant PDA underwent percutaneous PDA closure. Conventional, Doppler and tissue Doppler imaging and speckled-derived strain rate echocardiography were done at pre-closure, 1 day (early) and 1 month (late) post-closure. Mean age of the patients (female/male: 1.3) was 17.54 ± 24.7 months with the mean PDA diameter of 3.6 ± 0.8 mm. Systolic measures (ejection fraction, shortening fraction) reduced significantly early after PDA closure (P0.05). After 1 month, both improved significantly; ultimately, after 1 month no change was observed in systolic function measures compared with the pre-closure status. Early and late diastolic flow velocities of mitral (E M and A M) reduced considerably in early and late post-closure time (P0.05). Both early tissue Doppler early velocity of lateral mitral annulus (E'M) and early to late velocity ratio (E'M/A'M) of lateral mitral annulus decreased significantly (P = 0.02) in early post-closure. After 1 month, E'M increased considerably. (P = 0.01) but E'M/A'M had an insignificant rise (P0.05). E M/E'M ratio did not change in early post-closure but it had a considerable reduction in the subsequent month compared with the pre- and early post-closure (P0.001 for both occasions). Global and segmental longitudinal strain measures reduced significantly early after PDA closure (P0.05) but it improved remarkably in the subsequent month. Transcatheter PDA closure causes a significant decrease in left ventricular performance early after PDA closure which recovers completely within 1 month. Also PDA size can affect post-closure left ventricular function.