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Background: 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. Objectives: 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). Methods: 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. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Background: Conduction disturbances can occur early after total correction of Tetralogy of Fallot (TOF). Some of these arrhythmias and alterations like complete right bundle branch block are permanent, while some others like Junctional Ectopic Tachycardia (JET) are transient. Objective: This study aimed to evaluate the early post-operative arrhythmias and their risk factors among pediatric patients with TOF after surgical total correction. Methods: This retrospective study was conducted on pediatric patients aged six months to 15 years old (52.5% male and 47.5% female) with TOF who had consecutively underwent surgical total correction during 2015 - 2017. Patients with additional concomitant cardiac operations, previous arrhythmias before the surgery, or any co-existing cardiac anomalies as well as those with severe post-operative complications were excluded. All pertinent data and Electrocardiographic (ECG) documents were recorded several times right after the operation to detect arrhythmias and ECG changes and to find any association between the related risk factors and the occurrence of arrhythmias. Results: Out of the 118 TOF patients who underwent total correction, 27% were under the age of one year and 73% were above one year old. Among the patients, 78.5% developed permanent ECG changes, mostly complete right bundle branch block and left anterior or posterior hemiblock. Out of these patients, 8.5% had JET together with other permanent arrhythmias and 8.5% had transient JET solely. Therefore 17% of all patients developed JET and 21.5% had no permanent noticeable ECG changes. The most permanent arrhythmia was right bundle branch block followed by left anterior hemiblock and left posterior hemiblock. These arrhythmias mainly manifesed amongst the patients with lower age and those with severe subvalvar pulmonary stenosis. The results showed a significant relationship between the incidence of JET and use of milrinon after the operation. There was no myocardial ischemia or infarction from injury to aberrant coronary arteries passing over the right ventricular outflow tract. Conclusion: Early post-operative permanent arrhythmias after total TOF repair was probably more common amongst the patients who were below one year old with more severe subvalvar pulmonary stenosis. Nonetheless, the occurrence of transient JET was mostly observed in the patients who were given milrinone as a post-operative inotrope medicine. [ABSTRACT FROM AUTHOR]

Background: Congenital Aortic Stenosis (AS) is a common problem among congenital heart diseases and balloon valvuloplasty is known as the procedure of choice for treatment. Objectives: This study aimed at evaluation of the function and remodeling of the heart after a successful balloon AS valvuloplasty. Patients and Methods: 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. Results: 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. Conclusions: 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. [ABSTRACT FROM AUTHOR]

Ureteropelvic junction (UPJ) stenosis occurs in 13% of children. Antenatally, the diagnosis is possible by showing renal pelvis dilatation by obstetric ultrasonography. Giant hydronephrosis (GH) is a rare condition. There are different etiologies for GH which are mostly congenital and occur during infancy and childhood. GH was confirmed by complementary evaluations in a 2-month-old female infant with poor feeding, lethargy, and vomiting together with an antenatal sonography in favor of hydronephrosis, and a 5-year-old boy with an abdominal mass diagnosed by sonography following 2 weeks of progressive abdominal distention. Pyeloplasty was associated with an excellent result in both of them. [ABSTRACT FROM AUTHOR]

Background: This study aimed to evaluate the renal tubular function in the patients with congenital heart disease using β²-microglobulin. Methods: In this case-control study, based on oxymetry, the patients with congenital heart disease were divided into two groups of cyanotic (n=20) and acyanotic (n=20). Congenital heart disease was diagnosed by echocardiography. Healthy individuals within the same age and sex groups were used as controls. Na+, β²-micro globulin, creatinine (Cr), and β²-microglobulin/Cr ratio were measured in random urine samples and the results were compared to the same parameters in the control group using Tukey, One-Way ANOVA, and X² tests. Results: Based on the study results, urine sodium in the patients with cyanotic heart disease was significantly different from that of the controls (P=0.023). The results also revealed a significant difference between the two groups with congenital heart disease regarding urine β²-microglobulin (P=0.045). In addition, the patients with cyanotic heart disease were significantly different from those with acyanotic heart disease and the controls regarding urine β²-micro globulin/Cr ratio (P=0.012 and P=0.026, respectively). Conclusions: The results of this study demonstrated that renal tubular dysfunction began in the patients with congenital heart disease, especially in those with cyanotic congenital heart disease. Besides, early diagnosis before cardiac surgery leads to better control of renal tubular disease. [ABSTRACT FROM AUTHOR]

Background: Heart disease is the main cause of mortality and morbidity in patients with beta thalassemia, rendering its early diagnosis vital. We studied and compared echocardiographic findings in patients with beta thalassemia major, patients with beta thalassemia intermedia, and a control group. Methods: Eighty asymptomatic patients with thalassemia major and 22 asymptomatic cases with thalassemia intermedia (8-25 years old) were selected from those referred to Ali Asghar Hospital (Zahedan-Iran) between June 2008 and June 2009. Additionally, 80 healthy individuals within the same age and sex groups were used as controls. All the individuals underwent echocardiography, the data of which were analyzed with the Student t-test. Results: The mean value of the pre-ejection period/ejection time ratio of the left ventricle during systole, the diameter of the posterior wall of the left ventricle during diastole, the left and right isovolumic relaxation times, and the right myocardial performance index in the patients with beta thalassemia major and intermedia increased significantly compared to those of the controls, but the other parameters were similar between the two patient groups. The mean values of the left and right pre- ejection periods, left ventricular end systolic dimension, and left isovolumic contraction time in the patients with thalassemia intermedia increased significantly compared to those of the controls. In the left side, myocardial performance index, left ventricular mass index, isovolumic contraction time, and deceleration time exhibited significant changes between the patients with thalassemia major and those with thalassemia intermedia, whereas all the echocardiographic parameters of the right side were similar between these two groups. Conclusion: The results showed that the systolic and diastolic functions of the right and left sides of the heart would be impaired in patients with thalassemia major and thalassemia intermedia. Consequently, serial echocardiography is suggested in asymptomatic patients with beta thalassemia for an early diagnosis of heart dysfunction and proper treatment. [ABSTRACT FROM AUTHOR]

Background: 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. Methods: 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. Results: 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. Conclusion: 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. [ABSTRACT FROM AUTHOR]

Background: The timing of coronary angiography in patients with non-ST elevation myocardial infarction (NSTEMI) needs to be well defined. In this study, based on the timing of percutaneous coronary intervention (PCI), we evaluated the incidence of major adverse cardiovascular events (MACE) in NSTEMI patients., Methods: In this longitudinal study, we included 156 NSTEMI patients who underwent a PCI at three time points, including <12 hr. (n = 53), 12-24 hr. (n = 54), and ≥24 hr. (n = 49) and followed them for one, three, and six months to monitor major cardiovascular events. The data analyses were conducted using SPSS version 20., Result: Four patients (2.56%) were hospitalized during the one-month follow-up, and only one patient (0.06%) had NSTEMI. The incidence of complications, such as readmission, acute coronary syndrome (ACS; 4 patients [2.56%]), and unstable angina (UA; 3 patients [1.92%]) did not differ significantly among the three intervention times. The occurrence of NSTEMI, UA, and recurrent PCI was 2.56%, 3.20%, and 5.12% in four, five, and eight patients, respectively, and no significant differences were observed among the aforementioned times. In the follow-up after six months, the incidence of STEMI, stroke, TLR, and other all-course deaths was observed in one person (0.06%), which all occurred within 12-24 hours. The difference among the three intervention times was non-significant., Conclusion: Our findings revealed an insignificant difference between the incidence of complications and the three-intervention time., Competing Interests: None., (AJCD Copyright © 2024.)

Background: Heart disease is the main cause of mortality and morbidity in patients with beta thalassemia, rendering its early diagnosis vital. We studied and compared echocardiographic findings in patients with beta thalassemia major, patients with beta thalassemia intermedia, and a control group., Methods: Eighty asymptomatic patients with thalassemia major and 22 asymptomatic cases with thalassemia intermedia (8-25 years old) were selected from those referred to Ali Asghar Hospital (Zahedan-Iran) between June 2008 and June 2009. Additionally, 80 healthy individuals within the same age and sex groups were used as controls. All the individuals underwent echocardiography, the data of which were analyzed with the Student t-test., Results: The mean value of the pre-ejection period/ejection time ratio of the left ventricle during systole, the diameter of the posterior wall of the left ventricle during diastole, the left and right isovolumic relaxation times, and the right myocardial performance index in the patients with beta thalassemia major and intermedia increased significantly compared to those of the controls, but the other parameters were similar between the two patient groups. The mean values of the left and right pre-ejection periods, left ventricular end systolic dimension, and left isovolumic contraction time in the patients with thalassemia intermedia increased significantly compared to those of the controls. In the left side, myocardial performance index, left ventricular mass index, isovolumic contraction time, and deceleration time exhibited significant changes between the patients with thalassemia major and those with thalassemia intermedia, whereas all the echocardiographic parameters of the right side were similar between these two groups., Conclusion: The results showed that the systolic and diastolic functions of the right and left sides of the heart would be impaired in patients with thalassemia major and thalassemia intermedia. Consequently, serial echocardiography is suggested in asymptomatic patients with beta thalassemia for an early diagnosis of heart dysfunction and proper treatment.