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Aim:Early repolarization (ER) is a common electrocardiographic (ECG) finding. Although it is thought to be a benign phenomenon, recent studies have shown it to be an important marker of cardiac vulnerability, which can lead to sudden cardiac death. However, there are still conflicting data regarding the prognostic significance of ER in asymptomatic subjects, especially in children. The aim of this study was to investigate specific ECG markers that reflect ventricular repolarization in children with benign ER.Materials and Methods:The study group included 56 healthy children with a benign ER pattern on ECG and a control group was formed of 81 children with normal ECG. Benign ER pattern was defined as terminal QRS notching or slurring accompanied by rapidly ascending ST elevation (>0.1 mV from baseline) in two or more leads. The ECG parameters of QT dis, QTc dis, Tpe dis, Tp-e/QT and Tp-e/QTc were evaluated at rest by a single experienced pediatric cardiologist blinded to the groups.Results:Higher Tp-e, Tp-e dis, Tp-e/QT and Tp-e/QTc measurements were determined in the study group than in the control group. There were no significant differences in the studied ECG parameters with respect to ER location (inferior, lateral, inferolateral), or the ER type (slurring, notching or both).Conclusion:These findings suggest that benign ER in children is associated with the risk of arrhythmogenesis through alterations in ventricular repolarization.

Aim: Cardiac abnormalities associated with celiac disease (CD) have been reported. However, changes in electrocardiographic (ECG) parameters in children with CD have not been previously evaluated in the literature. In this study, we aimed to evaluate these parameters which may be determinative in the development of atrial and ventricular arrhythmia of children with CD. Materials and Methods: Patients diagnosed with CD in the gastroenterology clinic were included in the study. ECG measurements of QT, T peak to end (Tp-e) and P intervals were performed. The Tp-e/QT ratio was calculated from these measurements. Echocardiographic and ECG parameters of the patients and controls were compared. Those patients with elevated anti-tissue transglutaminase immunoglobulin A (IgA-tTG) levels were considered positive serology. The patients were divided in three groups and compared with each other according to ECG parameters; namely newly diagnosed patients, patients with seropositive and patients with seronegative who had been on a gluten free diet for at least 6 months. Results: Fifty-two patients with CD and 59 healthy volunteers were included. P-wave dispersion, QTd, corrected QT dispersion (QTcd), Tp-e dispersion and TP-e/QT ratio were higher in those children with CD compared to the controls. We did not find any correlation between IgA-tTG levels and P-wave, QT and Tp-e dispersions. There was no difference between the three groups of CD patients for ECG and echocardiographic parameters. Conclusion: CD is associated with changes in some ECG parameters, which are considered as predictors of atrial and ventricular arrhythmias in special populations. For this reason, children with CD should be evaluated in terms of these parameters, and more electrophysiological studies are necessary to reveal the clinical and prognostic effects of these parameters for CD.

A young child presented with hepatomegaly, ascites and bradycardia in the setting of COVID-19. Permanent complete atrioventricular block and severe right heart failure were diagnosed. He was treated with surgical epicardial pacemaker implantation. This report is the first description of COVID-19-induced permanent complete atrioventricular block in a child.

ECG is a common diagnostic tool in medical practice. Sudden cardiac death (SCD) is a rare but devastating event. The most common cause of SCD in the young is a primary arrhythmic event, which is often produced by malignant ventricular arrhythmia. Several electrocardiographic markers for ventricular repolarization and depolarization have been proposed to predict this arrhythmic risk and SCD in children. Although many of these parameters can easily be used in clinical practice, some of them need specific techniques for interpretation. In this review, we summarized the current knowledge regarding the clinical importance and the ability of these ECG parameters to predict adverse cardiac events in the pediatric population.

Objectives: Renal transplant recipients are at risk for ventricular arrhythmia and sudden death. To assess that risk, we compared the ventricular repolarization markers of pediatric renal transplant recipients with those of healthy children. Materials and Methods: We included 30 children and adolescents who were followed for at least 6 months after renal transplant; 30 age- and sex-matched children were included for the control group. Demographic features, medications, and laboratory findings were recorded. Blood pressure measurements, ventricular repolarization indexes including QT dispersion, corrected QT dispersion, T-wave peak-to-end interval dispersion, the T-wave peak-to-end interval/QT ratio, the T-wave peak-to-end interval/corrected QT ratio, left ventricular mass index, and relative wall thickness were compared between groups. In addition, the correlations of ventricular repolarization indexes with other variables were evaluated. Results: Blood pressure standard deviation scores, the mean heart rate, QT dispersion, corrected QT dispersion, the T-wave peak-to-end interval/QT ratio, the T-wave peak-to-end interval/corrected QT ratio, left ventricular mass index, and relative wall thickness values were significantly higher in renal transplant patients, whereas T-wave peak-to-end interval dispersion, ejection fraction, and fractional shortening were similar between groups. Although ventricular repolarization indexes were similar in patients with and without left ventricular hypertrophy, only corrected QT dispersion was significantly higher in patients with hypertension (P = .006). The only variable that significantly predicted prolonged corrected QT dispersion was the systolic blood pressure standard deviation score (P = .005, beta = .403). Conclusions: Ventricular repolarization anomalies, hypertension, left ventricular hypertrophy, and cardiac geometry irregularity may be observed after renal transplant in pediatric recipients despite acceptable allograft functions and normal serum electrolyte levels. Control of systolic blood pressure would decrease the risk of ventricular repolarization abnormalities, namely, the corrected QT dispersion. Follow-up of cardiovascular risks with noninvasive methods is recommended in all pediatric renal transplant recipients.

Patients with solitary kidneys (SKs) are at risk of hypertension (HT) and associated end-organ damage. The authors aimed to evaluate whether children with congenital SKs (CSKs) have higher office, ambulatory, or central blood pressure (BP), increased arterial stiffness or left ventricular mass index, or any risk for arrhythmia. With this purpose, patients with CSK and healthy controls being followed up between January 2018 and June 2019 were enrolled in the study. Demographic, biochemical, and office blood pressure (BP) data were recorded. Then, ambulatory blood pressure monitoring (ABPM) and measurements of central BP (cBP), pulse wave velocity (PWV), and augmentation index (AIx@75) were obtained. Ventricular repolarization parameters were acquired by 12-lead electrocardiography. Left ventricular mass index (LVMI) and abdominal aortic stiffness parameters including strain, pressure strain elastic modulus (Ep), and normalized Ep (Ep*) were calculated with echocardiographic measurements. Finally, 36 children with CSK and 36 healthy controls were included. Serum creatinine, uric acid, total cholesterol levels, ABPM parameters, cBP levels, and PWV values were significantly higher, and eGFR levels were significantly lower in the CSK group. VR parameters, abdominal aortic stiffness indices, and LVMI were similar between the groups. CSK increased the risk of HT in ABPM (HTABPM ) by 6 times. PWV was significantly correlated with Ep and Ep* in cases with CSK. Determination of cBP and PWV along with 24-hour ABPM would be a useful tool in children with CSK.

Iron overload is associated with an increased risk of atrial and ventricular arrhythmias. Data regarding the relationship between electrocardiographic parameters of atrial depolarisation and ventricular repolarisation with cardiac T2* MRI are scarce. Therefore, we aimed to investigate these electrocardiographic parameters and their relationship with cardiac T2* value in patients with β-thalassemia major. In this prospective study, 52 patients with β-thalassemia major and 52 age- and gender-matched healthy patients were included. Electrocardiographic measurements of QT, T peak to end interval, and P wave intervals were performed by one cardiologist who was blind to patients’ data. All patients underwent MRI for cardiac T2* evaluation. Cardiac T2* scores less than 20 ms were considered as iron overload. P wave dispersion, QTc interval, and the dispersions of QT and QTc were significantly prolonged in β-thalassemia major patients compared to controls. Interestingly, we found prolonged P waves, QT and T peak to end dispersions, T peak to end intervals, and increased T peak to end/QT ratios in patients with T2* greater than 20 ms. No significant correlation was observed between electrocardiographic parameters and cardiac T2* values and plasma ferritin levels. In conclusion, our study demonstrated that atrial depolarisation and ventricular repolarisation parameters are affected in β-thalassemia major patients and that these parameters are not correlated with cardiac iron load.

Background Recent studies have shown that the Tp-e interval, which on an electrocardiogram (ECG) is the interval between the peak and the end of the T wave, can be used as an index of transmural dispersion of ventricular repolarisation (TDR). Both Tp-e/QT and Tp-e/QTc ratios have also been used in that capacity. However, these novel repolarisation indices have not previously been studied in children with acute rheumaticcarditis (ARC). Methods A hundred and thirty-nine children who were diagnosed with ARC and 153 age- and gender-matched healthy controls were retrospectively reviewed. Twelve-lead ECGs were used to evaluate P-wave, QT and QTc dispersions, Tp-e interval, and Tp-e/QT and Tp-e/QT ratios. Results The mean age of the patients was 10.9 ± 2.4 years. The P-wave, QT and QTc dispersions were significantly higher in patients compared to the healthy control subjects. The Tp-e interval, and Tp-e/QT and Tp-e/QTc ratios were also significantly increased in patients compared to the controls. When the patients were compared in terms of either one- or two-valve involvement, we found no difference between the groups regarding P-wave, QTd and QTc dispersions, Tp-e interval, and Tp-e/QT and Tp-e/QT c ratios. There was no correlation between acute-phase reactants, white blood cell count and these repolarisation parameters. Conclusions This study showed that the new transmural dispersion of ventricular repolarisation parameters, Tp-e interval, Tp-e/QT ratios and QTd were increased in children with ARC. Prolongation of the Tp-e interval and an increased Tp-e/QT ratio might be useful markers for predicting myocardial involvement in children with ARC.

Background Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children with congenital heart disease associated pulmonary arterial hypertension (APAH-CHD) who survived and those who died. Objective The objective of this study to investigate the value of platelet indices with clinical and hemodynamic indicators predicting the disease severity and survival in children with APAH-CHD. Methods This was a nested case-control study. MPV, platecrit, and PDW levels measured in 37 patients with APAH-CHD and 43 healthy subjects at the beginning of the study. Right heart catheterization was performed in all 37 patients. Clinical and hemodynamic data were collected. All patients were followed from the date of laboratory testing. The study was conducted between March 2012-July 2015. The comparison of clinical, hemodynamic data and platelet indices were made between patients with APAH-CHD who died than APAH-CHD patients who survived. Results Of 37 patients, after a mean follow-up duration of 67.90 ± 47.90 months, 11 patients died. MPV (12.10 femoliter [fL; 8.20-12.50] vs 8.70 fL [6.40-9.70], P = .007), PDW (16.88 ± 1.09% vs 15.75 ± 1.58%, P = .04) and platecrit (0.28 ± 0.31 vs 0.22 ± 0.27, P = .01) were significantly higher in the patients with APAH-CHD who died than those who survived. Pearson's correlation analysis showed that MPV correlated with mean pulmonary artery pressure (r = 0.332, P = .04) and correlated negatively with six-minute walking distance (r = -0.600. P = .00). PDW and platecrit correlated positively with mean pulmonary artery pressure (r = 0.373, P = .02; r = 0.389, P = .01, respectively). Conclusion Our results showed that MPV, platecrit and PDW were increased in children with APAH-CHD. They might give clue about disease severity.

Both an excess of iron and iron deficiency (ID) may lead to significant cardiac problems. Parameters that represent ventricular repolarization heterogeneity, like QT dispersion (QTd), corrected QT dispersion (QTcd), the interval between the peak and the end of the T wave (Tp-e), and Tp-e dispersion, have not been evaluated in otherwise healthy children with low iron levels before. Here we assessed the effects of low iron storage on P wave dispersion (PWd), QTd, Tp-e intervals, and Tp-e dispersion in otherwise healthy children. We prospectively reviewed 283 patients who were referred to pediatric cardiology department for cardiac evaluation due to murmurs and who were found to have no structural heart disease. The patients were divided into three groups according to their ferritin levels: Group 1: ferritin15 ng/mL (n = 58); Group 2: ferritin 15-25 ng/mL (n = 80); Group 3: ferritin25 ng/mL (n = 145). P wave duration (PW), QT and Tp-e intervals, and PW, QT, corrected QT (QTc), and Tp-e dispersions were significantly higher in patients whose ferritin level was15 ng/mL. A negative correlation was found between ferritin level and QT and QTc intervals, and QT, QTc, and Tp-e dispersions. Our results showed that a low serum ferritin level is associated with changes in some ECG parameters such as prolonged PWd, Tp-e interval, QT, QTc, and Tp-e dispersions in otherwise healthy children, and studies of other populations indicated that these parameters may predict arrhythmias in selected patients. These patients may be considered at some risk of developing arrhythmias. Therefore, careful evaluation of these ECG parameters is necessary in otherwise healthy children with low iron stores.

Objective: There is no established current algorithm of treatment and clinical follow-up in children with coronary artery anomalies. We analysed the clinical characteristics, diagnosis treatment options of children and adolescents identified with coronary artery anomalies Method: In this retrospective study, 4000 children who attended to our clinic were included between January 2016 and January 2018 at paediatric cardiology department. We reviewed the medical records of 12 patients who had suspicion of coronary-artery anomalies by transthoracic echocardiography. Results: Twelve cases were detected to have coronary artery anomalies. All patients were children-adolescent with a mean age at the time of diagnosis of 10.7 +/- 5 years. None of the patients had cardiac ischemia symptoms. Most common anomaly (5 patients) was the left circumflex coronary artery (Cx) originating from the right coronary artery (RCA). Surgical therapy is recommended in two of 12 patients with interarterial course of coronary artery anomaly. Conclusion: Echocardiography is an important diagnostic tool in childhood due to acoustic window quality. It is crucial to identify children, particularly children with anomalous origin of coronary arteries who will participate competitive sports. Additional imaging tools including computer tomography angiography, magnetic resonance angiography and coronary angiography are needed to confirm the definite diagnosis.

SummaryAim We analysed 25 children with 21-hydroxylase deficiency who received glucocorticoid and/or mineralocorticoid treatment for at least 12 months to determine the effects of the disease and its treatment on vascular structures and ventricular function. Methods Twenty-five patients with 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH) and 25 control subjects were enrolled into this observational, cross-sectional study. The patients were investigated in terms of fasting blood glucose and insulin; fasting serum lipid profile; serum 17-hydroxyprogesterone; dehydroepiandrosterone sulphate; androstenedione; and adrenocorticotropic hormone. M-mode tracings of the wall motion of major arteries were obtained to measure carotid intima-media thickness (cIMT), as well as elasticity and distensibility of the aorta and carotid artery. Conventional and relatively new tissue Doppler imaging techniques were employed to assess ventricular systolic and diastolic functions. Results The median age and weight of patients were 9·4 years (1·5–16·75) and 35·5 kg (7·5–76·3), respectively. The median duration of treatment was 52·2 months. Tissue Doppler imaging measurements revealed left ventricular diastolic impairment in the patient group compared to the controls. Carotid intima-media thickness, stiffness index, elastic modulus of the aorta and carotid artery were significantly higher; meanwhile, aortic distensibility and carotid distensibility were lower in the patient group, all of which indicates the presence of subclinical atherosclerosis. BMI was found to be an independent variable for cIMT (s: 0·5, P = 0·01) and aortic stiffness index (s: 0·52, P < 0·001). Conclusion Cardiovascular function and the elastic properties of major arteries are disturbed in children and adolescents with 21-hydroxylase-deficient CAH.

Exercise stress echocardiography is rarely used in the follow-up of children with congenital heart disease following surgical repair. There are no data on global longitudinal strain (GLS) and global circumferential strain (GCS) during exercise in adolescents with repaired tetralogy of Fallot (TOF). The purpose of this prospective study was to investigate GLS and GCS using speckle tracking two-dimensional echocardiography (2D-STE) at rest and during exercise in adolescents with repaired TOF. Twenty patients with repaired TOF and 20 controls were included. The subjects exercised on a semi-supine cycle ergometer using a two-staged 3-min step protocol of 20-40 W at 60-75 rpm. Exercise images with 2D-STE measurements were available for all 40 subjects. In the study group, the GLS values changed significantly between the baseline and third and sixth minute of exercise (-18.10 ± 2.20, -16.23 ± 4.47, -14.22 ± 2.63, respectively, p 001). GCS did not vary significantly from baseline to end of the exercise testing (-17.49 ± 5.14, -17.15 ± 9.20, -15.66 ± 3.59, respectively, p 0.05). There was a statistically significant difference between the Stage I longitudinal strain values of the study and control groups (-14.26 ± 9.13 and -20.22 ± 2.88, respectively, p = 0.02) but no significant difference between the Stage II values of these groups (-14.22 ± 2.63 and -15.11 ± 2.45, respectively, p 0.05). The results revealed significant changes in GLS during exercise in patients with repaired TOF. Data gathered from deformation analysis of 2D-STE during exercise testing can enhance the ability to detect subtle ventricular dysfunction in the follow-up of children with repaired TOF.

Summary Introduction In this study, we aimed to evaluate Red blood cell distribution width (RDW) in patients with acute rheumatic carditis during the acute phase and after anti-inflammatory therapy. Methods Pediatric patients diagnosed with acute rheumatic carditis (ARC) between 2006 and 2014 and age- and sex-matched controls were retrospectively analyzed. At the time of diagnosis and after 2 months of medical therapy, we reviewed the obtained demographic features; echocardiographic data; complete blood count reports, including RDW; acute phase reactants, including C-reactive protein; and erythrocyte sedimentation rate values. Results The number of the cases with ARC and age- and sex-matched controls were 100 and 110, respectively. The mean age of patients was 11.6 ± 2.5 years. WBC and platelet counts, RDW were found to be significantly higher in patient group compared with controls at the time of diagnosis, prior to the onset of treatment. RDW, platelet count, CRP, and ESR levels significantly decreased after an 8 weeks of medical treatment. RDW values after the medical treatment were still significantly higher compared with controls. RDW values were significantly higher in patients with multiple valvular involvement both prior to and after the treatment. Moreover, we found a significant and positive correlation between the RDW and the severity of mitral regurgitation in our patients (r: 0.46, P < 0.001). Conclusions High levels of RDW after initial medical treatment may indicate an ongoing subtle inflammatory process that leads to future stenotic valvular lesions. However, long-term follow-up studies are needed involving adulthood period to support this hypothesis.

BACKGROUND Heterogeneity of ventricular repolarization has been assessed using the QT dispersion in Down syndrome (DS) patients with congenitally normal hearts. Novel repolarization indexes, that is, T-wave peak-end (Tp-e) interval and Tp-e/QT ratio, however, have not previously been evaluated in these patients. The aim of this study was therefore to evaluate the Tp-e interval and Tp-e/QT ratio in DS patients without congenital heart defects. METHODS Tp-e interval, Tp-e dispersion, and Tp-e/QT ratio were compared between 160 DS patients and 110 age- and sex-matched healthy controls on 12-lead surface electrocardiogram. RESULTS Heart rate, Tp-e interval, Tp-e dispersion, Tp-e/QT and Tp-e/QTc ratios were significantly higher in the DS group than the control group. CONCLUSION Myocardial repolarization indexes in DS patients with congenitally normal hearts were found to be prolonged compared with those in normal controls. Further evaluation is warranted to elucidate the relationship between prolonged repolarization indexes and arrhythmic events in these patients.

The aim of this study is to detect preeclampsia-related cardiac dysfunction within 24-48 hours of delivery in newborns born from preeclamptic mothers. Forty newborns from mildly preeclamptic mothers formed the study group and the control group was formed by 40 healthy newborns. Cardiac function for the groups were evaluated using conventional echocardiography and myocardial performance index (MPI) within the first 24-48 hours of their lifetime and the results of both groups were compared. A significant difference between the groups was observed especially in the PW Doppler MPI measurements (the left ventricle MPI 0.37 ± 0.09 and 0.26 ± 0.11, p .001; the right ventricle MPI 0.29 ± 0.08 and 0.26 ± 0.07, p .035) for the control group and the study group. Elongation in the left and right ventricle MPI was detected to be more significant in terms of comparing systolic and diastolic functions to determine preeclampsia-related cardiac injury in newborns from preeclamptic mothers within the first 24-48 hours of their lifetime. Impact statement Today, the methods which may detect cardiac injury earlier than conventional echocardiographic methods are used for evaluating cardiac functions. Among them, myocardial performance index (MPI) measurement with PW Doppler is the most common ones. While studies are available in the literature evaluating foetal cardiac functions with MPI in foetuses of preeclamptic women, studies evaluating cardiac functions with MPI index within the first 24-48 hours in postnatal period are not available. This is the first study to detect cardiac injury by measuring cardiac functions of the newborns of preeclamptic babies using conventional echocardiography (EF, SF, mitral and tricuspid E/A) and myocardial performance index within the first 24-48 hours of life and compare these values with those of a control group composed of healthy newborns with similar demographic characteristics. According to the results of the study, elongation in right and left ventricle MPI was detected to be more significant compared to systolic and diastolic functions for determining preeclampsia-related cardiac injury in newborns of preeclamptic mothers within 24-48 hours of delivery. Ventricle functions of the newborns of preeclamptic mothers should also be evaluated with MPI measurement besides conventional echocardiographic measurements.

Background There is increasing evidence of clustering of certain cardiac anomalies in some families. The frequency and echocardiographic features of such anomalies among the relatives of patients with bicuspid aortic valve (BAV) or other left-sided cardiovascular anomalies (LSCA) were evaluated. Methods The patients with BAV or any other LSCA and their relatives were enrolled in the study. They underwent an echocardiographic examination. The probands were assessed in three groups: BAV, BAV + coarctation of aorta (CoA), and other LSCA. Their relatives were also grouped and evaluated accordingly. The echocardiographic measurements were standardized by Z-scores. Results Eighty-six probands and 261 relatives were evaluated. The numbers of the patients in the BAV, BAV + CoA, and other LSCA group were 52, 14, and 20, respectively. Any LSCA was determined in 17 (6.5%) of the relatives. Thirteen (5%) had aortic dilatation and the remainder (1.5%) had BAV. Accordingly, BAV incidence among relatives of patients with BAV was found to be 1.9%. A second individual with an LSCA was observed in 12.8% of 86 families investigated. The frequencies of aortic stenosis, aortic regurgitation, aortic stenosis + aortic regurgitation, and aortic dilatation in the patients with BAV were found to be 37.9%, 53%, 25.8% and 48.5%, respectively. In contrast to previous reports, no enlargement was observed in the pulmonary arteries of BAV patients. Conclusions BAV and other LSCA are of clinical significance. Because the clustering of LSCA in some families is observed, we recommend echocardiographic screening of those relatives. If this is not possible, at least it should be achieved for BAV patients.

OBJECTIVE To assess the feasibility of 12-lead electrocardiographic (ECG) measures such as P wave dispersion (PWd), QT interval, QT dispersion (QTd), Tp-e interval, Tp-e/QT and Tp-e/QTc ratio in predicting poor outcome in patients diagnosed with sepsis in pediatric intensive care unit (PICU). METHODS Ninety-three patients diagnosed with sepsis, severe sepsis or septic shock and 103 age- and sex-matched healthy children were enrolled into the study. PWd, QT interval, QTd, Tp-e interval and Tp-e/QT, Tp-e/QTc ratios were obtained from a 12-lead electrocardiogram. RESULTS PWd, QTd, Tp-e interval and Tp-e/QT, Tp-e/QTc ratios were significantly higher in septic patients compared with the controls. During the study period, 41 patients had died. In multivariate logistic regression analyses, only Tp-e/QT ratio was found to be an independent predictor of mortality. CONCLUSION The ECG measurements can predict the poor outcome in patients with sepsis. The Tp-e/QT ratio may be a valuable tool in predicting mortality for patients with sepsis in the PICU.

Background The association between ketogenic diet (KD) and prolonged QT interval, life-threatening ventricular arrhythmias, and sudden death is controversial. Aim We aimed to prospectively evaluate the effect of KD on electrocardiography (ECG) measures in children with refractory epilepsy. Method A total of 70 children with drug-resistant epilepsy who received a KD for at least 12 months were included in the study. The standard 12-lead electrocardiography was performed in all patients before the beginning and in the 12th month of KD. Heart rate, P-wave duration and dispersion, corrected QT interval and QT dispersion, and Tp-e interval were measured. Results All ECG-derived parameters, but P-wave dispersion increased after 12 months of KD compared with the baseline values. However, these changes were not statistically significant. Conclusion A 12-month long 3:1 KD treatment exerts no deleterious effect on cardiac repolarization measures.

Korkmaz HA, Özdemir R, Küçük M, Karadeniz C, Meşe T, Özkan B. The impact of 21-hydroxylase deficiency on cardiac repolarization changes in children with 21-hydroxylase-deficient congenital adrenal hyperplasia. Turk J Pediatr 2019; 61: 228-235. 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH) is associated with cardiovascular risk factors such as, hypertension, obesity, dyslipidemia, and insulin resistance. It is not known whether 21-hydroxylase-deficient CAH is risk factor for atrial and ventricular arrhythmias. The purpose of this study was to compare the 12-lead electrocardiographic measures in patients of 21-hydroxylase-deficient congenital adrenal hyperplasia with those in healthy control subjects matched for age, sex, height, weight and body mass index (BMI). Twenty-five patients with 21-hydroxylase-deficient CAH and twenty-five heathy control subjects were enrolled into this observational, cross-sectional, controlled study. The evaluation consisted of anthropometric measurements, biochemical parameters, and electrocardiographic (ECG) measures. The standard 12-lead electrocardiography was performed in all patients and P-wave dispersion (PWd), QT interval, QTd, QTcd, Tp-e dispersion, Tp-e/QT and Tp-e/QTc ratios were calculated. There were no significant differences in the groups for age, sex, height, weight and BMI (median age 9.4 (1.5-16.75) years, mean weight 37.6±21.5 vs. 27.9±18.3 kg, mean height 125.4±28.9 vs. 114.7±31 cm, mean BMI 21.4±5.7 vs. 18.9±3.4 kg/m2, respectively). P dispersion and Tp-e dispersion were significantly higher in patients of 21-hydroxylase-deficient CAH compared to the healthy subjects (median P dispersion 50 (25) vs. 40 (40) ms, mean Tp-e dispersion 48±15.5 vs. 35.2±17.5 ms). Our study revealed that 21-hydroxylase deficient CAH is associated with high risk of atrial and ventricular arrhythmias in children.

Postnatal closure of the ductus arteriosus is a complicated two-phase process involving functional and structural changes. So far, the precise mechanisms regulating this process are not fully understood. A growing body of evidence from recent studies suggests that platelets play a key role in inflammatory processes including ductal closure via interaction with endothelial cells. The aim of this study is to assess whether a relationship exists between the occurrence and/or closure of hemodynamically significant ductus arteriosus (HSDA) and platelet parameters (platelet count, circulating platelet mass, mean platelet volume, platelet distribution width) in preterm newborns. This single-center, retrospective study included 824 premature infants between 24 and 34 gestational weeks, evaluated by echocardiography at postnatal 72-96 h. Infants with and without HSDA (n = 208 vs. n = 616) were compared in terms of platelet parameters recorded within the first 3 days of life. Oral or intravenous ibuprofen was commenced for medical treatment, and echocardiography was repeated 24 h thereafter to determine ductal closure. No statistically significant difference could be demonstrated between the groups in terms of baseline platelet parameters. HSDA was independently associated with early-onset neonatal sepsis. Thrombocytopenia, low circulating platelet mass, high platelet distribution width, or high mean platelet volume could not be demonstrated as a risk factor for HSDA. None of the platelet parameters had an influence on ductal closure after medical treatment. Unlike most reports in the literature, presence of HSDA was not associated with any platelet parameter in our study. We could not demonstrate an association between any platelet parameter and either persistence or closure after medical treatment.

Objective: The aims of this study were to demonstrate ventricular function changes in patients with congenital hypothyroidism and to investigate whether there is an association between any such changes and thyroid-stimulating hormone (TSH) levels using M-mode and Doppler electrocardiography (ECG) and tissue Doppler imaging (TDI). Methods: Twenty-seven patients 5-30 days of age with congenital hypothyroidism who were scheduled to receive L-thyroxine treatment and 20 healthy newborns were included in this study. Twelve-lead ECG and M-mode TDI recordings of the patient and healthy groups were obtained. The patient group was divided into two subgroups according to TSH level (>100 uIU/mL or 100 uIU/mL and 100 group. Conclusion: Impairment in left ventricular systolic function and increased risk of arrhythmia were observed in newborn infants with congenital hypothyroidism. TSH level was associated with heart rate and interventricular septum velocity.

Although it is considered to be a benign condition, previous studies have shown that a subset of patients with mitral valve prolapse (MVP) may be at risk of ventricular arrhythmia and sudden cardiac death (SCD). Previous studies have suggested that the interval between the peak and the end of the T wave (Tp-e) can be used as a marker for the transmural dispersion of repolarization. Increased Tp-e interval and Tp-e/QT ratio are associated with ventricular arrhythmias and SCD. The aim of this study was to assess alterations in ventricular repolarization by using the Tp-e interval and Tp-e/QT ratio in children with MVP and to investigate their relationships with the degree of valvular regurgitation. This study prospectively investigated 110 children with MVP and 107 age- and sex-matched healthy control subjects. Tp-e interval, Tp-e/QT ratio, and QT and QTc dispersions were measured from a 12-lead electrocardiogram and compared between groups. QT and QTc dispersions, Tp-e interval, and Tp-e/QTc ratio were found to be significantly higher in patients with MVP. A positive correlation was found between Tp-e/QTc ratio and increase in the degree of mitral regurgitation (MR) (p 0.05; r = 0.2). However, the degree of MR was not associated with QT, QTc, or Tp-e intervals; QT, QTc, or Tp-e dispersions; or Tp-e/QT ratio (all p values0.05). Individuals with MVP may be more prone to ventricular arrhythmias due to prolonged QTd, QTcd, and Tp-e interval and increased Tp-e/QT and Tp-e/QTc ratios. Therefore, due to their longer life expectancy, children with MVP should be followed up on regarding life-threatening arrhythmias.

Background: Prolonged Tp-e interval and Tp-e/QT ratio was found associated with sudden cardiac death in different clinical conditions. Granulomatous inflammation of ventricular myocardium can lead to inhomogeneity of ventricular repolarization and ventricular arrhythmia. Novel ventricular repolarization parameters, Tp-e interval, Tp-e/QT and Tp-e/QTc ratios have not been evaluated in patients with sarcoidosis without cardiac symptoms previously. Methods: Sixty-one consecutive patients diagnosed with pulmonary sarcoidosis were retrospectively reviewed. The 12-lead ECG examinations were performed. QTdispersion, Tp-e intervals and Tp-e/QT ratio were measured. Results: Comparison of ECG parameters between two groups showed a significantly higher average heart rate in the patients with sarcoidosis than controls. QTdispersion was higher in patients group. Tp-e interval was significantly prolonged in the study group compared to the control group. Tp-e/QT and Tp-e/QTc ratios were significantly higher in patients with sarcoidosis compared to control subjects . CONCLUSIONS: Tp-e interval, Tp-e/QT and Tp-e/QTc ratios are increased in patients with sarcoidosis without any cardiac symptoms. These parameters might be used as a marker for predicting the ventricular arrhythmias and sudden cardiac death in patients with sarcoidosis.

In this study, we investigated the association between brain natriuretic peptide (BNP) levels and tissue Doppler imaging measurements and also screening for deadly mutations in patients with hypertrophic cardiomyopathy (HCM). We enrolled 20 patients diagnosed with HCM (age:10.7±5 years (1-17), 85% male, weight:42.25±23.10 kg, height:141.80±32.45 cm) and 20 age, gender and body weight-matched control subjects. We performed electrocardiography, transthoracic echocardiography, and tissue Doppler echocardiography in each group, as well as genetic tests (for Arg403Gln, Arg453Cys, Arg719Trp and Arg719Gln mutations in MYH7 Exons 13, 14, 19) and BNP in the patients. The patients were divided into two groups according to the presence (Group 1) or absence (Group 2) of left ventricular (LV) outflow tract obstruction. QTc dispersion and the LV ejection fraction and left atrial (LA) volume index were increased in Group 1. The LA volume index and the mitral and septal E/Ea ratio and septum Z-score were increased while the mitral lateral annulus and septal annulus Ea wave velocities and the mitral and tricuspid E/A ratio were decreased in patients with high levels of BNP compared to those with normal BNP levels. There were no mutations that are associated with increased risk of sudden death found in patients included in this study. In the light of our data, we conclude that such parameters BNP levels above the 98 pg/mL, septal thickness Z-score ˃6, and higher mitral and septal E/Ea ratios can be used for management of patients with HCM according to life-threatening conditions.

Anomalous origin of the left coronary artery from the pulmonary artery is the most common cause of myocardial ischemia and infarction in children and infants. Most of the affected individuals become symptomatic, owing to dilated cardiomyopathy during the infant period. Because of congestive heart failure, pulmonary congestion and respiratory distress can be seen. Herein, we report a 35-day-old infant with anomalous origin of the left coronary artery from the pulmonary artery who presented with respiratory distress and dilated cardiomyopathy to draw attention to this rare surgically treatable anomaly.

We prospectively investigated the efficacy of modified myocardial performance index (mod-MPI) in the assessment of cardiac functions in fetuses with and without an isolated hyperechogenic focus (IHF) in the left ventricle and compared with conventional fetal echocardiography.The study group consisted of 50 fetuses with only an IHF in the left ventricle, without any other cardiac or extracardiac anomalies; 50 fetuses without IHF served as controls. All fetal echocardiographic studies were performed between 20th and 24th weeks of gestation. Left ventricular functions were evaluated with both conventional echocardiographic methods (peak velocity of the aortic valve, mitral E/A ratio, fractional shortening) and mod-MPI.There was no statistically significant difference between the groups in terms of maternal age, BMI or gestational age at the time of examination (p 0.05 for all). No statistically significant differences were found between the findings of conventional echocardiographic measurements and left ventricular mod-MPI between the study and control groups (p 0.05 for all).Fetal left ventricular mod-MPI is not affected by the presence of an IHF in the fetal left ventricle between 20th and 24th gestational weeks and thus it does not need to be assessed in this situation.

Reports state that Down syndrome (DS) patients with congenitally normal hearts might experience the development of cardiac abnormalities such as cardiac autonomic dysfunction, valvular lesions, bradycardia, and atrioventricular block. However, the presence of any difference in terms of P-wave dispersion (PWd) and QT dispersion (QTd) was not evaluated previously. This study prospectively investigated 100 DS patients with structurally normal hearts and 100 age- and sex-matched healthy control subjects. Standard 12-lead electrocardiograms were used to assess and compare P-wave and QT durations together with PWd and QTd. The median age of the DS patients and control subjects was 48 months. Heart rates and P-wave and QT dispersions were significantly greater in the DS group than in the control group (113 +/- A 22.9 vs 98.8 +/- A 16.6 bpm, p < 0.001; 31.3 +/- A 9.5 vs 24 +/- A 8.6 ms, p < 0.001; and 46.6 +/- A 15.9 vs 26 +/- A 9.1 ms, p < 0.001, respectively). A positive correlation was found between PWd and age in the DS patients (p < 0.05; r = 0.2). All children with DS should be followed up carefully with electrocardiography in terms of increased P-wave and QT dispersions even in the absence of concomitant congenital heart disease for management of susceptibility to arryhthmias.

In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p0.05). When compared to controls, operated group had statistically lower [corrected] tricuspid annular plane systolic excursion (p0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

OBJECTIVE: In this study, we aimed to evaluate 3-year mid-term follow-up results that consist of treatment response, frequency of recurrences, predisposing factors that cause recurrence and prognosis in patients with syncope and presyncope. We also aimed to obtain useful information that will contribute to follow-up and treatment of these patients. METHODS: 285 consecutive patients diagnosed with syncope between January 2008 and December 2009 were enrolled into study. Patients files were rewieved retrospectively. Etiology of syncope in these patients were evaluated. 114 patients were diagnosed with vasovagal syncope. RESULTS: Vasovagal syncope 51.2 %, pseudo-syncope 22.1%, syncope with neurological origin 14.4%, cardiac syncope 2,1 % and unexplained syncope 10.2 % were found in 285 patients that were examined for syncope. 114 patients diagnosed with vasovagal syncope. Of those 58.8% female, 41.2% were male. Female to male ratio was 1,4. Mean age at presentation 11.7 ± 2.5 years, the mean age of first syncope 11.3 ± 5.2 years, and the mean duration of follow-up 42.8 ± 3.4 months were found. During the follow-up period recurrence of syncope was found in 31 patients (27.2%). Any effects of sex, absence or persence of prodromal symptoms, response type to tilt table test at the time of diagnosis was not determined on the reccurence and type of syncope. Significant decrease was seen in the number of syncope attacks after the tilt table test. The recurrence of syncope was observed in 15 of 26 patients that were treated with beta blockers,whereas 16 of 88 untreated patients. Reccurence of syncope were found more frequent in patients with drug therapy. CONCLUSION: In childhood period, education and recommendations, increasing fluid and salt intake are successful form of treatment for vasovagal syncope. In patients with recurrent syncope attacks β blocker treatment is used besides the recommendations. But effect of the beta blocker therapy for preventing the recurrence is limited. For this reason, medication should be used only in cases with frequently repeated vasovagal syncope attacks.

The objective of this study was to find out the percentage of preterm infants that needed treatment for patent ductus arteriosus (PDA), when treatment decision was based on clinical signs and symptoms, besides echocardiographic findings.Daily echocardiographic evaluation was conducted in 39 preterms ≤29(6/7) weeks' gestation. Patients with ductus arteriosus were closely followed-up for clinical symptoms of PDA for treatment decision until ductus arteriosus was closed either spontaneously or by treatment.PDA was found in 25 (64%) infants. Mean gestational age and birth weight (BW) of the patients with PDA were 27.8 ± 1.2 and 998 ± 221 g, respectively. PDA closed spontaneously or had minimal ductal shunting before any signs and symptoms attributable to PDA were observed in 16 (41%) infants. Mean ductus size/BW ratio and mean left atrial/aortic root ratio were significantly higher in 9 (23%) symptomatic patients (2.06 ± 0.75 versus 1.32 ± 0.75 mm, p = 0.012 and 1.31 ± 0.52 versus 1.19 ± 0.2 mm, p = 0.043, respectively). PDA closure was observed after the first dose of ibuprofen in six of nine patients.Correlation of clinical signs with echocardiographic findings for the decision of PDA treatment can be appropriate to prevent unnecessary medical treatments.

Hemitruncus vs Tetralogy of Fallot. C. Karadeniz, Y. Yozgat, R. Ozdemir, O. Doksoz, M. Kucuk, M. Karacelik, T. Mese, O.N. Sariosmanoglu, N. Unal. Izmir Dr. Behcet Uz Children’s Hospital, Department of Pediatric Cardiology, _ Izmir, Turkey; Izmir Dr. Behcet Uz Children’s Hospital, Department of Cardiovascular Surgery, Izmir, Turkey. A six year-old girl patient who is diagnosed as tetralogy of fallot with a history of recurrent respiratory tract infection during first year of life, admitted with fatigue, mild cyanosis. On physical examination mild The American Journal of Cardiology MARCH 13e16, 2014 10 IN AND C cyanosis (SO2: 85-88 %), and a harsh grade 3 to 4/6 systolic ejection murmur was detected on the left upper sternal border. Electrocardiogram showed right axis deviation and right ventricular hypertrophy. Echocardiographic examination showed 50 % dextroposition of the aorta, subaortic non restrictive VSD with right to left shunt, pulmonary valve stenosis along with pulmonary infundibular stenosis with 76 mmHg peak systolic gradient. Left pulmonary artery couldn’t visualised. Upon this, diagnostic catheterization was done (Fig.1). Right ventricular angiogram showed main pulmonary with infundibular and valvular stenosis and narrow right pulmonary artery with absence of left pulmonary artery. Aortic root angiogram showed the abnormal orgin of the dilated left pulmonary artery from the ascending aorta. The left pulmonary artery presure was measured as 110/60 mmHg. Medical drug therapy was started for pulmonary hypertension. Abnormal origin of the one pulmonary artery from the ascending aorta (hemitruncus) is very rare congenital anomaly that has been reported previously in small case series. Abnormal orgin of the right pulmonary artery is more frequently reported than abnormal orgin of the left pulmonary artery. Although it is encountered as an isolated anomaly, it can be associated with other congenital anomalies such as tetralogy of Fallot, aortopulmonary window and coarctation of the aorta. Pulmonary hypertension is the most important cause of mortality in this patients. Although unexpected of the pulmonary hypertension in patient with tetralogy of fallot, abnormal orgin of the left or right pulmanary artery from the ascending aorta can lead the severe pulmonary hypertension during the first year of life. For this reason, left and right pulmonary artery should be visualised by the pediatric cardiologists for prevent the irreversible clinical conditions.