Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial.

BACKGROUND: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD). METHODS: Children with HF attributable to LVSD were randomized to sacubitril/valsartan versus enalapril to assess the efficacy and safety of sacubitril/valsartan at 52 weeks of follow-up. The primary end point of the study was to determine whether sacubitril/valsartan was superior to enalapril for the treatment of pediatric patients with HF attributable to systemic LVSD, assessed using a primary global rank end point consisting of ranking patients from worst to best on the basis of clinical events such as death, listing for urgent heart transplant, mechanical life support requirement, worsening HF, New York Heart Association (NYHA)/Ross class, Patient Global Impression of Severity (PGIS), and Pediatric Quality of Life Inventory physical functioning domain. The change from baseline to 52 weeks in NT-proBNP (N-terminal pro-B-type natriuretic peptide) was an exploratory end point. RESULTS: A total of 375 children (mean age, 8.1±5.6 years; 52% female) were randomized to sacubitril/valsartan (n=187) or enalapril (n=188). At week 52, no significant difference was observed between the 2 treatment arms in the global rank end point (Mann-Whitney probability, 0.52 [95% CI, 0.47-0.58]; Mann-Whitney odds, 0.91 [95% CI, 0.72-1.14]; P=0.42). At week 52, clinically meaningful reductions were observed in both treatment arms in NYHA/Ross, PGIS, Patient Global Impression of Change, and NT-proBNP, without significant differences between groups. Adverse events were similar between treatment arms (incidence: sacubitril/valsartan, 88.8%; enalapril, 87.8%), and the safety profile of sacubitril/valsartan was acceptable in children. CONCLUSIONS: In this study, sacubitril/valsartan did not show superiority over enalapril in the treatment of children with HF attributable to systemic LVSD using the prespecified global rank end point. However, both treatment arms showed clinically meaningful improvements over 52 weeks. REGISTRATION: URL: https://www.clinicaltrials.gov; Unique identifier: NCT02678312.

Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Leukotriene B4 levels in CHD-associated paediatric pulmonary hypertension.

BACKGROUND: The aim of this study is to evaluate the role of leukotriene B4, an inflammatory mediator, in the development of pulmonary hypertension in paediatric patients with CHD with left-right shunt. METHODS: The study included forty patients with CHD with left-right shunts. Based on haemodynamic data obtained from cardiac diagnostic catheterisation, 25 patients who met the criteria for pulmonary arterial hypertension were included in the patient group. The control group comprised 15 patients who did not meet the criteria. The standard cardiac haemodynamic study was conducted. Leukotriene B4 levels were assessed in blood samples taken from both pulmonary arteries and peripheral veins. RESULTS: The median age of patients with pulmonary arterial hypertension was 10 months (range: 3-168), while the median age of the control group was 50 months (range: 3-194). In the pulmonary hypertension group, the median pulmonary artery systolic/diastolic/mean pressures were 38/18/24 mmHg, compared to 26/10/18 mmHg in the control group. Leukotriene B4 levels in pulmonary artery blood samples were significantly higher in the pulmonary arterial hypertension group compared to the controls (p < 0.05). Peripheral leukotriene B4 levels were also elevated in the pulmonary arterial hypertension group in comparison to the control group, though the difference was not statistically significant. CONCLUSION: The discovery of elevated leukotriene B4 levels in pulmonary artery samples from paediatric patients with pulmonary arterial hypertension secondary to CHD with left-to-right shunt suggests that local inflammation may have a pathological role in the development of pulmonary arterial hypertension.

The Evaluation of Troponin I Levels and Myocarditis in Children with COVID-19: A Pediatric Single-Center Experience.

Children with COVID-19 usually show milder symptoms than adults; however, a minority of them may have cardiac involvement. We aimed to identify the role of troponin I levels that may predict early cardiac involvement in children with COVID-19. A single-center retrospective study was conducted to evaluate hospitalized children diagnosed with COVID-19 between March 11, 2020, and December 31, 2021. Patients with available troponin I levels and with no known cardiac disease were included. During the study period, 412 children with COVID-19 who had troponin I levels on admission were identified. Troponin levels were elevated in 7 (1.7%) patients and were normal in 395 (98.3%) patients. The median age of patients with elevated troponin levels was 4 (min. 2-max. 144) months, which was statistically lower than the age of patients with normal troponin levels (P = 0.035). All the patients with elevated troponin levels had tachycardia. Out of 7 patients with high troponin levels, 3 (42.9%) of them were admitted to the pediatric intensive care unit (PICU), 2 (28.6%) required oxygen support, and 1 (14.3%) required a mechanical ventilator. Patients with elevated troponin levels had a statistically longer hospital stay (P < 0.001). Neutropenia, tachycardia, PICU admission, oxygen support, and mechanical ventilation were statistically more common in patients with elevated troponin levels (P values were 0.033, 0.020, < 0.001, 0.050, and < 0.001, respectively). Electrocardiography (ECG) and echocardiography (ECHO) were performed on all patients with elevated troponin levels, and 6 (85.8%) patients were diagnosed with myocarditis. The ECG and ECHO have been performed in 58 (14.3%) out of 405 patients with normal troponin levels. Two (3.5%) patients had negative T waves on ECG, and all ECHOs were normal. Our results suggest that elevated troponin I levels in children with COVID-19 can be used to evaluate cardiac involvement and decide the need for further pediatric cardiologist evaluation.

Turkish validity and reliability study of Pediatric Quality of Life Inventory (PedsQL) 3.0 cardiac module for 8-18 years old children and parents.

BACKGROUND: This study aimed to evaluate the Turkish validity and reliability of the 8-12- and 13-18-years child and parent forms of the Pediatric Quality of Life Inventory (PedSQL) 3.0 Cardiac Module. METHODS: This methodological study was conducted in children (8-18 years old) with cardiac disease and their parents. The PedsQL 4.0 were also used to collect data for the parallel form method. Pearson correlation coefficients between the scale and its sub-dimensions were evaluated for construct validity. For construct validity, mean scores of children with cardiac disease and healthy children and their parents were compared. Cronbach's alpha coefficient was calculated to evaluate the internal consistency of the items. RESULTS: In this study, 136 children aged 8-12 years and 135 adolescents aged 13-18 years with cardiac diagnosis and their parents participated. The Cronbach alpha coefficients were found to be above 0.80 for all sub-dimensions and the total scale. Correlations between PedsQL 3.0 Cardiac module and PedsQL 4.0 scores were moderate to highly significant. A significant difference was found between the mean scores of the children with cardiac disease and healthy child and parent forms (p < .001). CONCLUSION: PedsQL 3.0 Cardiac Module's 8-12- and 13-18-year child and parent forms are valid and reliable for the Turkish language. APPLICATION TO PRACTICE: It is important to evaluate the PedsQL cardiac module, which is a very comprehensive scale, with accurate measurements to increase the general health level and life satisfaction of these patient groups.

Evaluation for Metastatic Candida Focus and Mortality at Candida-associated Catheter-related Bloodstream Infections at the Pediatric Hematology-oncology Patients.

BACKGROUND: Candidemia and Candida-associated catheter-related bloodstream infections (CRBSIs) are the significant cause of mortality and morbidity in patients with malignancy. METHODS: A retrospective analysis including all pediatric hematologic/oncologic malignancies patients with CRBSIs treated in Dr. Behçet Uz Children Diseases and Surgery Training and Research Hospital between the period of 2009 and 2020. RESULTS: During the study period, 53 children with CRBSIs associated with Candida species were included. The most common malignancy was acute lymphoblastic leukemia (45.3%) and acute myeloid leukemia (15.1%). A total of 56 Candida isolates were present including non-albicans Candida species (80.4%) and Candida albicans (19.6%). The most common isolated Candida species was Candida parapsilosis (42.9%) and followed by C. albicans (19.6%). The ratio of azole prophylaxis was significantly higher in patients with the non-albicans Candida group (P=0.031). Candida-related endocarditis (vegetation) was present in 2 (3.8%) patients, and the overall rate of hepatosplenic candidiasis was 3.8%. Seven days Candida attributable mortality was 7.5% (4 patients) and 30 days Candida attributable mortality was 11.3% (6 patients). The Candida species responsible for the Candida-related deaths were as following: Candida tropicalis (n=3), C. parapsilosis (n=2), and C. lusitanae (n=1). CONCLUSION: In pediatric cancer patients with Candida-associated CRBSIs, evaluation of the patient for organ involvement including liver and spleen ultrasonography and cardiac involvement with echocardiography are essential regardless of the patients' clinical picture.

Endovascular treatment of aortic coarctation with a novel BeGraft aortic stent in children and young adults: a single-centre experience with short-term follow-up results.

OBJECTIVES: We present our experience and outcomes with the BeGraft in the treatment of aortic coarctation in a predominantly paediatric population. METHODS: This study includes a retrospective analysis of patients who had Begraft aortic stent implantation between 2018 and 2020 from a single centre. RESULTS: The BeGraft aortic stent was used in 11 patients (7 males, 4 females) with a median age of 14 (13-21) years and a median weight of 65 (46-103) kg. Coarctation was native in five patients and recurrent in six patients. Median stent diameter and length were 16 mm and 38 mm, respectively. The median peak-to-peak pressure was 30 (12-55) mmHg before the procedure and 5 (0-17) mmHg after the procedure. The stenting procedure was successful in 10 of the 11 patients. Stent migration to the abdominal aorta occurred on post-procedure day 1 in the 21-year-old patient, who had previously undergone surgical closure of the ventricular septal defect and balloon angioplasty for coarctation. After repositioning failed, the stent was safely fixed in the abdominal aorta. Strut distortion also occurred during balloon retrieval in one patient, but no aneurysm or in-stent restenosis was observed at 1-year follow-up. The patients were followed for a median of 14 (4-25) months and none required redilation. CONCLUSIONS: Our initial results demonstrated that the BeGraft aortic stent effectively reduced the pressure gradient in selected native and recurrent cases. Despite advantages such as a smaller sheath and low profile, more experience and medium- to long-term results are needed.

Effectiveness of music during cardiac catheterization on children's pain, fear, anxiety and vital signs: A randomized, blind controlled trial.

PURPOSE: This study aims to examine the effectiveness of music during pediatric cardiac catheterization procedure on children's pain, fear, anxiety and vital signs. DESIGN AND METHODS: This randomized, blind controlled study was conducted with the guidelines of Consolidated Standards of Reporting Trials (CONSORT). The sample of children was allocated to the Control Group, the Classic Music Group, and the Self-Selected Group. Pain, fear, and anxiety levels were measured before and after the pediatric cardiac catheterization procedure. Vital signs were measured before, during, and after the pediatric cardiac catheterization procedure. RESULTS: There was no significant difference between the groups in terms of demographic status, pain, fear, or anxiety levels and vital signs before the procedure. In post pediatric cardiac catheterization measurement in the recovery phase, pain, fear levels, anxiety levels, heart rate, systolic and diastolic blood pressure of children in the musical intervention groups were lower than the control group. No statistical difference was found in terms of pain, fear and anxiety levels of the children in groups Classic and Self-Selected. CONCLUSION AND PRACTICE IMPLICATIONS: The result of this research reveals that musical intervention applied during the pediatric cardiac catheterization procedure in children affects the reduction of pain, fear and anxiety levels and stabilization of vital signs. This intervention could be an effective nonpharmacological method to improve outcomes.Nurses can improve children's comfort by using musical interventions. CLINICAL REGISTRATION: This study was registered at the U.S. National Library of Medicine Clinical Trials (code: NCT04316754).

Infective endocarditis developing early after percutaneous closure of a patent ductus arteriosus in a child using the Amplatzer Duct Occluder II.

A 10-month-old girl underwent patent ductus arteriosus closure with an Amplatzer Duct Occluder II. After 1 week, she was admitted to our emergency room with tachypnoea, fatigue, and fever. Consecutive blood cultures yielded vancomycin-sensitive Staphylococcus aureus. The patient was already receiving vancomycin, but the fever did not respond to this treatment. The device was successfully removed via left lateral thoracotomy.

Orodental, Facial and Clinical Features of Mutation-Positive Noonan Syndrome: A Monocentric Study.

OBJECTIVE: To evaluate orodental, facial, clinical and molecular characteristics of the patients with Noonan Syndrome (NS). STUDY DESIGN: The orodental, clinical and molecular characteristics of 29 mutation-positive patients with NS were recorded. Orodental examination was performed in 17 patients. All exons and exonintron boundries of PTPN11 and SOS1 genes were analyzed by Sanger sequencing. RESULTS: A total of 29 patients with NS from 27 unrelated families were included in the study. Seventeen patients were examined by a specialist in oral medicine. The most common orodental findings were high-arched palate (n=13), gingivitis (n=6) and severe caries (n=6). Anterior open bite, posterior cross bite, Class II malocclusion, hypodontia, prognathism (maxillary or mandibular), macroglossia and gingival hyperplasia were also detected. Thirteen different mutations were observed in PTPN11 gene and exon 3 was the hotspot region. Hypodontia was detected in two patients who had the same mutation in PTPN11 gene, c.181G>A, p.D61N. CONCLUSION: This study indicated a high prevalance of orodental problems including high-arched palate, severe dental caries and gingivitis in patients with mutation-positive NS. The mutation in PTPN11 gene, c.181G>A, p.D61N, may be associated with hypodontia in patients with NS.

Prolonged T-wave peak-end interval in Down syndrome patients with congenitally normal hearts.

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.

Cardiac function in children with premature ventricular contractions: the effect of omega-3 polyunsaturated fatty acid supplementation.

BACKGROUND: Premature ventricular contractions are accepted as benign in structurally normal hearts. However, reversible cardiomyopathy can sometimes develop. Omega-3 polyunsaturated fatty acids have anti-arrhythmic properties in animals and humans.AimWe evaluated left ventricular function in children with premature ventricular contractions with normal cardiac anatomy and assessed the impact of omega-3 fatty acid supplementation on left ventricular function in a prospective trial. METHODS: A total of 25 patients with premature ventricular contraction, with more than 2% premature ventricular contractions on 24-hour Holter electrocardiography, and 30 healthy patients were included into study. All patients underwent electrocardiography, left ventricular M-mode echocardiography, and myocardial performance index testing. Patients with premature ventricular contraction were given omega-3 fatty acids at a dose of 1 g/day for 3 months, and control echocardiography and 24-hour Holter electrocardiography were performed. Neither placebo nor omega-3 fatty acids were given to the control group. RESULTS: Compared with the values of the control group, the patients with premature ventricular contraction had significantly lower fractional shortening. The myocardial performance index decreased markedly in the patient groups. The mean heart rate and mean premature ventricular contraction percentage of Group 2 significantly decreased in comparison with their baseline values after the omega-3 supplementation. CONCLUSION: In conclusion, premature ventricular contractions can lead to systolic cardiac dysfunction in children. Omega-3 supplementation may improve cardiac function in children with premature ventricular contractions. This is the first study conducted in children to investigate the possible role of omega-3 fatty acid supplementation on treatment of premature ventricular contractions.

Echocardiographic evaluation of cardiac functions in newborns of mildly preeclamptic pregnant women within postnatal 24-48 hours.

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.

Neurodevelopmental evaluation of children with cyanotic congenital heart disease.

BACKGROUND: The aim of the study was to perform a neurodevelopmental evaluation of the children with cyanotic congenital heart disease and to determine the factors that affect the neurodevelopmental status. METHODS: The study was performed in the Pediatric Cardiology Department of Behcet Uz Children's Hospital between February and August 2013. Children between the age of six to forty-two months were included in the study and were evaluated in three groups (two patient groups and the control group). In group A, patients with isolated cyanotic congenital heart disease were enrolled. Group B consisted of the patients with cyanotic congenital heart disease with other concomitant diseases. Group C included the healthy control group. For the neurodevelopmental evaluation Bayley Scale of Infant Development- II (BSID-II) was used. Mental Developmental Index (MDI) and Psychmotor Developmental Index (PDI) scores were calculated. Factors possibly effective on neurodevelopment were evaluated. RESULTS: Thirty eight patients (32 in group A and 6 in group B) and 33 healthy subjects in group C were included in the study. Mean age of the patient group was 22.5±11.2 months. In group A mean MDI Score (82.5±14.7) was significantly lower than group C (92.3±6.9) (P=0.001). Similarly mean PDI Score in group A (82.0±18.2) was found significantly lower than group C (92.5±7.4) (P=0.003). When group A and B were compared, mean MDI and PDI scores were lower in group B, but the difference was not statistically significant. For group A, according to the psychomotor development index, 41.6% of the patients were found to be mildly to severely retarded. In terms of the mental development index, 34.4% of the patients had moderate or mild retardation. The sex, socioeconomic status, gestational age, birth weight, comorbidities, history of surgery and reoperation, length of stay in intensive care unit were not found influential on the MDI and PDI scores of the patients. CONCLUSIONS: Mental-motor retardation is frequently encountered in children with cyanotic congenital heart disease. These patients may benefit from motor, language, speech, developmental and educational therapies. For this reason, these children have to be under regular follow up for neurodevelopmental status.

Assessment of early atherosclerosis and left ventricular dysfunction in children with 21-hydroxylase deficiency.

AIM: 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 (ß: 0·5, P = 0·01) and aortic stiffness index (ß: 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.

Global Deformation Parameters Response to Exercise in Adolescents with Repaired Tetralogy of Fallot.

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.

Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

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: ferritin <15 ng/mL (n = 58); Group 2: ferritin 15-25 ng/mL (n = 80); Group 3: ferritin >25 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 was <15 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.

The Impact of 3:1 Ketogenic Diet on Cardiac Repolarization Changes in Children with Refractory Seizures: A Prospective Follow-Up Study.

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.

Assessment of Microvolt T Wave Alternans in Children with Repaired Tetralogy of Fallot during 24-Hour Holter Electrocardiography.

BACKGROUND: We aimed to examine microvolt T wave alternans (MTWA) in 24-hour Holter electrocardiography (ECG) of children with repaired tetralogy of Fallot (TOF) to assess associations of MTWA with ventricular arrhythmias, ECG parameters, and echocardiographic findings. METHODS: Holter ECG records and archive files of 56 repaired TOF patients (62.5% male) who were analyzed retrospectively. Subjects' ECG parameters and MTWA values were compared with age-sex-matched control group. T wave changes were analyzed by time-domain-modified moving average method from the three channels of 24-hour Holter ECG. RESULTS: Mean age was 123.4 ± 48.3 months. Median MTWA value was 55.5 µV in the control group, whereas 95.5 µV in patients group (P < 0.001). A significant weak positive correlation was found between the presence of ventricular extrasystoles and tricuspid regurgitation. There was no correlation between ECG parameters, echocardiographic findings, and MTWA. CONCLUSIONS: MTWA was increased in children with repaired TOF as reported before. To our knowledge, this is the first study analyzing MTWA with 24-hour Holter ECG in repaired TOF patients.

Prolonged Tp-e Interval and Tp-e/QT Ratio in Children with Mitral Valve Prolapse.

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 values >0.05). 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.