Cardiac Abnormalities In Children Research Articles

Right Aortic Arch in Pediatric: Experience of a Center

Background: Congenital aortic arch anomalies encompass various malformations, which can range from asymptomatic to presenting with severe respiratory or gastrointestinal issues. These anomalies may occur alone or alongside other congenital heart defects. Imaging is crucial for identifying these conditions and guiding accurate preoperative decisions. Objectives: This study aimed to assess the frequency of these abnormalities in patients with a right aortic arch, explore the reasons for their referral, and evaluate patient outcomes. Methods: This cross-sectional study was conducted on children under the age of 20 who visited the Congenital Heart Diseases Clinic at Imam Reza Hospital, Iran. We collected data from 277 records of patients with right aortic arch from 2002 to 2022. Data were gathered using a checklist, including echocardiographic results, angiography findings, and other diagnostic modalities for the patients. Statistical analysis was performed using IBM SPSS software, Version 25. Results: In this study, 118 (29.8%) patients had heterotaxia, of which 106 (26.8%) had dextrocardia and were excluded [82 (20.7%) with situs inversus, 21 (5.3%) with situs solitus, and 15 (3.7%) with situs ambiguous]. Among the 277 patients with a right aortic arch, 7 (2.3%) had an aberrant left subclavian artery branch. There were 141 (50.9%) female patients. The trachea and esophagus were compressed. Respiratory symptoms were commonly present in patients with a compressive right arch and aberrant left subclavian artery (Kommerell’s diverticulum), which showed a significant relationship. Congenital major heart anomalies were found in 261 (94.2%) patients, with the most common being right obstructive lesions related to tetralogy of fallot (TOF), seen in 115 (41.5%) patients. Complex congenital heart disease was present in 75 (27.1%) patients. Septal defects were observed in 44 (15.9%) patients, including ventricular septal defect in 39 (14.0%). Nine (3.2%) patients had left obstructive lesions, including coarctation of the aorta. Arterial anomalies, such as patent ductus arteriosus, were present in 14 (5.0%) patients. Sixteen patients had normal heart structures or minor cardiac anomalies. The most common non-cardiac abnormality was musculoskeletal. Finally, 189 (68.2%) patients underwent surgery, and 16 (5.8%) died. Conclusions: More detailed examinations during patient visits, attention to clinical symptoms, and modern imaging can hasten the identification of cardiac abnormalities in children, which plays an important role in the diagnosis and prognosis of patients.

Subclinical cardiac abnormalities in children with biliary atresia correlate with outcomes after liver transplantation.

There are subclinical cardiac abnormalities (SCA) in children with biliary atresia (BA). However, data on the consequences of these cardiac changes after liver transplantation (LT) remain controversial in the pediatric field. We aimed to determine the relationship between outcomes and the subclinical cardiac abnormalities in pediatric patients with BA based on two-dimensional echocardiography (2DE) parameters. A total of 205 children with BA were enrolled in this study. The relationship between 2DE parameters and outcomes, including death and serious adverse events (SAE) after LT, was analyzed by regression analysis. Using receiver operator characteristic (ROC) curves to determine the optimal cut-off values of 2DE parameters for outcomes. Differences in the AUCs were compared using DeLong's test. The Kaplan -Meier method with log-rank testing was used to evaluate survival outcomes between groups. Left ventricular mass index (LVMI) and relative wall thickness (RWT) were found to be independently associated with SAE (OR: 1.112, 95% CI: 1.061 - 1.165, P < 0.001 and OR: 1.193, 95% CI: 1.078 - 1.320, P = 0.001, respectively). The cutoff value of LVMI for predicting the SAE was 68 g/m2.7 (AUC = 0.833, 95% CI 0.727-0.940, P < 0.001), and the cutoff value of RWT for predicting the SAE was 0.41 (AUC = 0.732, 95% CI 0.641-0.823, P < 0.001). The presence of subclinical cardiac abnormalities (LVMI > 68 g/m2.7, and/or RWT > 0.41) was associated with lower patient survival (1-year, 90.5% vs 100.0%; 3-year, 89.7% vs 100.0, log-rank P = 0.001). and higher incidence of SAE events. Subclinical cardiac abnormalities were correlated with post-LT mortality and morbidity in children with BA. LVMI can predict the occurrence of death and serious adverse events after liver transplantation.

Evaluation of left ventricular systolic function in children with sickle cell anemia: contribution of 2D strain.

Cardiovascular involvement is not well studied in children with sickle cell disease. The aim of this study was to evaluate the echocardiographic parameters and to investigate speckle tracking echocardiography (STE) interest in detecting subclinical myocardial impairment of children with sickle cell disease. The study was directed in the echocardiographic laboratory in the military hospital of Tunis between July 2018 and December 2018. 30 patients with sickle cell anemia (SCA) and 30 controls were compared. The echocardiographic measurements were indexed according to body surface. Cardiac output, left ventricular ejection fraction, wall thickness, as well as LV 2-D longitudinal systolic strain were assessed. The SCA Group included 30 patients (11.8 ± 2yrs, sex ratio: 1.31) with homozygous SCA and the C Group included 30 healthy controls (12.7 ± 1,2yrs, sex ratio: 1.27). According to the findings, SCA Group showed significantly larger LV diameter (36.2±2.5mm/m2 vs 29.3±1.3mm/m2, p=0.005). SCA Group also showed lower LV ejection fraction (62%±0.5 vs 65%±5, p=0.001). No significant difference was observed for cardiac output (p=0.4). Otherwise, two-dimensional longitudinal strain of LV was higher in SCA group (-21%±3.07 vs -25%±2.98; p<0.01). Our study highlights several cardiac abnormalities in children with SCA, which could represent a marker of disease severity and point out the importance of the cardiologic screening of these patients.

Proinflammatory and cardiovascular biomarkers are associated with echocardiographic abnormalities in children with HIV taking antiretroviral therapy.

Children with perinatally acquired HIV (PHIV) and taking antiretroviral therapy (ART) have a high prevalence of subclinical cardiac disease. We hypothesized that cardiac disease may be a consequence of dysregulated systemic immune activation driven by HIV infection. We examined cardiovascular and proinflammatory biomarkers and their association with echocardiographic abnormalities in children with PHIV. Cross-sectional analysis of soluble biomarkers from a prospective cohort of children aged 6-16 years with PHIV and age-matched HIV-uninfected comparison group. Cryopreserved plasma samples were used to measure seven soluble biomarkers using multiplex bead assay (Luminex). Multivariable logistic regression assessed how biomarker levels related to cardiac abnormalities. A total of 406 children participated in this study (195 PHIV and 211 HIV-uninfected). Mean [standard deviation (SD)] ages of PHIV and HIV-uninfected participants were 10.7 (2.6) and 10.8 (2.8) years, respectively. Plasma levels of CRP, TNF-α, ST2, VCAM-1 and GDF-15 were significantly higher in the PHIV group compared with uninfected control ( P < 0.001). Among children with PHIV, with one-unit representing one SD in biomarker level, a one-unit increase in CRP and GDF-15, was associated with increased odds of having left ventricular (LV) diastolic dysfunction [adjusted odds ratio (aOR), 1.49 (1.02-2.18; P < 0.040)] and [aOR 1.71 (1.18-2.53; P = 0.006)], respectively. Each one unit increase in GDF-15 was associated with increased odds of LV hypertrophy [aOR 1.84 (95% CI 1.10-3.10; P < 0.021)]. Children with PHIV had higher levels of proinflammatory and cardiovascular biomarkers compared with HIV-uninfected children. Increased CRP and GDF-15 were associated with cardiac abnormalities in children with PHIV.

Predictive factors associated with subclinical altered left ventricular function in children with sickle cell anemia

Sickle cell anemia (SCA) is a hemoglobinopathy that is common worldwide. Cardiac abnormalities in sickle cell anemia are frequent and early. However, they are not well investigated among children. The aim of our study was to evaluate left ventricular (LV) function in children with sickle cell anemia. We conducted a study in the echocardiographic laboratory in the military hospital of Tunis between July 2019 and December 2019. Thirty patients with SCA and fifty-eight controls matched for age and sex without SCA or heart disease, were compared. The echocardiographic measurements were indexed according to body surface. Our study population was divided into two matched groups: Group 1: included 30 patients with homozygous SCA. Group 2: included 30 healthy controls. Group 1 showed significantly larger LV diameter (36.2 ± 2.5 mm/m2 vs. 29.3 ± 1.3 mm/m2, P = 0.005). Group 1 also showed lower LV ejection fraction (62% ± 0.5 vs. 65% ± 5, P = 0.001). Whereas, no significant difference was observed for cardiac output (P = 0.4). Otherwise, two-dimensional longitudinal strain of LV was higher in SCA group (−21% ± 3.07 vs. −25% ± 2.98; P < 0.01). We found a P-value within the limit of significance between the occurrence of certain complications and the alteration of SGL such as the occurrence of osteonecrosis (P = 0.053, R = 0.44), splenomegaly (P = 0.051, R = 0.61), splenic sequestration (P = 0.074, R = 0.34) and dyspnea (P = 0.075, R = 0.38). The influence of hydroxycarbamine treatment on alteration of SGL was close to significance (P = 0.075, R = −0.07). We found a statistically significant influence between the alteration of SGL and the dosage of hydroxycarbamine (P = 0.043, R = −0.46). Our study highlights several cardiac abnormalities in children with SCA, which could represent a marker of disease severity. Our study suggests that hydroxycarbamine treatment may attenuate the alteration of myocardial function in children with SCA.

Cardiac Abnormalities in Children with Pre-Dialysis Chronic Kidney Disease in a Resource-Limited Setting: A Cross-Sectional Observational Study.

Cardiovascular disease is the leading cause of morbidity and mortality in children with chronic kidney disease (CKD). We aim to estimate the prevalence of cardiac abnormalities in children up to age 16 years with CKD and their association with various risk factors. This cross-sectional observational study was conducted on 107 CKD children. We assessed the systolic and diastolic function using 2D echocardiographic evaluation and M-mode measurements of the left ventricle (LV) indexed for BSA and z-scores were calculated. Results were compared with age, sex, stage of CKD, anaemia, estimated glomerular filtration rate (eGFR) and various laboratory parameters. LV diastolic dysfunction was seen in 88%, followed by increased LV dimensions in 33.6%, LV systolic dysfunction in 16%, right ventricle systolic dysfunction in 11.2% while increased pulmonary artery (PA) systolic pressure was seen in 9.3% of cases. LV dimensions correlated directly with parathormone levels and inversely with eGFR, serum calcium and haemoglobin levels. Left ventricular hypertrophy correlated directly with parathormone while inversely with eGFR, serum calcium and haemoglobin. Ejection fraction directly correlated to eGFR and serum calcium while inversely related to parathormone. Left PA pressure directly correlated with age and inversely with eGFR. Right ventricular systolic function assessed by tricuspid annular plane systolic excursion correlated inversely with haemoglobin. LV diastolic dysfunction and increased LV dimensions were the most common cardiac abnormality in children with CKD. LV dimensions correlated directly with parathormone levels and inversely with eGFR, serum calcium and haemoglobin. Diastolic dysfunction positively correlated with serum creatinine and parathormone levels.

Echocardiographic findings in children with obstructive sleep apnea: A systematic review

ObjectiveTo systematically review the incidence of cardiac abnormalities in pediatric patients with obstructive sleep apnea (OSA) in order to assess the utility of preoperative echocardiographic evaluation for patients undergoing surgery. Study designA systematic literature review was performed following the Preferred Reporting Items for Systematic Reviews (PRISMA) guidelines. Data sources were searched from January 1, 1980 to March 25, 2020. Studies that examined echocardiographic findings and polysomnographic data for patients between birth and 18 years of age with polysomnogram-confirmed OSA were included. Studies that included patients with preexisting cardiac, metabolic, or hematologic disorders that could affect hemodynamic parameters were excluded. Included studies were assessed for quality and risk of bias using the U.S. National Institute of Health's Quality Assessment Tools. ResultsThirteen studies met inclusion criteria. Five studies were categorized as high risk of bias, three were categorized as medium risk, and five were categorized as low risk. Study design varied considerably between studies, including heterogeneous classifications of OSA severity, discrepant reporting of echocardiographic parameters, and differing estimations of pulmonary hemodynamics. Significant disagreement regarding the effect of OSA on cardiac function was found between all included studies. ConclusionData demonstrating significant associations between OSA and cardiac abnormalities in children is inconsistent. Echocardiographic abnormalities are inconsistently found and, when present, their clinical significance remains poorly understood. Assessing the utility of preoperative echocardiography in children with OSA requires further investigation with prospective studies utilizing standardized classifications of OSA severity, reporting of echocardiographic parameters, and estimations of pulmonary hemodynamics.

Ventricular hypertrophy and ischaemic changes in Children with Sickle Cell Anaemia and its relationship with the Haemoglobin Concentration in Steady State

Objective: The prevalence and burden of Sickle Cell Anaemia (SCA) in Nigeria are high and they contribute to childhood morbidity and mortality. Chronic anaemia and vaso-occlusion usually involve different organs. The involvement of the heart is a common complication of SCA, thus the need for early detection of cardiac abnormalities in children with SCA. To assess cardiac structure using ECG in children with SCA in steady-state and to determine the relationship between abnormal ECG findings and the participants’ haemoglobin concentration.&#x0D; Material and Method: It was a cross-sectional study done in one of the tertiary hospitals in southeast Nigeria. The study participants were 164 children with SCA in steady state within the ages of 2 -17 years. A 12-lead ECG was carried out on the participants and their haemoglobin concentrations determined. The relationship between the presence of cardiac abnormality and independent variables like haemoglobin concentration, age and gender were analyzed.&#x0D; Result: The prevalence of cardiac abnormality was 59.1%. The commonest cardiac structure abnormality was Left ventricular hypertrophy (LVH) (39.6%). Abnormality in the P-R interval was seen in 14.6%, while ST segment abnormality was seen in 18.3%. The mean Hb of those with abnormal ECG was lower than that of those without abnormal ECG, across each age group. Severe anaemia was a significant (p&lt; 0.01) predictor of LVH. However, there was no significant association between the degree of anaemia and the occurrence of ST-segment (p: 0.26) and PR interval abnormalities (p: 0.52).&#x0D; Conclusion: Cardiac anomalies are common findings in SCA children.

Prevalence of Cardiac Abnormalities in Children with Chronic Kidney Disease: A Cross-sectional Study from a Developing Country.

Improved therapeutic modalities in chronic kidney diseases (CKD) children and consequent extension of life expectancy, draws more attention towards secondary complications. Cardiovascular adaptations precipitating such terminal events, begin in pre-dialysis CKD. Hence, it's imperative to identify modifiable risk factors to direct care and resources in haltering CKD progression, evade long-term dialysis and anticipate kidney transplantation to avert cardiac complications in predialysis period. One hundred and six pre-dialysis patients aged one year to 15 years, with estimated glomerular filtration rate of <90 mL/min/1.73 m2 and proteinuria were included. Patient's history, weight, height and blood pressures (BPs) performed. Left ventricular mass index (LVMI) calculated to correct for patient height to determine raised values of >38.6 g/m2.7 and of left ventricular hypertrophy (LVH) >55 g/m2.7. Shortening fraction and ejection fraction measured to assess systolic function. Diastolic function assessed by Doppler measuring the mitral inflow (e/a) ratio. Hemoglobin, calcium phosphorous product, parathyroid hormone and hypertension measured to assess cardiac risk factor. The total prevalence of cardiac abnormality was found in 66.9% (95% confidence interval [CI] 57.6%-75.2%. Raised LVMI was seen in 64%, among which 34.9% had LVH. Diastolic and systolic dysfunction was found in 12.2% and 11.3% respectively. The cardiac abnormality was more prevalent in CKD grade IV and V. The independent risk factors were anemia and abnormal diastolic BP index which increase the risk for LVH by 3-fold and 5-fold respectively. Proportion of cardiac abnormalities were more prevalent in CKD IV and V. Anemia and diastolic hypertension were independent risk factors for LVH.

Pattern of Cardiac Abnormalities in Children &amp;lt; 5 Years of Age - Experience in a Tertiary Care Hospital

<i>Background</i>: Congenital heart diseases (CHDs) are the most common birth defects, responsible for nearly one-third of all congenital birth defects. The birth prevalence of CHD is reported to be 8-12/1000 live births. Early diagnosis of Congenital as well acquired heart diseases are of paramount important for timely management plan and intervention and thus reduce the mortality and morbidity of childhood heart disease patients. <i>Method</i>: This is a prospective observational study carried out during January 2020 to December’ 2020 at Dhaka Shishu Hospital, Bangladesh. The study population were 100 enrolled cardiac Children who were clinically suggestive of having cardiac disease evaluated through chest X-ray, ECG, Echocardiography and other necessary Investigation. Proper informed consent was taken under the study and purpose of the study was explained. Ethical approval was obtained from the Institutional Ethics Committee. All data were processed, analyzed, and disseminated by MS Office and SPSS version 26 as per need. <i>Results</i>: Among the 100 enrolled cardiac patient 87 were diagnosed to have congenital heart disease and remaining 13% percent presented with acquired heart disease. Out of 87 patients suffering from congenital heart disease 59 (67.81%) had acyanotic heart disease where Ventricular septal defect (VSD) is in the top of the list 27 (31.34%). Cyanotic congenital heart disease was found in 28 (32.19%) where Tetralogy of fallot (TOF) was the most common lesion 9 (10.34%). <i>Conclusion</i>: Childhood Heart diseases are responsible for a significant number of mortality and morbidity. Earlier detection and timely initiative regarding medical or surgical management is of utmost importance to avoid complications, reduce mortality and improve quality of life.

Echocardiographic abnormalities in children and adolescents living with human immunodeficiency virus on highly active antiretroviral treatment.

The availability and use of highly active antiretroviral treatment (HAART) has turned human immunodeficiency virus (HIV) into a chronic disease, allowing patients to live much longer. To report asymptomatic cardiac abnormalities in children and adolescents based on both conventional and tissue Doppler imaging (TDI) echocardiography. One hundred and fifty-one patients on HAART were recruited. Demographic and clinical variables were collected through patient interviews and medical record reviews. Conventional echocardiography and TDI were performed on each patient. Mean age was 13.0 ± 3.2 (4.0-19.0) years. Eightythree patients (55%) were female. Age at diagnosis of HIV infection was 5.7 ± 3.3 years. Age at initiation of HAART was 7.34 ± 3.54 years, while duration of HAART was 59 ± 39.1 months. On conventional echocardiography, three cases of left ventricular (LV) systolic dysfunction, two of pulmonary hypertension and one of minimal pericardial effusion were identified. Calculation of myocardial mass index (MMI) revealed that 16 patients had abnormal values. Twenty-seven (17.9%) patients had evidence of LV diastolic dysfunction and 18 (11.9%) had right ventricular (RV) diastolic dysfunction. Nineteen (12.6%) patients had tricuspid annular systolic velocity of < 9.5 cm/s, indicating asymptomatic RV systolic dysfunction. While few patients had abnormalities such as reduced LV ejection fraction, pulmonary hypertension and minimal pericardial effusion detectable on conventional echocardiography, a larger proportion of patients had subtle abnormalities such as increased MMI, LV diastolic dysfunction on TDI, RV dysfunction and abnormal myocardial performance index. Such patients may need routine screening and cardiac follow up.

Importance of Mean Platelet Volume in Predicting Cardiac Mechanics Parameters and Carotid-Intima Media Thickness in Children With End-Stage Renal Disease and Comparison With Healthy Children.

Cardiovascular disease (CVD) is the major cause of death in children with ESRD. Echocardiography and Doppler ultrasound are useful devices for diagnosing cardiovascular abnormalities in such patients. However, they are expensive, difficult to perform as a routine, and not available in many centers. Therefore, finding a more accessible and inexpensive method for CVD evaluation biomarkers is needed. The aim of this study was to evaluate the relationship between mean platelet volume (MPV) as a routine hematological parameter with cardiac mechanics characteristics in children with ESRD. Forty-two children under dialysis and 60 age- and sex-matched healthy subjects as control group were enrolled in the study. Carotid-intima media thickness (CIMT) and echocardiographic parameters were measured in both groups. In addition, hematological and biochemical variables were evaluated in blood samples of participants. MPV was significantly higher in patients than in controls. CIMT, left ventricular mass index (LVMI), end diastolic diameter, strain rate, and global longitudinal strain were significantly different between the two groups. MPV was positively correlated with LVMI and inversely with ejection fraction. In receiver operating characteristic (ROC) curve analysis, the area under the ROC curve (AUC) values for MPV in predicting left ventricular hypertrophy (LVH) and abnormal CIMT were 0.65 (P = 0.07) and 0.53 (P = 0.74), respectively. MPV was correlated with some cardiac abnormalities in children with ESRD. However, it could not show appropriate predictive values in diagnosing LVH and subclinical atherosclerosis. Further studies with prospective design could shed more light in this topic.

Left ventricular functions in children with newly diagnosed Graves’ disease. A single-center study from Upper Egypt

Children with newly diagnosed GD may have significant subclinical changes in LV structure and function (diastolic and global). TDI is more sensitive than conventional Doppler in detecting LV dysfunction. These findings highlight the importance of early monitoring of children with GD for left ventricular mass index and diastolic function. What is Known: • There is an increased risk for cardiac abnormalities in children with Graves' disease (GD). • Limited studies assessed left ventricular function in patients with GD. What is New: • Children with newly diagnosed GD may have significant subclinical changes in left ventricular structure and functions. • Children with newly diagnosed GD should be monitored for left ventricular mass index and diastolic function.

Longitudinal Analysis of Echocardiographic Abnormalities in Children With Sickle Cell Disease.

Cardiac abnormalities have been described in echocardiograms of children with sickle cell disease (SCD). However, longitudinal studies investigating progression of echocardiographic abnormalities across the pediatric age spectrum in SCD are lacking. A retrospective longitudinal analysis of 829 echocardiograms from pediatric patients with SCD at steady-state was performed. Left heart parameters included left ventricular end-systolic, end-diastolic diameters, fractional shortening, and mass. Right ventricular pressure was estimated by tricuspid regurgitation gradient. Tricuspid regurgitation gradient ≥25 mm Hg, a z-score ≥2 for LV parameters and ≤-2 for left ventricular fractional shortening were considered abnormal. Kaplan-Meier analysis revealed that echocardiographic abnormalities were detected by 5 years of age, and the cumulative incidence progressively increased throughout childhood. Age, male gender, HbSS and Sβ thalassemia genotype, white blood cell count, platelet count, total bilirubin, admissions for pain crises and acute chest syndrome were positively, whereas hemoglobin was negatively associated with cardiac abnormalities. Cardiac abnormalities began early in childhood and progressively increased with age. Our study highlights the high cumulative incidence of cardiac abnormalities in children with SCD, which could represent a marker of disease severity.

Using N-terminal pro-B-type natriuretic peptide to diagnose cardiac abnormalities in children with dyspneaen with dyspnea

Background. Dyspnea could be caused by various reason, one of which is the presence of cardiac abnormality. Physical examination sometimes difficult to distinguish breath caused by heart abnormalities, especially small children, so we need another way to find out.Objective. To evaluate whether the examination of NT-proBNP levels can be used as a screening tool to diagnose cardiac abnormality in children presenting with dyspnea.Methods. A Cross sectional study was conducted from August to October 2015 on pediatric patients aged 1 month to 18 years presenting with dyspnea in pediatric ward Mohammad Hoesin Hospital Palembang. All subjects performed blood sampling for NT-proBNP examination and echocardiography to assess the presence of cardiac abnormalities. The diagnostic value analyzed by ROC curve, and determined the optimal cut-off point, sensitivity and specificity. Result. We obtained 58 subjects with median age 9.5 (1-180) months, consisted of 39 subjects with cardiac abnormalities and 19 subjects without cardiac abnormality. There is a significant difference (p = 0.002) of NT-proBNP levels in both groups with a median 1,775 (189-9,000) pg/ml vs 759 (245-9,000) pg/ml. In ROC curve analysis, AUC value was 0.75, and at the optimal cut-off point 1,235 pg/ml, sensitivity was 74.4% and specificity was 73.7%.Conclusion. The level of NT-proBNP can be used to diagnose cardiac abnormalities in children presenting with dyspnea.

Structural Cardiac Abnormalities in Children with Congenital Malformations in Lagos

Background: Congenital heart disease has a great impact on a child’s morbidity and mortality as well as on the health systems cost. They represent the main cause of death among children with congenital malformations. Method: The study was prospective, cross-sectional involving consecutive subjects from two centers which were; a tertiary hospital, a private hospital and a major cardiology center. Children with congenital malformations had transthoracic echocardiography done by a cardiologist. Data was analyzed using Microsoft excel software supplemented with Statistical Package for Social Sciences (SPSS) version 20. Means of continuous variables were compared using the Student t test and proportions using Chi-square test. Level of significance set at p<0.05. Results: A total of 366 children with obvious congenital malformation were recruited in the study. The age range of the patients was between 1 day to 12 years with a mean age of about 6 + 508.03. Majority of the patients were neonates as shown in Table 1. Male to female ratio was 1.1:1. Up to 26.5% of the subjects had a structurally normal heart. The most common congenital heart defect observed observed was atrial septal defect (14.8%) followed by isolated ventricular septal defect (14.1%). Atrioventricular septal defect 13.7%, Patent ductus arteriosus 7.1%, tetralogy of Fallot 4.5%. Some of the patients had more than one cardiac defects. Conclusion: The prevalence of congenital heart defects among children with congenital malformation is very high. Routine screening for cardiac defects in any patients with congenital malformation is advocated to improve quality of life, reduce morbidity and mortality in these subjects.

Magnetic resonance tissue phase mapping demonstrates altered left ventricular diastolic function in children with chronic kidney disease.

Echocardiographic examinations have revealed functional cardiac abnormalities in children with chronic kidney disease. To assess the feasibility of MRI tissue phase mapping in children and to assess regional left ventricular wall movements in children with chronic kidney disease. Twenty pediatric patients with chronic kidney disease (before or after renal transplantation) and 12 healthy controls underwent tissue phase mapping (TPM) to quantify regional left ventricular function through myocardial long (Vz) and short-axis (Vr) velocities at all 3 levels of the left ventricle. Patients and controls (age: 8years-20years) were matched for age, height, weight, gender and heart rate. Patients had higher systolic blood pressure. No patient had left ventricular hypertrophy on MRI or diastolic dysfunction on echocardiography. Fifteen patients underwent tissue Doppler echocardiography, with normal z-scores for mitral early diastolic (VE), late diastolic (VA) and peak systolic (VS) velocities. Throughout all left ventricular levels, peak diastolic Vz and Vr (cm/s) were reduced in patients: Vzbase -10.6 ± 1.9 vs. -13.4 ± 2.0 (P < 0.0003), Vzmid -7.8 ± 1.6 vs. -11 ± 1.5 (P < 0.0001), Vzapex -3.8 ± 1.6 vs. -5.3 ± 1.6 (P = 0.01), Vrbase -4.2 ± 0.8 vs. -4.9 ± 0.7 (P = 0.01), Vrmid -4.7 ± 0.7 vs. -5.4 ± 0.7 (P = 0.01), Vrapex -4.7 ± 1.4 vs. -5.6 ± 1.1 (P = 0.05). Tissue phase mapping is feasible in children and adolescents. Children with chronic kidney disease show significantly reduced peak diastolic long- and short-axis left ventricular wall velocities, reflecting impaired early diastolic filling. Thus, tissue phase mapping detects chronic kidney disease-related functional myocardial changes before overt left ventricular hypertrophy or echocardiographic diastolic dysfunction occurs.

Cardiac abnormalities in children with thalassemia major: correlation of echocardiographic parameters with serum ferritin levels

Background: Thalassemias are a group of inherited disorders of haemoglobin synthesis, with significant morbidity and mortality. The leading cause of death in transfused thalassemia major children is myocardial haemosiderosis. Echocardiographic estimation of left ventricular function may reveal abnormalities before there is any clinical evidence of cardiac disease. The aim of the study was to determine the magnitude and nature of cardiac involvement in children with thalassemia major with respect to various serum ferritin levels. Methods: A prospective study was done on 30 cases of Thalassemia major attending our tertiary referral centre for a period of two yrs. The relationship of blood transfusion and serum ferritin with the parameters PWT-d, PWT-s, LViedd, LViesd, septal thickness, E/A and ES were studied. Results: Out of 30 patients studied, mean age was 4.02 yrs, mean number of transfusions was 28.97 and mean serum ferritin level was 1475. Mean serum ferritin levels of the patients receiving transfusions were estimated in various groups, ferritin levels were higher (2610 ±115.33) in patients who had received more than 50 transfusions.3 of our patients had features of left ventricular hypertrophy in ECG, rest were normal and none of them had evidence of cardiac arrhythmia. Echo parameters were compared; an increase in LViesd was noted in cases when compared to controls which was statistically significant (0.006). Conclusions: Persistent ferritin levels greater than 2500 mcg/ml and assessment of annual blood requirement can assist in prediction of development of cardiac disease. Thus, regular monitoring for cardiac dysfunction and arrhythmias with annual echocardiogram and ECG is extremely important.

Echocardiographic Evaluation of Cardiac Functions in Children with Chronic Adenotonsillar Hypertrophy

Objective: Chronic adenotonsillar hypertrophy (ATH) causing upper airway obstruction may lead to pulmonary hypertension and other cardiac abnormalities. This study aimed at the clinical and echocardiographic evaluation of cardiac abnormalities in children with chronic ATH and evaluation of these abnormalities after adenotonsillectomy. Subjects and method: We studied 40 children with ATH indicated for adenotonsillectomy (group I) compared with another 35 sex and age matched normal children (Group II). Both groups were examined by otorhinolaryngologist and adenotonsillar hypertrophy was diagnosed with lateral neck x-ray. Clinical and echocardiographic examinations were compared in both groups. Adenotonsillectomy were performed in group I and reevaluation of clinical and echocardiographic findings were performed 6 months later in this group and results were compared with the preoperative findings in the same group. Results: Compared with group II, group I has a significantly higher pulmonary artery pressure, right ventricular size, Interventricular septal wall thickness, left ventricular end-diastolic dimension, and longer E-wave deceleration time. Postoperative echocardiographic findings showed statistically significant decrease only in pulmonary artery pressure and E-wave deceleration time. Significant improvement in clinical symptoms and growth parameters were also noted postoperatively in group II. Conclusion: Upper airway obstruction in children with ATH is associated with cardiac structural and functional abnormalities as demonstrated by echocardiography. Some of these abnormalities i.e. pulmonary hypertension and delayed LV diastolic relaxation are reversible after adenotonsillectomy.

Maternal post-absorptive leucine kinetics during late pregnancy in US women with HIV taking antiretroviral therapy: A cross-sectional pilot study

Despite the success of combination antiretroviral therapy (cART) for the prevention of mother to child transmission of HIV, infants exposed to cART in utero frequently are born smaller and have mild cardiac abnormalities. The mechanisms responsible for lower birth weight and cardiac abnormalities in children exposed to cART are unclear but could be related to dysregulation of maternal amino acid metabolism during pregnancy. Previous data in HIV(-) women have shown a relationship between abnormal maternal protein metabolism during pregnancy and low infant birth weight and animal data demonstrate a relationship between altered maternal protein metabolism and increased risk for offspring cardiovascular abnormalities. The objectives of this study were to: characterize post-absorptive maternal leucine kinetics during late pregnancy andexamine the relationships between maternal leucine kinetics and offspring birth weight and cardiac function. Post-absorptive maternal leucine kinetics (evaluated by using stable isotope tracer methodology) in 16 HIV(+) women receiving cART and 14 HIV(-) US women during the 3rd trimester of pregnancy were compared. Relationships between post-absorptive maternal leucine kinetics, cardiac function (echocardiography) and birth weight were statistically examined. Maternal plasma leucine concentration (HIV(-): 82.8 ± 10.7 vs. HIV(+): 72.3 ± 13.5 μM, p=0.06) and leucine oxidation rate (HIV(-): 6.1 ± 1.6 vs. HIV(+): 4.9 ± 1.8 μmol/kgBW/min, p=0.03) were lower in HIV+ women compared to controls. Total leucine turnover rate, non-oxidative leucine disposal rate and post-absorptive maternal glucose and palmitate kinetics did not differ between groups. Left ventricular fractional shortening tended to be lower in children born to HIV(+) compared to controls (HIV(-): 42 ± 1 vs. HIV+: 36 ± 5 %, p=0.08) and associated with lower maternal plasma leucine concentration (r= 0.43, p=0.08). Preliminary results indicate that post-absorptive maternal leucine metabolism during late pregnancy is mildly altered in HIV+ US women taking cART. The clinical significance of maternal leucine metabolism on adverse infant outcomes is unclear and should be further explored in more expansive studies.