Early myocardial functional abnormalities in primary dyslipidemia: clinical and echocardiographic observations in young children from a highly consanguineous population.

BACKGROUND: Dyslipidemia is a major health problem among children and adolescents worldwide due to its significant association with cardiovascular disease. Primary dyslipidemias are commonly familial syndromes that can be completely asymptomatic. PURPOSE: Apart from the risk of coronary artery disease (CAD), limited data are currently available on the direct effects of dyslipidemia on myocardial function in children. METHODS: We recruited 25 children with primary dyslipidemia (14 with isolated hypercholesterolemia, 4 with isolated hypertriglyceridemia, and 7 with combined dyslipidemia). Relevant clinical manifestations and laboratory and radiological investigations were evaluated. Pulsed-wave Doppler and tissue Doppler imaging echocardiography were performed for all recruited patients and the results were compared with those of 15 age- and sex-matched healthy children. RESULTS: The median age of the dyslipidemic children was 8 years (range, 1.5-16 years). A family history was documented in 13 cases (52%), while 18 (72%) had consanguineous parents. None of the dyslipidemic children had a personal history or clinical manifestations of CAD. In contrast, echocardiographic findings differed in several diastolic function parameters of both right and left ventricles in dyslipidemic children compared to controls. Based on normalized z scores, aortic valve narrowing was detected in 7 patients (28%), while narrowing of the aortic sinus (sinus of Valsalva) was detected in 15 patients (60%). CONCLUSION: Different types of primary dyslipidemia produce functional myocardial abnormalities early in childhood. Biochemical and echocardiographic screening of high-risk children is advised to minimize the incidence of serious cardiovascular complications.

The clinical value of ficolin-3 gene polymorphism in rheumatic heart disease. An Egyptian adolescents study.

OBJECTIVE: Ficolin-3 is one of the innate immunity molecules that was thought to play a pivotal role in Streptococcus pyogenes autoimmunity and its complications; rheumatic fever (RF) and rheumatic heart disease (RHD). We aimed to disclose if there is an association between ficolin-3 (FCN3) gene polymorphisms (rs4494157 and rs10794501) and RF with or without RHD for the first time in Egyptian adolescents. RESULTS: Serum ficolin-3 level was significantly elevated in patients suffering from RF with and without RHD in comparison with control. Regarding FCN3 gene (rs4494157) polymorphism, a significant correlation was found between the A allele and the susceptibility to RF with or without RHD (OR = 2.93, P = 0.0002 and OR = 2.23, P = 0.008 respectively). Besides, AA homozygous genotype showed a significant association with RHD risk (OR = 3.47, P = 0.026). Patients carrying the A allele (CA + AA) had significantly higher serum ficolin-3 than those carrying the CC genotype (P ˂ 0.0001). While the frequency of (rs10794501) polymorphism revealed no significant differences between the controls and RF patients with or without RHD (OR = 1.43, P = 0.261 and OR = 1.48, P = 0.208 respectively).

Biventricular reverse remodeling and relationship with mitral valve prolapse after transcatheter closure of ASD secundum, a 3D echocardiographic study.

Introduction: Mitral valve prolapse (MVP) is the most common anomaly of the mitral valve. Several studies have shown prevalence of MVP in atrial septal defect (ASD) especially secundum types (II). The aims of this study is to show the potential role of 3D echocardiography in improving the diagnosis of MVP and to depict the relationship between reverse remodeling of the right and left ventricles (RV, LV) and MVP after transcatheter closure of ASD II. Methods: Sixty patients underwent transcatheter closure of ASD II and completed follow up by 2D and 3D echocardiography in Cairo University Children Hospital before the procedure and at 24 hours, 1 and 6 months after the procedure. Results: 3D echocardiography was more accurate than 2D echocardiography in detecting MVP frequency in ASD II patients (75% vs. 50%). Maximum statistically significant remodeling was detected by 3D echocardiography 1 month after the procedure (RV: LV ratio by 3D echocardiography 1.9±0.03 24 hours after the procedure vs. 1.6±0.03 1 months after the procedure, P <0.01) while 2D echocardiography was delayed in detecting biventricular reverse remodeling. 3D derived RV: LV ratio was accurate in detecting MVP status with a sensitivity of 88%. Conclusion: MVP in ASD II may be related to Biventricular remodeling; 3D echocardiography is accurate in the detection of reverse remodeling as well as MVP in ASD II patients before and after device closure.

New guidelines for diagnosis of rheumatic fever; do they apply to all populations?

BACKGROUND AND OBJECTIVES: To evaluate the efficacy of recently updated Jones criteria for diagnosis of rheumatic fever in high incidence populations like Egypt. METHODS: Clinical data of 891 Egyptian patients with rheumatic fever, aged 5-15 years in a highly specialized rheumatic fever clinic were reviewed retrospectively from March 2014 to March 2016. Discriminant analysis was used to detect the most effective predictors for diagnosis of rheumatic fever in our patients incorporating echocardiographic criteria. We compared our results to the most recent update by the American Heart Association. RESULTS: The most effective predictors of rheumatic fever included arthritis, carditis, chorea, aortic regurgitation, grades of mitral regurgitation ≥10mm length and velocity ≥2.5 m/s, thick anterior mitral valve leaflets, elevated acute phase reactants, positive family history and prolonged PR interval. Our predictors showed a high sensitivity of 93%, a specificity of 62% and an overall prediction accuracy of 81.4%. CONCLUSION: We concluded that strict application of updated Jones criteria may lead to under diagnosis of rheumatic fever in highly endemic countries. We recommend further studies to examine the sensitivity of the most recent update of Jones criteria on other highly endemic populations.

Left ventricular mass index and subendocardial myocardial function in children with chronic kidney disease, a transmural strain and three-dimensional echocardiographic study.

Introduction: Left ventricular hypertrophy (LVH) is the commonest myocardial response to chronic kidney disease (CKD); this response has been regarded detrimental as it impairs the blood flow to the deepest layers of the myocardium causing progressive myocardial dysfunction. The aim of these series is to assess the determinants of LVH in CKD patients and its impact on subendocardial function in such patients. Methods: This study has been conducted on 40 CKD patients (Group 1) and 40 age-matched controls, both groups were assessed by transmural echocardiography to determine the subepicardial and subendocardial global longitudinal strain (GLS) as an expression of the systolic function of each of those layers. LVH was assessed by calculation of left ventricle mass index (LVMI). Both groups underwent ambulatory blood pressure monitoring. Group 1 was assessed as regards lipid profile and insulin resistance by homeostasis model assessment of insulin resistance (HOMA-IR). Results: HOMA-IR proved to be a more important determinant of LV hypertrophy than SBP and DBP with a P of 0.01. Moreover subendocardial GLS was negatively correlated with LVMI with r = 0.69 and P < 0.01 denoting the negative effect. LVH plays on subendocardial function probably by impairing myocardial perfusion. Conclusion: This study points toward the importance of insulin resistance in aggravation of myocardial remodeling in CKD patients; more studies are warranted to examine the role of insulin Sensitizers in reversing such remodeling and restoring subendocardial function in such important systemic disorder.

Diagnostic dilemma of cardiac syncope in pediatric patients.

AIMS: Syncope is defined as temporary loss of consciousness and postural tone resulting from an abrupt transient decrease in cerebral blood flow. The present work aimed at determining how diagnostic tests are used in the evaluation of pediatric syncope at a tertiary pediatric referral center and to report on the utility and the yield of these tests. SETTINGS AND DESIGN: Retrospective study conducted at a tertiary referral arrhythmolology service METHODS AND MATERIAL: The clinical charts of 234 pediatric patients presenting with a primary complaint of syncope with an average age of 7.48 +/- 3.82(3.5-16) years were reviewed by the investigators. STATISTICAL ANALYSIS USED: Statistical Package of social science (SPSS) version 9,0 was used for analysis of data. RESULTS: The commonest trigger for syncope in the study population was early following exercise (n=65) and the commonest prodrome was palpitation, noted in 25 patients. A murmur was present in 19 of our patients (8.3%) while 10.7% (n=25) had abnormal ECGs. Of the 106 echocardiograms done, 14 (13.2%) were abnormal. Only two of them were missed by ECG. All patients were offered ambulatory 24 hour ECG. One patient with sick sinus syndrome was diagnosed only with Holter. CONCLUSIONS: Clues to the presence of cardiac syncope may include acute onset of syncope, frequent episodes, low difference between blood pressure readings in supine and erect positions (after standing for 2 minutes) and most importantly an abnormal 12 lead ECG. Transthoracic echo and Holter monitoring have low yield in pediatric syncope.

Nutritional rehabilitation for children with congenital heart disease with left to right shunt.

El-Koofy N, Mahmoud AM, Fattaouh AM. Nutritional rehabilitation for children with congenital heart disease with left to right shunt. Turk J Pediatr 2017; 59: 442-451. Children with congenital heart disease (CHD) are prone to malnutrition. We aimed to assess the nutritional status of infants with CHD and evaluate the role of nutritional counseling. A cohort study included 50 patients with acyanotic CHD causing increased pulmonary blood flow consecutively enrolled from the cardiology clinic in Cairo University Children Hospital. The patients were subjected to clinical evaluation, nutritional assessment with detailed nutritional history, anthropometric measurements and subjective global assessment (SGA) score and complete blood count, serum albumin and total protein assessment. Degree of failure to thrive (FTT) was assessed and nutritional counselling was offered to those patients; followed up for 3 months then re-evaluated. The mean age was (8.6±4.8 months) with male: female ratio 1:1. The overall prevalence of malnutrition was 62%. Fourteen patients had mild FTT and 17 patients had severe FTT [47.1% with mild heart failure (HF) and 47.1% had moderate or severe HF]. All patients with moderate to severe HF had severe FTT and 80% of those with mild HF had severe FTT. Anemia was present in 96% of the patients. Patients with moderate or severe FTT had more severe degree of HF (p < 0.001), more complications (p < 0.001), more frequent hospital admission (p < 0.001) and less optimum breast feeding (p < 0.001). It was found that 76.5% of those with moderate to severe FTT were formula fed (p 0.002) and significantly lower rate of optimum weaning among (p 0.037). There was significant improvement in the anthropometry, the SGA and degree of FTT, level of albumin with decrease in the percent of anemia among the included patients after nutritional rehabilitation. Malnutrition represents serious problem to children with CHD. Nutritional assessment and proper counseling help to improve the nutritional status of those patients.

Assessment of Left Ventricular Volume and Function Using Real-Time 3D Echocardiography versus Angiocardiography in Children with Tetralogy of Fallot.

BACKGROUND: Evaluation of left ventricular (LV) size and function is one of the important reasons for performing echocardiography. Real time three dimensional echocardiography (RT3DE) is now available for a precise non-invasive ventricular volumetry. Aim of work was to validate RT3DE as a non-invasive cardiac imaging method for measurement of LV volumes using cardiac angiography as the reference technique. METHODS: Prospective study on 40 consecutive patients with tetralogy of Fallot (TOF) referred for cardiac catheterization for preoperative assessment. Biplane cineangiography, conventional 2 dimensional echocardiography (2DE) and RT3DE were performed for the patients. A control group of 18 age and sex matched children was included and 2DE and RT3DE were performed for them. RESULTS: The mean LV end diastolic volume (LVEDV) and LVEDV index (LVEDVI) measured by RT3DE of patients were lower than controls (p value = 0.004, 0.01, respectively). There was strong correlation between the mean value of the LVEDV and the LVEDVI measured by RT3DE and angiography (r = 0.97, p < 0.001). The mean value of LV ejection fraction measured by RT3DE was lower than that assessed by 2DE (50 ± 6.2%, 65 ± 4.6%, respectively, p value < 0.001) in the studied TOF cases. There was good intra- and inter-observer reliability for all measurements. CONCLUSION: RT3DE is a noninvasive and feasible tool for measurement of LV volumes that strongly correlates with LV volumetry done by angiography in very young infants and children, and further studies needed.

Evaluation of cardiac functions of cirrhotic children using serum brain natriuretic peptide and tissue Doppler imaging.

BACKGROUND: Cirrhotic cardiomyopathy (CCM) is described as the presence of cardiac dysfunction in cirrhotic patients. In children with chronic liver disease, CCM has been very rarely investigated. THE AIM OF THE STUDY: Is to evaluate the cardiac function of cirrhotic children to identify those with CCM. PATIENTS AND METHODS: Fifty-two cirrhotic patients and 53 age and sex matched controls were assessed using serum brain-type natriuretic peptide (BNP), conventional echocardiography, and tissue Doppler imaging. RESULTS: Patients' mean ages were 7.66 ± 4.16 years (vs. 6.88 ± 3.04 years for the controls). The study included 27 males and 25 females (28 and 25 respectively for the controls). Patients had larger left atrium and right ventricle (RV) (P value 0.05) and increased LV posterior wall thickness than controls (P value 0.04). They had higher late atrial diastolic filling velocity (A) of tricuspid valve (TV) inflow (0.59 ± 0.17 vs. 0.5 ± 0.1 m/s, P < 0.001) and lower ratios between the early diastolic filling velocity (E) and A wave velocity (E/A) of both mitral valve and TV inflow (1.7 ± 0.35 vs. 1.87 ± 0.34 and 1.3 ± 0.3 vs. 1.5 ± 0.3, P < 0.005 and 0.0008, respectively). Patients had significantly longer isovolumic relaxation time of LV (45.5 ± 11.1 vs. 40.5 ± 7.7 ms P 0.008), higher late diastolic peak myocardial velocity (A') (11.8 ± 3.6 vs. 9.5 ± 2.7 ms, P 0.0003) and systolic velocity (S') of the RV (14.5 ± 2.7 vs. 13.2 ± 2.9, P 0.01) and significantly higher myocardial performance index of both LV and RV (P 0.001 and 0.01). BNP levels were significantly higher in cases than controls (5.25 ng/l vs. 3.75 ng/l, P < 0.04) and was correlated with the E wave velocity of the TV (r 0.004) and the E/E' ratio of the RV (r 0.001). None of the clinical or laboratory data were correlated with the BNP level. CONCLUSION: Cirrhotic children have cardiac dysfunction mainly in the form of diastolic dysfunction. There is a need that CCM be more accurately described in children.

The prevalence of congenital heart defects in infants with cholestatic disorders of infancy: a single-centre study.

BACKGROUND: There is deficiency of data about congenital heart defects (CHDs) in cholestatic disorders of infancy other than Alagille syndrome (AGS). We aimed to define the prevalence and types of CHDs in infants with various causes of cholestatic disorders of infancy. METHODS: This cross-sectional study was conducted on 139 infants presenting with cholestasis whether surgical or non-surgical. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt. Full examination and investigations were done in an attempt to reach an aetiologic diagnosis for cholestasis, in addition to a comprehensive echocardiographic study. RESULTS: The age at the onset of cholestasis ranged from 1 day to 7 months. Males constituted 61.2%. Biliary atresia (BA) was diagnosed in 39 patients (28%), AGS in 16 patients (11.5%), 27 patients had miscellaneous diagnoses and 57 cases had indeterminate aetiology. CHDs were detected in 55 patients (39.5%). Shunt lesions were detected in 24 patients (43.6%), pulmonary stenosis in 18 patients (32.7%) and combined lesions in 9 patients (16.4%). Three patients (5.5%) had abnormal cardiac situs. Only seven patients had clinical presentation suggestive of CHD. CHDs were detected in 14 patients with BA (35.9%), 15 patients with AGS (93.7%) and 26 patients in the remaining group (30.9%). CONCLUSION: CHDs are not uncommon among cholestatic infants and are mostly asymptomatic. Echocardiographic examination of cholestatic infants is recommended particularly for patients with BA before undergoing hepatic portoenterostomy as presence of CHD may impact the anaesthetic planning and affect the outcome of hepatobiliary surgery.

Acute lower respiratory tract infection due to respiratory syncytial virus in a group of Egyptian children under 5 years of age.

BACKGROUND AND AIM: Respiratory syncytial virus (RSV) is one of the most important causes of acute lower respiratory tract infections (ALRTI) in infants and young children. This study was conducted to describe the epidemiology of ALRTI associated with RSV among children ≤ 5 years old in Egypt. PATIENTS AND METHODS: We enrolled 427 children ≤ 5 years old diagnosed with ALRTI attending the outpatient clinic or Emergency Department (ED) of Children Hospital, Cairo University during a one- year period. Nasopharyngeal aspirates were obtained from the patients, kept on ice and processed within 2 hours of collection. Immunoflourescent assay (IFA) for RSV was performed. RESULTS: 91 cases (21.3%) had viral etiology with RSV antigens detected in 70 cases (16.4%). The RSV positive cases were significantly younger than other non-RSV cases (mean age 8.2 months versus 14.2 months, p <0.001). RSV cases had significantly higher respiratory rate in the age group between 2-11 months (mean 58.4 versus 52.7/minute, p < 0.001) and no significant difference in the mean respiratory rate in the age group between 12-59 months. More RSV cases required supplemental oxygen (46% versus 23.5%, p < 0.001) with higher rate of hospitalization (37.1% versus 11.2%, p < 0.001) than the non-RSV cases. 97% of RSV cases occurred in winter season (p < 0.001). CONCLUSION: RSV is the most common viral etiology of ALRTI in children below 5 years of age, especially in young infants below 6 months of age. It is more prevalent in winter and tends to cause severe infection.