Mid-term follow-up by speckle tracking and cardiac MRI of children post-transcatheter closure of large atrial septal defects.

This is a case-control study of our experience of mid-term follow-up of 40 children who had a transcatheter closure of very large atrial septal defects group (1). All cases had an atrial septal defect device size more than 1.5 times their weight, a ratio considered a contraindication for trans catheter closure (TCC) in some previous reports. The aim of this study is to report the outcomes and mid-term follow-up of transcatheter closure of large atrial septal defects using two-dimensional conventional echocardiography, tissue Doppler imaging, and four-dimensional speckle tracking imaging, and as such to compare results of same echocardiographic examination of age-matched control group of 40 healthy children group (2). Cardiac MRI was performed on cases group (1) only to detect right ventricle and left ventricle volumes and function and early signs of complications. There was no difference between cases and matched healthy controls in terms of the assessment of left ventricle and right ventricle by two-dimensional echocardiography, tissue Doppler imaging, and four-dimensional speckle tracking imaging. Similarly, there was no statistically significant difference between four-dimensional echocardiography and cardiac MRI in their respective assessment of both left ventricle and right ventricle volumes and function. We also detected no complications by echo or by cardiac MRI after a median follow-up period of 2 years and recorded a complete remodelling of right ventricle volumes in all children studied. This points to the safety and efficiency of transcatheter closure of large atrial septal defects in children on mid-term follow-up.

Evolution of ventricular function in children with permanent right ventricular pacing after tetralogy of Fallot repair: A midterm follow-up.

OBJECTIVE: We aim to evaluate the midterm effect of chronic apical right ventricular (RV) pacing on right and left ventricular (LV) function using different modalities of echocardiography including conventional echocardiography, tissue Doppler imaging and two-dimension speckle tracking echocardiography. METHODS: This case-control study enrolled 49 patients divided into two groups: a paced group and a nonpaced group. The paced group included 23 patients that underwent tetralogy of Fallot (TOF) repair and required permanent pacemaker insertion for postoperative complete heart block. The nonpaced group included 26 patients that had TOF repair at the same period. RESULTS: The median age for the paced and nonpaced groups was 6 and 8 years, respectively (P = .169). The follow-up duration after TOF surgical repair was 4 years for the paced patients and 5 years for nonpaced patients (P = .411). In the nonpaced group, the QRS duration increased and LV global longitudinal strain (GLS) decreased significantly with increasing duration after TOF repair, P value was .006 and .042, respectively. In the paced group, tricuspid annular systolic plane excursion (TAPSE) was significantly correlated with age (r = .578; P = .004) and duration following TOF correction (r = .724; P < .001). CONCLUSION: Chronic RV apical pacing in children after TOF repair was associated with better clinical status, preservation of RV systolic function, and prevention of progressive QRS prolongation. RV pacing was not associated with progressive deterioration of LV systolic function with increasing the time interval following TOF repair. Therefore, RV pacing can be beneficial in corrected TOF patients presenting with RV failure.

Propranolol versus captopril in the treatment of infantile hemangioma (IH): A randomized controlled trial.

BACKGROUND: Renin-angiotensin system components have been demonstrated in the biology of infantile hemangioma (IH). Captopril, an angiotensin-converting enzyme inhibitor, is proposed as a therapeutic alternative to oral propranolol. OBJECTIVES: We sought to compare the benefit of propranolol and captopril in the treatment of IH, and to assess angiotensin-converting enzyme gene polymorphism in patients with IH and in control subjects. METHODS: Thirty patients with IH and 35 healthy control subjects were enrolled in this study. Patients were randomly assigned to treatment with either propranolol or captopril. Assessment was done clinically and by measurement of serum vascular endothelial growth factor and angiotensin II in patients and control subjects. Angiotensin-converting enzyme gene polymorphism was also studied. RESULTS: Clinical improvement was significantly better and faster in the patients treated with propranolol. Both groups showed reduced vascular endothelial growth factor and angiotensin II levels posttreatment, with a significantly higher percentage reduction in the propranolol-treated group. Cardiac side effects were reported only in the captopril-treated group. Baseline vascular endothelial growth factor level was significantly higher, and baseline angiotensin II level was significantly lower, in patients than control subjects. LIMITATIONS: We studied a relatively small number of patients and control subjects. CONCLUSION: Propranolol shows greater benefit than captopril in the treatment of IH.

Permanent cardiac pacing in pediatric patients.

Pediatric pacemaker (PM) implants comprise less than 1 % of all PM implants. This study aimed to investigate permanent cardiac pacing among the pediatric population, identifying different indications and complications of pediatric cardiac pacing, especially focusing on the effect of the pacing sites, the PM lead type, and the indications for pacing. The current work is a cross-sectional study of 103 procedures for permanent PM insertion in pediatric patients between January 2001 and December 2010. The patients were followed up 1, 3, and 6 months after implantation, then every 6 months or as needed. Evaluation included routine clinical examination, electrocardiography, chest X-ray, echocardiography, and a full analysis of the pacing system measurements. The ages of the patients ranged from 0.09 to 12 years (median, 2.3 years). The most common indication for pacing was postoperative complete heart bock, noted in 54 patients (52.4 %). Transvenous endocardial PM insertion was performed in 92 procedures (89.3 %), whereas transthoracic epicardial insertion was performed in 11 procedures (10.7 %). The most common site of pacing was the right ventricular apex (n = 64, 62 %), followed by the right ventricular outflow tract (n = 25, 24.3 %). Transthoracic epicardial PM insertion was associated with a significantly higher percentage and greater severity of complications. In this study, 65 % of the patients with left ventricle (LV) dilation before pacing showed a significant improvement in LV dimensions and function after pacing. This was noted only in those with endocardially inserted PM leads in both the congenital and the postoperative groups regardless of the pacing site. Endocardial PM insertion in children is a safe procedure with fewer complications and a lower ventricular threshold than the epicardial route. Permanent single-chamber right ventricle pacing is safe and can lead to significant improvement in LV function and dimensions. However, long-term follow-up assessment is needed for further evaluation.

Oral propranolol: an effective, safe treatment for infantile hemangiomas.

Infantile hemangiomas (IH) are the most common childhood tumors. In 2008, Labreze reported the serendipitous effect of oral propranolol on hemangioma and since then it has overshadowed the use of other therapeutic modalities in the treatment of IH. The aim of this prospective, clinical study was to assess the efficacy and safety profile of oral propranolol at a fixed dose of 2 mgkg(-1) in the treatment of 30 patients with problematic IH. Propranolol treatment continued for a duration of 2-14 months where 60% of the patients (n=18) showed a final excellent response with complete resolution of the lesion (P<0.001). 20% (n=6) showed a good response with more than 50% reduction in the size of the IH. 16.6% showed a fair response (n=5) with less than 50% reduction in the size of the IH. Only one patient (3.3%) was resistant to treatment. Five patients (17.24%) showed evidence of rebound growth after cessation of therapy and responded well to re-treatment.We did not face any side effects related to the oral propranolol. In conclusion, propranolol therapy at a fixed dose of 2 mgkg(-1), given in three equally divided doses, is a very safe and effective regimen in the treatment of IH.

Renal Doppler indices in diabetic children with insulin resistance syndrome.

End-stage renal failure is still a leading cause of mortality among type 1 diabetes patients. Insulin resistance plays a larger role in type 1 diabetes disease process than is commonly recognized. Detection of diabetic nephropathy as early as possible currently offers the best chance of delaying or possibly preventing progression to end-stage disease. Renal resistive index (RI) and pulsatility index (PI), measured using renal Doppler ultrasonography, reflect intrarenal vascular resistance. The present work aimed at examining renal Doppler indices (RI and PI) in type 1 diabetic children and their relation to features of insulin resistance and other established parameters of early diabetic nephropathy as microalbuminuria. One hundred diabetic children with a mean age of 13.4 ± 2.9 yr and an average diabetes duration of (7.2 ± 2.5 yr) were included. Thirty healthy children served as controls. All renal Doppler indices were significantly higher in children with type 1 diabetes mellitus (p ≤ 0.01). The worst parameters were observed in children diagnosed with insulin resistance syndrome (IRS) (38%), hypertensive (12%), and obese (4%) children. Resistive index showed a significant correlation to blood pressure (r = 0.2, p = 0.04), waist-hip ratio (r = 0.5, p = 0.02), insulin dose (r = 0.2, p = 0.02) and estimated glucose disposal rate (r = -0.5, p = 0.01). No correlation was noted to microalbuminuria, HbA1c, or duration of diabetes. The present work concluded that renal Doppler indices are worse in diabetic children and particularly those with IRS. These children appear to be at graver risk for diabetic nephropathy. In these patients adding renal Doppler assessment to their work up, might diagnose diabetic nephropathy at a prealbuminuric stage.

Arrhythmia in the neonatal intensive care unit.

A random sample of 457 neonates was prospectively studied in order to identify the incidence, common types, and risk factors for arrhythmias in the neonatal intensive care unit (NICU). A 12-lead EKG was studied in all neonates (n = 457). A total of 139 Holter studies was done in every fourth baby with a normal EKG (n = 100) and in all babies with an abnormal EKG (n = 39). Of the 100 infants who were thought to be arrhythmia-free by EKG, nine infants demonstrated an arrhythmia on Holter studies. When we correlated screening results with maternal, obstetrical, and neonatal risk factors; arrhythmias were significantly associated with male gender, more mature gestational age, lower glucose levels, maternal smoking, high umbilical artery lines, and the use of the nebulized beta-2 adrenergic treatment, whereas umbilical venous lines and dopamine infusion did not relate to arrhythmia. We conclude that arrhythmias are more common in the NICU than in the general neonatal population. Compared to Holter monitoring, the sensitivity of the EKG was only 89%.

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.

The value of Holter monitoring in the assessment of Pediatric patients.

AIMS: Holter monitoring (HM) has been established as one of the most effective noninvasive clinical tools in the diagnosis, assessment and risk stratification of cardiac patients. However, studies in the pediatric age group are limited. The present work aims at determining the value of HM in the diagnosis and management of children. SETTINGS AND DESIGN: Retrospective study conducted at a tertiary referral arrhythmolology service. METHODS AND MATERIAL: Holter records of 1319 pediatric patients (54.1% males and 45.9% females) were reviewed. Their average age was 6.7+/- 4.1 years (5 days-16 years). Indications for which Holter monitoring was done were analysed as well as all the abnormalities diagnosed and factors that may increase Holter yield. STATISTICAL ANALYSIS USED: Statistical Package of social science (SPSS) version 9,0 was used for analysis of data. RESULTS: The most common indications were palpitations (19.8%), syncope (17.8%), cardiomyopathy (12.6%), chest pain (10%), evaluation of antiarrhythmic therapy (6.8%), postoperative assessment (2.6%) and complete AV Block (2.4%). A sum of 141 Holter recordings were found abnormal with a total diagnostic yield of 10.7%. The highest contribution to diagnosis was in postoperative assessment (32.4%) and in cardiomyopathy (19.9%) where the most common abnormalities were frequent supraventricular / ventricular premature beats, supraventricular tachycardia, ventricular tachycardia and AV block. Diagnostic yield was low in patients with palpitations (5.7%) and syncope (0.4%). An abnormal ECG was significantly associated with a higher diagnostic yield (p=0.0001). None of the children with chest pain had abnormal Holter recordings. CONCLUSIONS: HM has an extremely valuable role in the assessment of high risk patients (postoperative and cardiomyopathy). However in children with palpitations, syncope and chest pain HM has a low yield. In this group of patients an abnormal ECG is more likely to be associated with abnormal Holter recordings.

MEFV gene mutations and cardiac phenotype in children with familial Mediterranean fever: a cohort study.

BACKGROUND: Familial Mediterranean fever (FMF) is the most common autoinflammatory disorder in the world. It is characterized by recurrent febrile inflammatory attacks of serosal and synovial membranes. MEFV gene mutations are responsible for the disease and its protein product, pyrin or marenostrin, plays an essential role in the regulation of the inflammatory reactions. Although the disease may carry a potential for cardiovascular disorders because of sustained inflammation during its course, the spectrum of cardiac involvement in children with FMF has not been well studied. We aimed at defining the frequency and spectrum of cardiac affection in children with FMF. The correlation between these affections and MEFV gene mutations was searched for to establish the relationship between cardiac phenotype and the patient's genotype in FMF. METHODS: The present work is a cohort study including 55 patients with the clinical diagnosis of FMF based on the Tel-Hashomere criteria, confirmed by genetic analysis showing homozygous or compound heterozygous mutation of MEFV genes. Fifty age- and sex-matched normal children were included as controls. The entire study group underwent detailed cardiac examination, 12-lead ECG and echocardiography. All data was statistically analysed using SPSS version-15. RESULTS: Patients had an average age of 8.5+/-4.2 years; with an average disease duration of 2.1+/-2.2 years; 28 were males. All controls showed no MEVF gene mutations. The most frequent gene mutation of the studied cases was E148Q mutation seen in 34% of cases and the most frequent compound mutation was E148Q/V726A seen in 16.6% of cases. Echocardiographic examination revealed pericardial effusion in nine patients. Twelve had aortic regurgitation; nine had mitral regurgitation and six had pulmonary regurgitation. The most common mutation associated with pericardial effusion was E148Q/V726A in 5/9 of cases. Valvular involvement were significantly more common in FMF patients with gene mutations. Also cardiac involvement was more common in patients with positive consanguinity. However, these cardiac manifestations showed no correlation to age, family history of FMF, or response to therapy or laboratory data. CONCLUSIONS: In our cohort of children with FMF, cardiac involvement appears to be common. Pericardial effusions are significantly related to presence of mutation types E48Q, P 369S, V726A. These associations may warrant genetic screening of children with FMF to detect cardiac risk.

Transcatheter closure of secundum ASD using Occlutech Figulla-N device in symptomatic children younger than 2 years of age.

INTRODUCTION: Atrial septal defect (ASD) transcatheter occlusion techniques have become alternatives to surgical procedures. We evaluated the efficiency and safety of the Occlutech Figulla-N device in percutaneous closure of secundum ASDs in symptomatic children younger than 2 years of age. METHODS: The study included 17 patients (9 girls, 8 boys; mean age, 10.3 ± 2.1 months). Mean weight was 7.4 ± 1.3 kg, with secundum ASDs measuring more than 8 mm with a hemodynamically significant shunt, resulting in failure to thrive, right ventricular dilatation, and pulmonary hypertension. Two girls had fenestrated ASD secundum. Defect size and total interatrial septal length were estimated by transthoracic (TTE) and transesophageal (TEE) echocardiography in 3 views. Procedures were performed under fluoroscopic and TEE guidance. Patients were followed-up at 1, 3, 6, and 12 months with TTE. RESULTS: The mean defect size was 15.4 ± 2.7 mm on TTE and 17.1 ± 1.9 mm on TEE. The mean device size was 17.8 ± 3.6 mm (range, 10 to 24 mm). The mean pulmonary artery pressure was 54 ± 18 mm Hg. The device was placed successfully in all patients including fenestrated ASDs that were closed with a single device placement. No residual flow was seen after device placement in patients. Complications were seen in 2/17 patients (11.8%) in the form of sinus tachycardia in 1 patient and femoral vein hematoma in 1 patient. At 6 and 12 months, all the patients were asymptomatic. No cardiac perforation, device erosion, embolization, thrombus formation, or malposition of the device was observed. Three patients developed mild insignificant mitral regurgitation. CONCLUSIONS: ASD closure in severely symptomatic children younger than 2 years of age using the Occlutech Figulla-N occluder is safe and efficient. Meticulous care to patient selection, adequate defect sizing, and device size selection are keys to lower incidence of complications.

Propranolol and infantile hemangiomas: different routes of administration, a randomized clinical trial.

UNLABELLED: Oral propranolol has become the treatment of choice of infantile hemangiomas (IH)s. However, the safety of systemic propranolol is questioned. Topical therapy with 1% propranolol has been reported to be safe and effective. Intralesional (IL) administration may possibly allow safe delivery of higher drug dosages. AIM: To assess the efficacy and safety of two locally administered routes of propranolol (topical and IL), in comparison with its systemic oral use in the treatment of IHs. PATIENTS AND METHODS: 45 patients with IHs were randomly divided into 3 groups, A, B and C (n = 15 in each), receiving oral propranolol, 2 mg/kg/day, topical propranolol 1% ointment twice daily, IL propranolol, 1 mg of propranolol hydrochloride in 1 ml of injection once weekly, respectively. Follow up was done for 6 months after treatment was stopped. RESULTS: Excellent response was achieved in 9 patients in group A (60%), 3 in group B (20%) and 2 in group C (13.3%), (P value : 0.04). As regards safety, all 3 modalities proved safe with no major side effects apart from 1 patient in group A and 3 in group C who dropped out due to pain or inconvenience of therapy. CONCLUSIONS: Further work is needed to establish clear guidelines and reach best formulations. Nevertheless, in properly selected patients with IHs, we recommend the usage of oral propranolol. Topically administered propranolol could be considered in patients at risk of potential side effects from oral administration. As IL application did not offer any more benefits, it could not be recommended.

Socioeconomic risk factors of malnutrition among Egyptian children between 6 months and 2 years of age, Cairo, Egypt.

BACKGROUND: Malnutrition continues to be a significant public health and development concern around the world with about one-third of the world's children malnourished. OBJECTIVE: To identify and determine the risk factors for undernutrition in children between 6 months and 2 years of age attending outpatient clinics of the pediatric hospital, University of Cairo. PARTICIPANTS AND METHODS: A matched case-control study design was applied. Two hundred malnourished children attending the outpatient malnutrition clinic of the Center for Social and Preventive Medicine (CSPM), Department of Pediatrics, Faculty of Medicine, Cairo University, and 200 healthy controls were included in the study. The study was conducted from January 2010 to June 2010. RESULTS: The mean ages of the patients and controls were 11.31 (±5.6) and 11.5 (±5.3) months, respectively. The identified socioeconomic risk factors for malnutrition in the bivariate analysis were maternal illiteracy [odds ratio (OR=1.85), 95% confidence interval (CI) 1.2-2.85], paternal illiteracy (OR=2.09, 95% CI 1.08-4.04), and large family size, with the number of children greater than three (OR=3.76, 95% CI 2.48-5.71). Logistic regression analysis revealed that the risk for malnutrition was independently associated with lack of exclusive breastfeeding for the first 6 months of life (OR=5.77, 95% CI 3.28-10.17), maternal illiteracy (OR=5.28, 95% CI 1.92-14.5), reluctance to seek medical advice during illness (OR=2.36, 95% CI 1.23-4.54), late initiation of breastfeeding after birth (OR=2.33, 95% CI 1.25-4.33), and nonattendance of health or nutrition sessions (OR=2.02, 95% CI 1.17-3.51). CONCLUSION AND RECOMMENDATIONS: Lack of parental education, poor nutritional behavior, and child-feeding practices are important risk factors among malnourished children. These characteristics can be used for identifying high-risk groups and for designing and targeting preventive intervention programs.

Percutaneous closure of medium and large PDAs using amplatzer duct occluder (ADO) I and II in infants: safety and efficacy.

BACKGROUND: The use of the Amplatzer duct occluder (ADO; AGA Medical Corporation) is well established and reported in children and adults, but there are only occasional reports on use in symptomatic infants. METHODS AND RESULTS: Between March 2009 and January 2011, a group of 47 infants less than 2 years of age with symptomatic patent ductus arteriosus (PDA) was treated using ADO I and II devices. Patients were divided into infants less than 1 year old (group A; n = 28/47; 59.6%) and children between 1 and 2 years old (group B; n = 19/47; 40.4%). Physical examinations and echocardiograms were performed before the procedure and at follow-up (3, 6, and 12 months). Mean age was 5.3 ± 2.3 months for group A and 12.6 ± 1.7 months for group B. Mean weight at closure was 4.8 ± 1.9 kg for group A and 7.3 ± 2.1 kg for group B. ADO I was deployed in 19/28 of group A (67.9%) and 16/19 of group B (84.2%). ADO II was used in 9/28 of group A (32.1%) and 3/19 of group B (15.8%). No residual shunt at the end of the procedure was detected by angiography in any of the cases closed with ADO I and only 3/12 (25%) closed with ADO II showed a minimal residual flow. No mortality or major complications occurred. Six months after closure, weight gain, control of respiratory infections, and regression of left ventricular dilatation with improved systolic function were observed. CONCLUSIONS: Percutaneous closure of moderate to large PDAs using ADO I and II devices in infants and children younger than 2 years of age is safe and effective.

The effect of hemodialysis on Q-T interval on children with chronic renal failure.

The hemodialysis patients have a wide variety of electrocardiographic (ECG) abnormalities and, in certain instances; hemodialysis itself seems to be a cause of ECG changes and different kinds of dysrhythmias. The purpose of the study was to assess the effect of hemodialysis on QT and corrected QT (QTc) interval and QTc interval dispersion in patients with end-stage renal failure 10 min before (pre-HD) and 10 min after each hemodialysis (post-HD). An observational cross-section study was conducted on 30 patients'admitted to the dialysis unit in Abou El Ressh pediatric hospital along a period of 6 months starting from October 2008. The total number of cases on regular hemodialysis throughout the year was: 60 cases. 30 cases were picked at random to be included in the present work. Clinical examination, history taking and laboratory analysis were offered to all patients, 12 lead ECG was done before and after hemodialysis. The results showed that after hemodialysis there were significant changes for the QTc and QT dispersion with 3.3% of cases had prolonged QT interval after hemodialysis and 43.3% had prolonged QTc after dialysis and 50% had abnormal QTd after dialysis. After dialysis there was significant negative correlation between Na level and QTc, also between K level and QTc.