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Articles by Iain J.H. McCrindle in JoVE

 JoVE Applied Physics

Simulation, Fabrication and Characterization of THz Metamaterial Absorbers

1School of Engineering, University of Glasgow


JoVE 50114

This protocol outlines the simulation, fabrication and characterization of THz metamaterial absorbers. Such absorbers, when coupled with an appropriate sensor, have applications in THz imaging and spectroscopy.

Other articles by Iain J.H. McCrindle on PubMed

Cardiovascular Consequences of Paediatric Obesity: Will There Be a Future Epidemic of Premature Cardiovascular Disease?

Reconstituted Fresh Whole Blood Improves Clinical Outcomes Compared with Stored Component Blood Therapy for Neonates Undergoing Cardiopulmonary Bypass for Cardiac Surgery: a Randomized Controlled Trial

This study compared the effects of reconstituted fresh whole blood against standard blood component therapy in neonates undergoing cardiac surgery.

Making Sense of Congenital Cardiac Disease with a Research Database: The Congenital Heart Surgeons' Society Data Center

Challenges inherent in researching rare congenital cardiac lesions led to creation of the Congenital Heart Surgeons' Society Data Center (Data Center) two decades ago. The Data Center pools experiences from up to 60 institutions, and over 4,700 children have been prospectively recruited within nine diagnostic inception cohorts. This report describes the operations of our research database, with particular focus on analytic strategies employed.

The Effect of Calcination on Multi-walled Carbon Nanotubes Produced by Dc-arc Discharge

Multi-walled carbon nanotubes were synthesized by dc-arc discharge in helium atmosphere and the effect of calcination at different temperatures ranging from 300-600 degrees C was studied in detail. The degree of degradation to the structural integrity of the multi-walled carbon nanotubes during the thermal process was studied by Raman spectroscopy, Scanning electron microscopy and High resolution transmission electron microscopy. The thermal behaviour of the as prepared and calcined samples was investigated by thermogravimetric analysis. Calcination in air at 400 degrees C for 2 hours was found to be an efficient and simple method to eliminate carbonaceous impurities from the nanotube bundles with minimal damage to the tube walls and length. The impurities were oxidized at a faster rate when compared to the nanotubes and gave good yield of about 50%. The nanotubes were observed to be damaged at temperature higher than 450 degrees C. The results show that this method is less destructive when compared liquid phase oxidation with 5 M HNO3.

The Rush to Atrial Septal Defect Closure: is the Introduction of Percutaneous Closure Driving Utilization?

Surgical repair of isolated atrial septal defect (ASD) and patent foramen ovale (PFO) has been largely supplanted by percutaneous closure. Whether the perceived benefit of percutaneous closure has lowered thresholds for intervention and thus increased overall utilization rates is unknown. We sought to determine nationwide trends in the use of percutaneous compared with surgical closure and their respective outcomes over an 18-year period.

Frequency, Predictors, and Neurologic Outcomes of Vaso-occlusive Strokes Associated with Cardiac Surgery in Children

Our aim was to define the frequency, predictors, and outcomes of stroke associated with cardiac surgery in children with congenital heart disease.

Longitudinal Evaluation and Assessment of Cardiovascular Disease in Patients with Homozygous Familial Hypercholesterolemia

Homozygous familial hypercholesterolemia (hoFH) is caused by mutations in the low-density lipoprotein receptor gene and is characterized by severe hypercholesterolemia from birth and onset of premature cardiovascular disease (CVD) during childhood. The onset and progression of CVD using currently available testing methods in children with hoFH have not been fully characterized. A large cohort of patients with hoFH referred to our subspecialty clinic was studied. Thirty-nine patients (22 aged < or =16 years) underwent extensive cardiovascular, lipid, and genetic evaluation. Sixteen children < or =16 years without known CVD when first evaluated were followed up longitudinally for up to 8 years. CVD was clinically evident in 88% of subjects aged >16 years and 9% of those < or =16 years. Markers of atherosclerosis correlated significantly with age at which lipid-lowering treatment was initiated (abnormal coronary angiogram, abnormal aortic valve using echocardiography, and high calcium score using electron beam computed tomography; all p <0.01; abnormal carotid Doppler result; p = 0.03). Twenty of 22 children had no clinical evidence of coronary artery disease, yet 7 of these children had angiographically confirmed mild coronary artery disease (<50%) and 8 had mild to moderate aortic regurgitation using echocardiography. Of noninvasive tests, only evaluation of aortic valve regurgitation using echocardiography predicted the presence of angiographic coronary stenosis (p <0.001). During follow-up, 7 children developed progression of coronary and/or aortic valvular disease during their teenage years and 4 required surgical interventions. In conclusion, in these patients aggressive lipid-lowering treatment initiated in early childhood is warranted. Careful coronary and valvular surveillance strategies and coronary revascularization when appropriate are also warranted in this high-risk population.

Congenital Supravalvular Aortic Stenosis: Defining Surgical and Nonsurgical Outcomes

Supravalvular aortic stenosis is a rare stenotic lesion of the left ventricular outflow tract (LVOT). We characterized the natural history of the disease and the effect of surgical intervention.

Design and Rationale of a Randomized Trial Comparing the Blalock-Taussig and Right Ventricle-pulmonary Artery Shunts in the Norwood Procedure

The initial palliative procedure for patients born with hypoplastic left heart syndrome and related single right ventricle anomalies, the Norwood procedure, remains among the highest risk procedures in congenital heart surgery. The classic Norwood procedure provides pulmonary blood flow with a modified Blalock-Taussig shunt. Improved outcomes have been reported in a few small, nonrandomized studies of a modification of the Norwood procedure that uses a right ventricle-pulmonary artery shunt to provide pulmonary blood flow. Other nonrandomized studies have shown no differences between the two techniques.

Echocardiographic and Electrocardiographic Trends in Children with Acute Kawasaki Disease

Kawasaki disease (KD), while primarily an acute, self-limited, multisystem vasculitis, is more appropriately described as a pancarditis, from a cardiac perspective. Many patients are noted to have ventricular dilation on initial echocardiography; however, functional and structural measurements may remain within the normal range.

Intraoperative Coronary Artery Pulse Doppler Patterns in Patients with Complete Transposition of the Great Arteries Undergoing the Arterial Switch Operation

Arterial switch operation is standard repair for complete transposition of the great arteries (TGA). Coronary artery abnormalities are the most common cause of morbidity and mortality in the acute postoperative phase. This study was designed to determine whether coronary artery pulse Doppler flow patterns obtained by transesophageal echocardiography during the arterial switch operation are correlated with acute postoperative outcomes.

Evaluation of Problems and Possible Solutions Linked to the Surveillance and Control of Bovine Brucellosis in Sub-Saharan Africa, with Special Emphasis on Nigeria

Bovine brucellosis is disease of economic and public health significance in sub-Saharan Africa. The disease is distributed worldwide but some countries have been able to eradicate brucellosis from their territories using elaborate brucellosis control and eradication programmes that have been targeted primarily at livestock (the main reservoir host for the disease). This has been achieved mainly by vaccination, test and slaughter, as well as by regular surveillance for early detection of the disease. Despite the level of knowledge on the epidemiology of bovine brucellosis, there has been limited success in controlling bovine brucellosis in sub-Saharan Africa. Some of the problems associated with the surveillance and control of bovine brucellosis in sub-Saharan Africa include poor disease reporting, insufficient financial resources of governments (poor economic status of most countries in sub-Saharan Africa), as well as competing national health priorities, inadequate infrastructures and personnel, the commonly practised seasonal grazing or transhumant husbandry systems and communal grazing, inadequate monitoring of the disease in wildlife and poor communication and education of stakeholders. Since previous attempts at the control of bovine brucellosis have failed in Africa, it was considered important to address this aspect, using an approach that differed from the classic veterinary regulatory approach. Possible ways of dealing with this problem using complementary measures to the conventional approaches are also proposed.

The Three-dimensional Structural Characterization of Hexachlorocyclopentenyl-dibromocyclooctane (HCDBCO)

The 1H NMR spectrum and the crystal structure of HCDBCO [(1R,2R,5R,6R,9S,10S)-5,6-dibromo-1,10,11,12,13,13-hexachlorotricyclo[8,2,1,0(2,9)]-tridec-11-ene)] are reported. The measured dihedral angles from the X-ray structure correlate very well with those calculated from the proton-proton coupling constants indicating that the conformations in solution and in the solid state are probably very similar. Attempts at calculating the 3D model structure of HCDBCO only produced a very poor match between the measured dihedral angles between vicinal protons and the observed proton-proton coupling constants from the 1H NMR spectrum. GC/MS analysis with an injector temperature of 250 degrees C produced minor amounts of debrominated HCDBCO. Reducing the temperature to 200 degrees C eliminated this problem.

Ambulatory Blood Pressure Monitoring in Children and Adolescents: Recommendations for Standard Assessment: a Scientific Statement from the American Heart Association Atherosclerosis, Hypertension, and Obesity in Youth Committee of the Council on Cardiovascular Disease in the Young and the Council for High Blood Pressure Research

Contemporary Outcomes After the Fontan Procedure: a Pediatric Heart Network Multicenter Study

We characterized a large cohort of children who had a Fontan procedure, with measures of functional health status, ventricular size and function, exercise capacity, heart rhythm, and brain natriuretic peptide (BNP).

International Dairy Federation Guide to Good Animal Welfare in Dairy Production--2008

An Investigation of the Quality of Meat Sold in Lesotho

Since the closure of the Lesotho abattoir in 2003, only imported meat can be legally sold. However, it was estimated in 2007 that 80% of the meat sold at butcheries comes from informal slaughter. The aim of this study was to investigate the situation. The number and location of informal butcheries in Lesotho (n = 143) were recorded and mapped using Geographical Information Systems. Observations (photographs) of informal slaughter indicated a lack of hygiene, unskilled slaughtermen and illegal disposal of offal with possible environmental pollution. In addition, a cross-sectional study was undertaken to determine the microbiological quality of meat from randomly selected carcasses (n = 237) of cattle, sheep and pigs from a sample of 44 butcheries, 4 of which were associated with registered supermarkets. As a control, samples for microbiological assay were taken from imported meat originating from carcasses (n = 20) slaughtered at a registered abattoir in South Africa. Of the 44 butcheries investigated only the 4 commercial butcheries associated with supermarkets sold imported meat only; 3 butcheries sold meat inspected at government slaughter slabs (n = 3), while the rest (n = 37) sold both imported and informally slaughtered meat. In terms of Lesotho legislation, informally slaughtered meat is only for home consumption. The bacteriological counts from all samples showed a total bacterial plate count exceeding 30 organisms per ml in contrast with the controls which all showed a count less than 5 colonies per ml. This was found for both imported and informally slaughtered meat sold in Lesotho. In addition, meat samples from butcheries showed the presence of the potential pathogens Salmonella (n = 2), Staphylococcus aureus (n = 12) and Escherichia coli (n = 15). During the study, anthrax was confirmed in 9 human patients, 5 of whom died, after consumption of informally slaughtered livestock. Although no cases of animal abuse were detected, it was considered that informal slaughter can prejudice livestock welfare. It was concluded that the current situation is not acceptable in terms of meat safety. Thus, the Veterinary Public Health Directorate of the National Veterinary Services has prioritised the monitoring of butchers, registration of slaughter slabs and a general extension campaign to improve hygiene and animal welfare standards for slaughter in Lesotho. This paper highlights the major risks associated with meat consumption without Veterinary Public Health intervention in accordance with international standards.

Physical Activity Participation in Youth with Surgically Corrected Congenital Heart Disease: Devising Guidelines So Johnny Can Participate

While most youth with congenital heart disease have simple structural lesions and near-normal activity tolerance and functioning, youth with complex structural defects may be affected by significant morbidity. Efforts are being made to consider the impact of exercise and physical activity on quality of life. The benefits of physical activity are well documented; however, the levels of participation and self-efficacy for activity remain low, thus increasing the risk of obesity and cardiovascular morbidity. Rehabilitation is a novel and emerging area, and decisions regarding advocacy versus restriction of physical activity may pose a challenge for both cardiologists and nonspecialists. The present article outlines the rationale for physical activity recommendations in youth with surgically corrected congenital heart disease and the psychosocial determinants of participation. Clinical recommendations and future directions are proposed.

Diastereomers of the Brominated Flame Retardant 1,2-dibromo-4-(1,2 Dibromoethyl)cyclohexane Induce Androgen Receptor Activation in the Hepg2 Hepatocellular Carcinoma Cell Line and the Lncap Prostate Cancer Cell Line

Reported incidences of prostate cancer and masculinization of animals indicate a release of compounds with androgenic properties into the environment. Large numbers of environmental pollutants have been screened to identify such compounds; however, not until recently was 1,2-dibromo-4-(1,2-dibromoethyl)cyclohexane (TBECH) identified as the first potent activator of the human androgen receptor (hAR). TBECH has been found in beluga whales and bird eggs and has also been found to be maternally transferred in zebrafish.

Comparison of Factors Associated with Coronary Artery Dilation Only Versus Coronary Artery Aneurysms in Patients with Kawasaki Disease

We sought to determine whether differences exist in the factors associated with transient coronary artery (CA) dilation only compared to CA aneurysms after Kawasaki disease (KD). We reviewed all patients with KD assessed and treated at a single tertiary institution from January 1990 to April 2007. Of 1,374 patients (63% men) with KD, 1,108 (81%) had no CA abnormalities; 180 (13%) had CA dilation only, and 86 (6%) had CA aneurysms. The factors associated with any CA abnormality included male gender (odds ratio [OR] 1.8, p <0.001), lower albumin (OR per 1 g/dl, 1.07; p < 0.001), lower hemoglobin (OR per 10 g/dl; 1.02, p = 0.008), diagnosed outside of the 1 to 9-year-age range (OR for <1 year old, 1.5; p = 0.04; OR for >9 years old, 1.9, p = 0.03), longer duration of fever before treatment (OR per day, 1.05, p = 0.002), greater platelet count (OR per 10 x 10(9)/L, 1.14, p = 0.008), and nonresponse to initial intravenous immunoglobulin treatment (OR 2.4, p <0.001). Only age at diagnosis, duration of fever before treatment, greater platelet count, and nonresponse to initial intravenous immunoglobulin discriminated between CA dilation only versus CA aneurysms (c-statistic, 0.80, p <0.001). In conclusion, CA dilation only and CA aneurysms differ only in the total duration of inflammation, as measured by the number of days from fever onset to defervescence.

Perfluorooctane Sulfonate (PFOS) Precursors Can Be Metabolized Enantioselectively: Principle for a New PFOS Source Tracking Tool

Perfluorooctane sulfonate (PFOS) is the most prominent perfluoroalkyl substance found in the serum of humans and wildlife, yet the major routes by which exposure occurs are not clear. An important issue facing both the scientific and chemical regulatory communities is the extent to which PFOS concentrations in biota are attributable to direct exposure versus metabolism of PFOS-precursors (higher molecular weight derivatives that can be biotransformed to PFOS). Given that certain branched PFOS-precursors are chiral, we hypothesized that nonracemic proportions of PFOS isomers in biological samples could be used as a marker of significant exposure to PFOS-precursors. In this proof-of-principle study we examined the enantiomer-specific biotransformation of a high-purity model PFOS-precursor isomer: C(6)F(13)C*F(CF(3))SO(2)N(H)CH(2)(C(6)H(4))OCH(3) (named 1m-PreFOS hereafter, and whereby * indicates the chiral carbon center). A method for the enantiospecific separation of a compound with a long perfluoroalkyl chain and a chiral center was developed and applied to evaluate the enantioselectivity of 1m-PreFOS biotransformation in human liver microsomes. Gradient elution in reversed-phase mode on a Chiralpak IC column permitted the near-baseline separation of the two enantiomers (E1 and E2, nomenclature based on retention order) in 65 min. Microsome incubations demonstrated that E1 and E2 were metabolized at significantly different rates; k(E1) = 6.5(+/-0.3) x 10(-2) min(-1) (half-life = 10.6 min) and k(E2) = 5.2(+/-0.3) x 10(-2) min(-1) (half-life = 13.3 min), respectively. These results suggest that tracking of PFOS exposure sources by enantiomeric fractionation is feasible, and that new analytical methods for the enantioselective analysis of PFOS isomers in human and environmental samples should be developed.

Branched Perfluorooctane Sulfonate Isomer Quantification and Characterization in Blood Serum Samples by HPLC/ESI-MS(/MS)

Perfluorooctane sulfonate (PFOS) is a global contaminant and is currently among the most prominent contaminants in human blood and wildlife samples. Although "total PFOS" (SigmaPFOS) analytical methods continue to be the most commonly used for quantification, recent analytical method developments have made it possible to resolve the various isomers of PFOS by HPLC-MS/MS. Characterized technical PFOS standards (i.e., containing a mixture of PFOS isomers) are now available that enable isomer specific quantification of PFOS, however the advantages of such an analysis have notyet been examined systematically. Herein, PFOS isomers have been individually quantified for the first time in real samples and the results are compared to a traditional SigmaPFOS method; the influence of analytical standards and isomer specific electrospray and MS/ MS behavior were also investigated. The two human serum standard reference materials chosen for analysis contained dramaticallydifferent PFOS isomer profiles (approximately 30-50% total branched isomers) emphasizing that isomer patterns should not be ignored and may provide useful information on exposure sources (i.e., direct exposure to PFOS vs indirect exposure from PFOS-precursors). Depending on the sample and the particular MS/MS transition chosen for SigmaPFOS analysis (i.e., 499-->80 or 499-->99), SigmaPFOS concentrations may be over- or underestimated compared to the isomer specific analysis. Differences in the extent of in-source fragmentation and MS/MS dissociation contributed to the systematic analytical bias. It was also shown that SigmaPFOS data are prone to interlaboratory variation due to various choices of PFOS standards and instrumental conditions used. In the future, for either SigmaPFOS or isomer specific PFOS analyses, we suggest that accuracy can be maximized and interlaboratory discrepancies minimized by using a common chemically pure technical PFOS standard characterized by 19F NMR.

Association Between Parental Anxiety and Compliance with Preoperative Requirements for Pediatric Outpatient Surgery

The purpose of this study was to determine if parental anxiety interferes with the ability to follow preoperative requirements.

Parent- Versus Child-reported Functional Health Status After the Fontan Procedure

We sought to compare perceptions of functional health status between children who had undergone a Fontan procedure and their parents.

Comparison of Impact of Prenatal Versus Postnatal Diagnosis of Congenitally Corrected Transposition of the Great Arteries

Congenitally corrected transposition of the great arteries (CCTGA) in the absence of major cardiac anomalies is thought to have a good outcome, although this has not been well documented. The objective of the present study was to compare the characteristics and outcomes of patients with a prenatal diagnosis of CCTGA to the characteristics and outcomes of those diagnosed postnatally. The optimal outcome was defined as intervention-free survival. All patients with CCTGA diagnosed prenatally and postnatally from 1999 to 2006 at 2 tertiary care institutions were reviewed. Patients with a single ventricle, heterotaxy, or valvar atresia were excluded. The differences between groups were assessed using the t test and chi-square test. A total of 54 patients (16 prenatal with 14 live born and 39 postnatal) were included. The patients diagnosed prenatally were diagnosed at a median gestational age of 20 weeks (range 16 to 37). Two deaths in each group were due to heart failure. The intervention-free survival rate for the prenatal and postnatal groups at 1, 6, and 36 months was 79%, 45%, and 30% and 85%, 61%, and 23%, respectively (p = NS). Of 37 patients, 14 (38%) underwent an arterial switch plus atrial baffling so that the morphologic left ventricle supported the systemic circulation, and 6 (16%) underwent repair of associated lesions so the morphologic right ventricle supported the systemic circulation; 4 (11%) of the 37 patients had pacemaker only. Prenatal patients with >1 fetal echocardiogram (12 of 14) did not have progression before birth. In conclusion, CCTGA has a >70% risk of intervention in the first 3 years after birth. The outlook is guarded and has an important effect on prenatal counseling.

Transition to Adult Health Care for Adolescents and Young Adults with Congenital Heart Disease: Perspectives of the Patient, Parent and Health Care Provider

Pediatric institutions play a large role in preparing young adults with congenital heart disease to transition to adult care.

Functional Health Status of Adolescents After the Fontan Procedure -- Comparison with Their Siblings

Studies have suggested that patients who have undergone the Fontan procedure experience lower functional health status and diminished exercise capacity compared with other children.

Noninvasive Assessment of Subclinical Atherosclerosis in Children and Adolescents: Recommendations for Standard Assessment for Clinical Research: a Scientific Statement from the American Heart Association

Deterioration in endothelial function and arterial stiffness are early events in the development of cardiovascular diseases. In adults, noninvasive measures of atherosclerosis have become established as valid and reliable tools for refining cardiovascular risk to target individuals who need early intervention. With limited pediatric data, the use of these techniques in children and adolescents largely has been reserved for research purposes. Therefore, this scientific statement was written to (1) review the current literature on the noninvasive assessment of atherosclerosis in children and adolescents, (2) make recommendations for the standardization of these tools for research, and (3) stimulate further research with a goal of developing valid and reliable techniques with normative data for noninvasive clinical evaluation of atherosclerosis in pediatric patients. Precise and reliable noninvasive tests for atherosclerosis in youth will improve our ability to estimate future risk for heart attack and stroke. Currently, large longitudinal studies of cardiovascular risk factors in youth, such as the Bogalusa and Muscatine studies, lack sufficient adult subjects experiencing hard outcomes, such as heart attack and stroke, to produce meaningful risk scores like those developed from Framingham data.

Kawasaki Disease at the Extremes of the Age Spectrum

We sought to determine outcomes of Kawasaki disease (KD) and to explore factors associated with poor clinical outcomes for patients diagnosed outside the age range of 1 to 4 years.

Zoonotic Tuberculosis and Brucellosis in Africa: Neglected Zoonoses or Minor Public-health Issues? The Outcomes of a Multi-disciplinary Workshop

Late in 2007, veterinary, medical and anthropological professionals from Europe and Africa met in a 2-day workshop in Pretoria, South Africa, to evaluate the burden, surveillance and control of zoonotic tuberculosis and brucellosis in sub-Saharan Africa. Keynote presentations reviewed the burden of these diseases on human and livestock health, the existing diagnostic tools, and the available control methods. These presentations were followed by group discussions and the formulation of recommendations. The presence of Mycobacterium bovis and Brucella spp. in livestock was considered to be a serious threat to public health, since livestock and animal products are the only source of such infections in human beings. The impact of these pathogens on human health appears to be relatively marginal, however, when compared with Mycobacterium tuberculosis infections and drug resistance, HIV and malaria. Appropriate diagnostic tools are needed to improve the detection of M. bovis and Brucella spp. in humans. In livestock, the 'test-and-slaughter' approach and the pasteurization of milk, which have been used successfully in industrialized countries, might not be the optimal control tools in Africa. Control strategies should fit the needs and perceptions of local communities. Improved intersectoral and international collaboration in surveillance, diagnosis and control, and in the education of medical and veterinary personnel, are advocated.

Kawasaki Disease: a Childhood Disease with Important Consequences into Adulthood

Exercise Capacity Improves with Time in Pediatric Heart Transplant Recipients

The purpose of this study was to profile the exercise capacity of pediatric heart transplant recipients over time and to identify factors associated with lower exercise capacity.

Left Ventricular Morphology Influences Mortality After the Norwood Operation

Within the spectrum of congenital heart disease referred to as hypoplastic left heart syndrome (HLHS), there is variation in the morphology and function of the left ventricle which could influence outcomes after stage I Norwood palliation.

Recipient Genotype is a Predictor of Allograft Cytokine Expression and Outcomes After Pediatric Cardiac Transplantation

This study sought to investigate the influence of recipient renin-angiotensin-aldosterone system (RAAS) genotype on cardiac function, rejection, and outcomes after heart transplantation.

Physical Activity Restrictions for Children After the Fontan Operation: Disagreement Between Parent, Cardiologist, and Medical Record Reports

Physical activity is important for the health of children after the Fontan procedure. Parents uncertain about physical activity have children who are more sedentary. To understand parent uncertainty, we examined agreement regarding activity restrictions from parents, cardiologists, and medical charts.

Aortic and Mitral Valve Replacement in Children: is There Any Role for Biologic and Bioprosthetic Substitutes?

The ideal valve substitute in children does not exist. Biologic and bioprosthetic valves do not require anticoagulation, however their use is complicated by accelerated degeneration and requirement for reoperation. We examine results following mitral (MVR) or aortic (AVR) replacement with biologic and bioprosthetic valves at our institution.

Extra-corporeal Life Support Following Cardiac Surgery in Children: Analysis of Risk Factors and Survival in a Single Institution

Application of extra-corporeal life support (ECLS) following pediatric cardiac surgery varies between different institutions based on manpower availability and philosophy towards ECLS utilization. We examined a large single institution experience with postoperative ECLS in children aiming to identify outcome predictors.

American Heart Association Childhood Obesity Research Summit: Executive Summary

American Heart Association Childhood Obesity Research Summit Report

Mechanical Valves Versus the Ross Procedure for Aortic Valve Replacement in Children: Propensity-adjusted Comparison of Long-term Outcomes

We aimed to identify characteristics differentiating children undergoing aortic valve replacement by using mechanical prostheses versus the Ross procedure and to compare survival and the need for aortic valve reoperation after each procedure.

Arterial Complications Associated with Cardiac Catheterization in Pediatric Patients with a Previous History of Kawasaki Disease

To determine whether patients with Kawasaki Disease (KD) undergoing percutaneous catheter angiography were at increased risk of arterial complications at the point of arterial access compared to patients with structural heart abnormalities, but normal vessels.

Spectrum and Management of Hypertriglyceridemia Among Children in Clinical Practice

The prevalence and identification of hypertriglyceridemia in youths will likely will increase in the future as a consequence of childhood obesity and increased screening for dyslipidemias. We sought to review our clinical experience with hypertriglyceridemia, evaluate factors associated with increased triglyceride levels, and review treatment options to provide guidance for management.

Progress and Challenges in Metabolic Syndrome in Children and Adolescents: a Scientific Statement from the American Heart Association Atherosclerosis, Hypertension, and Obesity in the Young Committee of the Council on Cardiovascular Disease in the Young; Council on Cardiovascular Nursing; and Council on Nutrition, Physical Activity, and Metabolism

Structural Characterization and Thermal Stabilities of the Isomers of the Brominated Flame Retardant 1,2,5,6-tetrabromocyclooctane (TBCO)

1,2,5,6-Tetrabromocyclooctane (TBCO) is a commercial brominated flame retardant that is employed mainly as an additive in textiles, paints and plastics. Very little is known about its presence or behavior in the environment or its analysis. TBCO can exist as two diastereomers, the stereochemistries of which have not been previously reported. We have named the first eluting isomer, under HPLC conditions, as alpha-TBCO (alpha-TBCO) and the later eluting isomer as beta-TBCO (beta-TBCO) when using an Acquity UPLC BEH C(18) column with methanol/acetonitrile/water as the mobile phase. The structural elucidation of these two isomers was accomplished by 1H NMR spectroscopy, GC/MS, LC/MS and X-ray structure determinations. alpha-TBCO is (1R,2R,5S,6S)-1,2,5,6-tetrabromocyclooctane and beta-TBCO is rac-(1R,2R,5R,6R)-1,2,5,6-tetrabromocyclooctane. As with some other brominated cycloaliphatic compounds, TBCO is thermally labile and the isomers easily interconvert. A thermal equilibrium mixture of alpha- and beta-TBCO consists of approximately 15% and 85% of these isomers, respectively. Separation of the two diastereomers, with minimal thermal interconversion between them, is achievable by careful selection of GC-capillary column length and injector temperature. LC/MS analyses of TBCO also presents an analytical challenge due to poor resolution of the isomers on chromatographic stationary phases, and weak intensity of molecular ions (or major fragment ions) when using LC-ESI/MS. Only bromide ions were seen in the mass spectra. APCI and APPI also failed to produce the molecular ion with sufficient intensity for identification.

Rationale and Design of a Trial of Angiotensin-converting Enzyme Inhibition in Infants with Single Ventricle

Angiotensin converting enzyme (ACE) inhibitors are known to improve clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology show abnormalities in ventricular function as well as poor growth. The ability of an ACE inhibitor to preserve ventricular function and improve growth in these infants is unknown.

Continuous Infusion of Thymoglobulin for Induction Therapy in Pediatric Heart Transplant Recipients; Experience and Outcomes with a Novel Strategy for Administration

Minimal data exist on the perioperative use of TG for induction in pediatric HTx recipients. We report our experience using continuous infusion of TG on (i) perioperative adverse events, (ii) rejection, (iii) CAV, and (iv) PTLD. TG was infused via peripheral intravenous intra- and perioperatively as a continuous infusion (24 h/day). Starting dose was 1.5 mg/kg/day titrated to achieve target lymphocyte count of 0.1-0.3 x 10(9)/L. Fifty-five patients received TG; mean age at HTx was 4.4 yr (1 day-17.8 yr). The mean duration of TG was three and a half days (2-7 days). Median platelet count during TG infusion was 95 x 10(9)/L (28-228). Five patients had TG stopped for low platelets (at 4-6 days post-HTx) - all started maintenance immunosuppression. There was no perioperative mortality due to infection. Mean follow-up of 46 survivors was 2.3 yr (0.6-5.8 yr). Fifty-one percent had > or = ISHLT 2R rejection at a median time of 33 days post-HTx (7 days-2 yr). One patient developed PTLD 1.4 yr post-HTx; three patients developed mild-moderate CAV. TG as a continuous infusion appears to have a good safety profile. Though mild thrombocytopenia was prevalent, there was no bleeding attributable solely to TG. Whether early depletion of T-cell function will translate into long-term benefits remains to be determined.

Case-control Study to Determine Whether River Water Can Spread Tetracycline Resistance to Unexposed Impala (Aepyceros Melampus) in Kruger National Park (South Africa)

A case-control study was performed in the Kruger National Park (KNP), South Africa, to find out whether impala (Aepyceros melampus) were more likely to harbor tetracycline-resistant Escherichia coli (TREC) in their feces when they drank from rivers that contained these bacteria than when they drank from rivers that were uncontaminated with TREC. The following five perennial rivers were selected: the Crocodile, the Letaba, the Olifants, the Sabie, and the Sand. Samples of river water (n = 33) and feces (n = 209), collected at 11 different sites, were cultured for E. coli. The resulting colonies were screened for tetracycline resistance by use of the Lederberg replica plating method (breakpoint, 4 mg/liter). A resistant and/or a susceptible isolate was then selected from each sample and subjected to the CLSI MIC broth microdilution test for tetracyclines. Among the 21 water specimens contaminated by E. coli, 19.05% (n = 4) were found to be resistant by the MIC method (breakpoint, >/=8 mg/liter). This led to the Crocodile, Olifants, and Letaba rivers being classified as TREC positive. Among the 209 impala feces sampled, 191 were positive for the presence of E. coli (91.38%). Within these (n = 191), 9.95% (n = 19) of the isolates were shown to be TREC by the MIC method. It was found that 1.11% (n = 1) of the E. coli isolates cultured from the feces of the control group (n = 90) were TREC, in comparison with 17.82% (n = 18) of those in feces from the exposed group (n = 101). The calculation of the odds ratio showed that impala drinking from TREC-contaminated rivers were 19.3 (2.63 to 141.69) times more likely to be infected with TREC than were unexposed impala. This is a significant finding, indicating that surface water could be a possible source of antimicrobial resistance in naïve animal populations and that impala could act as sentinels for antimicrobial resistance.

Disposition of Perfluorinated Acid Isomers in Sprague-Dawley Rats; Part 2: Subchronic Dose

Two major industrial synthetic pathways have been used to produce perfluorinated acids (PFAs) or their precursors: Telomerization and electrochemical fluorination (ECF). Products of telomer and ECF origin can be distinguished by structural isomer profiles. A mixture of linear and branched perfluoroalkyl isomers is associated with ECF. Telomer products characteristically consist of a single perfluoroalkyl geometry, typically linear. In biota, it is unclear if the isomer profile is conserved relative to the exposure medium and hence whether PFA isomer profiles in organisms are useful for distinguishing environmental PFA sources. A companion study suggested isomer-specific disposition following a single oral gavage exposure to rats. To confirm these findings under a more realistic subchronic feeding scenario, male and female rats were administered PFA isomers by diet for 12 weeks, followed by a 12-week depuration period. The diet contained 500 ng/g each of ECF perfluorooctanoate (PFOA, approximately 80% n-PFOA), ECF perfluorooctane sulfonate (PFOS, approximately 70% n-PFOS), and linear and isopropyl perfluorononanoate (n- and iso-PFNA). Blood sampling during the exposure phase revealed preferential accumulation of n-PFOA and n-PFNA compared to most branched isomers. Female rats depurated all isomers faster than males. Both sexes eliminated most branched perfluorocarboxylate isomers more rapidly than the n-isomer. Elimination rates of the major branched PFOS isomers were not statistically different from n-PFOS. Two minor isomers of ECF PFOA and one branched PFOS isomer had longer elimination half-lives than the n-isomers. Although extrapolation of these pharmacokinetics trends in rats to humans and wildlife requires careful consideration of dosage level and species-specific physiology, cumulative evidence suggests that perfluorocarboxylate isomer profiles in biota may not be suitable for quantifying the relative contributions of telomer and ECF sources.

Disposition of Perfluorinated Acid Isomers in Sprague-Dawley Rats; Part 1: Single Dose

Perfluorinated acids (PFAs) and their precursors (PFA-precursors) exist in the environment as linear and multiple branched isomers. These isomers are hypothesized to have different biological properties, but no isomer-specific data are currently available. The present study is the first in a two-part project examining PFA isomer-specific uptake, tissue distribution, and elimination in a rodent model. Seven male Sprague-Dawley rats were administered a single gavage dose of approximately 500 microg/kg body weight perfluorooctane sulfonate (C(8)F(17)SO(3)(-), PFOS), perfluorooctanoic acid (C(7)F(15)CO(2)H, PFOA), and perfluorononanoic acid (C(8)F(17)CO(2)H, PFNA) and 30 microg/kg body weight perfluorohexane sulfonate (C(6)F(13)SO(3)(-), PFHxS). Over the subsequent 38 d, urine, feces, and tail-vein blood samples were collected intermittently, while larger blood volumes and tissues were collected on days 3 and 38 for isomer analysis by high-performance liquid chromatography-tandem mass spectrometry (HPLC-MS/MS). For all PFAs, branched isomers generally had lower blood depuration half-lives than the corresponding linear isomer. The most remarkable exception was for the PFOS isomer containing an alpha-perfluoromethyl branch (1m-PFOS), which was threefold more persistent than linear PFOS, possibly due to steric shielding of the hydrophilic sulfonate moiety. For perfluoromonomethyl-branched isomers of PFOS, a structure-property relationship was observed whereby branching toward the sulfonate end of the perfluoroalkyl chain resulted in increased half-lives. For PFHxS, PFOA, and PFOS, preferential elimination of branched isomers occurred primarily via urine, whereas for PFNA preferential elimination of the isopropyl isomer occurred via both urine and feces. Changes in the blood isomer profiles over time and their inverse correlation to isomer elimination patterns in urine, feces, or both provided unequivocal evidence of significant isomer-specific biological handling. Source assignment based on PFA isomer profiles in biota must therefore be conducted with caution, because isomer profiles are unlikely to be conserved in biological samples.

Late Risk of Outcomes for Adults with Repaired Tetralogy of Fallot from an Inception Cohort Spanning Four Decades

Adult survivors with tetralogy of Fallot constitute a growing population with congenital heart disease. We investigated an inception cohort who underwent surgical repair. We aimed to characterize late hazard or risk for death, and determine the time-related risk of late re-operation and pulmonary valve replacement (PVR).

Infective Endocarditis in Children: Native Valve Preservation is Frequently Possible Despite Advanced Clinical Disease

Recent reports describing surgical experiences with childhood IE are sparse. We sought to determine patient-specific characteristics and their impact on outcome for children with infective endocarditis (IE) undergoing surgical intervention. We therefore reviewed all cases of culture-proven IE referred for surgical intervention at our institution over the last three decades.

Coronary Artery Dilation After Kawasaki Disease for Children Within the Normal Range

Kawasaki disease is an acute, self-limited vasculitis of unknown etiology. Coronary artery involvement is the predominant complication, ranging from no involvement to dilation through to aneurysm formation. We sought to determine trends over time and associated factors for normalized coronary artery dimensions for patients with acute KD.

Seroprevalence of Mycobacterium Avium Subspecies Paratuberculosis in Cows in Umbria, Italy

Veterinary Extension on Sampling Techniques Related to Heartwater Research

Heartwater, a tick-borne disease caused by Ehrlichia ruminantium, is considered to be a significant cause of mortality amongst domestic and wild ruminants in South Africa. The main vector is Amblyomma hebraeum and although previous epidemiological studies have outlined endemic areas based on mortalities, these have been limited by diagnostic methods which relied mainly on positive brain smears. The indirect fluorescent antibody test (IFA) has a low specificity for heartwater organisms as it cross-reacts with some other species. Since the advent of biotechnology and genomics, molecular epidemiology has evolved using the methodology of traditional epidemiology coupled with the new molecular techniques. A new quantitative real-time polymerase chain reaction (qPCR) test has been developed for rapid and accurate diagnosis of heartwater in the live animal. This method can also be used to survey populations of A. hebraeum ticks for heartwater. Sampling whole blood and ticks for this qPCR differs from routine serum sampling, which is used for many serological tests. Veterinary field staff, particularly animal health technicians, are involved in surveillance and monitoring of controlled and other diseases of animals in South Africa. However, it was found that the sampling of whole blood was not done correctly, probably because it is a new sampling technique specific for new technology, where the heartwater organism is much more labile than the serum antibodies required for other tests. This qPCR technique is highly sensitive and can diagnose heartwater in the living animal within 2 hours, in time to treat it. Poor sampling techniques that decrease the sensitivity of the test will, however, result in a false negative diagnosis. This paper describes the development of a skills training programme for para-veterinary field staff, to facilitate research into the molecular epidemiology of heartwater in ruminants and eliminate any sampling bias due to collection errors. Humane handling techniques were also included in the training, in line with the current focus on improved livestock welfare.

Anthropometric Measures After Fontan Procedure: Implications for Suboptimal Functional Outcome

Abnormal height and adiposity are observed after the Fontan operation. These abnormalities may be associated with worse functional outcome.

Characteristics and Outcomes of Double Outlet Left Ventricle

Double outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. There are limited data regarding the anatomical spectrum, characteristics and outcomes of DOLV.

Randomized, Controlled Trial of Individualized Heparin and Protamine Management in Infants Undergoing Cardiac Surgery with Cardiopulmonary Bypass

We sought to determine whether infants (younger than 1 year old) had similar clinical benefits with individualized anticoagulation management as older children and adult undergoing cardiopulmonary bypass (CPB).

Variability in Tacrolimus Blood Levels Increases the Risk of Late Rejection and Graft Loss After Solid Organ Transplantation in Older Children

  Late graft rejection impairs the long-term function of organ transplants in children. Previous studies suggest patients with wide variation in tacrolimus levels may have higher rates of late kidney and liver graft rejection. The reproducibility of this finding and impact on graft and recipient survival have not been reported. We investigated factors associated with late rejection > 6 months post-transplant in 144 heart, kidney, liver, and lung transplant recipients (ages 8-18, ≥ 1-yr survivors, receiving tacrolimus-based immunosuppression), comparing late rejectors (n = 61, 42%) to non-rejectors (no rejection > 6 months); groups had similar mean tacrolimus concentrations ≤ 6 months post-transplant. For all organ types, increased standard deviation in intrapatient tacrolimus blood levels was an independent risk factor for late rejection (OR 1.6 [CI 1.1-2.1]; p = 0.02). Each 1-point increase in s.d. > 2 of tacrolimus level > 6 months post-transplant associated with 1.58 increase in hazard of graft loss (p = 0.003). Graft survival (conditional on one-yr survival) was significantly better for those with s.d. < 2 at > 6 months post-transplant: 98% at three and five yr, versus 88%, 70%, at three and five yr, in patients with s.d. > 2 (p = 0.003). In conclusion, high s.d. in serial tacrolimus concentrations associated with increased risk of late rejection and graft loss in pediatric organ transplant recipients, providing opportunities for screening and interventions.

Population Trends Toward Increasing Cardiovascular Risk Factors in Canadian Adolescents

To determine prevalence and cross-sectional trends over time for cardiovascular risk factors in Canadian adolescents.

Persistent Risk of Subsequent Procedures and Mortality in Patients After Interrupted Aortic Arch Repair: a Congenital Heart Surgeons' Society Study

Multiple subsequent procedures directed at the arch and/or the left ventricular outflow tract are frequently required after interrupted aortic arch repair. We the investigated patterns and factors associated with these subsequent procedures and mortality.

The Fontan Patient: Inconsistencies in Medication Therapy Across Seven Pediatric Heart Network Centers

Patients who have undergone the Fontan procedure are at risk for thrombosis, ventricular dysfunction, and valve regurgitation, but data to guide the medical treatment and prevention of these adverse outcomes in this population are lacking. This analysis examined medication usage among Fontan patients by putative indication and by study center. The medical history and current medications of 546 Fontan subjects, ages 6-18 years, were assessed in a Pediatric Heart Network multicenter cross-sectional study. Cardiac imaging was performed within 3 months of enrollment. The majority of the subjects (64%) were taking two or more medications. Antithrombotics were taken by 86% of those with a history of stroke, thrombosis, or both and 67% of those without such a history (P = 0.01). Conversely, 14% of those with a history of stroke, thrombosis, or both were taking no antithrombotic. Angiotensin-converting enzyme inhibitor (ACEi) therapy was independently associated with moderate or severe atrioventricular valve regurgitation (P = 0.004), right ventricular morphology (P < 0.001), and shorter time since Fontan (P = 0.004) but not with ventricular systolic dysfunction. Glycoside therapy and diuretic therapy each was associated with older age at Fontan (P = 0.001 and P = 0.023, respectively) and a history of post-Fontan arrhythmia (P < 0.001 and P = 0.003, respectively) but not with ventricular systolic dysfunction. Medication use rates varied widely among the centers, even with controls for center differences in patient characteristics. Prospective therapeutic trials are needed to guide the medical treatment of Fontan patients.

Impact of Prenatal Diagnosis and Anatomical Subtype on Outcome in Double Outlet Right Ventricle

We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy.

Validation of the Actiheart Activity Monitor for Measurement of Activity Energy Expenditure in Children and Adolescents with Chronic Disease

The purpose of this study was to develop an activity energy expenditure (AEE) prediction equation for the Actiheart activity monitor for use in children with chronic disease.

Living at an Altitude Adversely Affects Exercise Capacity in Fontan Patients

Data assessing the effect of altitude on Fontan haemodynamics are limited to experimental models and case reports. Both suggest a detrimental impact. This study describes exercise performance in patients with Fontan circulation and matched controls at a low altitude versus at sea level. We sought to assess the impact of increasing altitude on functional capacity in patients with Fontan palliation.

Echocardiographic Definition and Surgical Decision-making in Unbalanced Atrioventricular Septal Defect: a Congenital Heart Surgeons' Society Multiinstitutional Study

Although identification of unbalanced atrioventricular septal defect (AVSD) is obvious when extreme, exact criteria to define the limits of unbalanced are not available. We sought to validate an atrioventricular valve index (AVVI) (left atrioventricular valve area/total atrioventricular valve area, centimeters squared) as a discriminator of balanced and unbalanced forms of complete AVSD and to characterize the association of AVVI with surgical strategies and outcomes.

Cor Triatriatum Sinistrum in Childhood. A Single Institution's Experience

Cor triatriatum sinistrum (CTS) is a rare congenital cardiac defect that can present with a wide range of symptoms and may be associated with other structural cardiac defects. Very limited data are available for pediatric patients.

Determinants of Short- and Long-term Outcomes Following Triple Valve Surgery

Triple valve surgery (3VS) is required in some cardiac disorders that simultaneously involve the aortic valve (AV), mitral valve (MV), and tricuspid valve (TCV). Herein are reported details of the authors' experience with 3VS, and their attempts to identify those factors associated with a poor clinical outcome.

Myocardial Tissue Doppler Velocity Imaging in Children: Comparative Study Between Two Ultrasound Systems

The investigators compared pulsed-wave tissue Doppler (PWTD) and color tissue Doppler (CTD) ventricular tissue velocities obtained using Vivid 7 (GE) and iE33 (Philips) ultrasound systems within a 30-minute time frame and examined interobserver and intraobserver variability.

Anthropometric Growth and Utilization of Enteral Feeding Support in Pediatric Heart Transplant Recipients

We sought to outline trends in anthropometric growth before and after cardiac transplantation and to document our experience with the use of EFS in this population. A total of 130 patients (59% male) were enrolled and followed for a median of 4.4 yr after transplantation. Negative changes over time in weight z-score (EST: -0.256 [0.160] z/yr, p = 0.01), height z-score (EST: -0.214 [0.096] z/yr, p = 0.03), and BMI z-score (EST: -0.287 [0.161] z/yr, p = 0.07) were observed prior to transplantation. Significant increases in weight z-score (EST: +0.342 [0.143] z/yr, p < 0.001) and BMI z-score (EST: +0.396 [0.140] z/yr, p = 0.005) were seen in the first 18 months following transplantation. No further increases in height, weight, or BMI z-score were seen beyond this. Forty-two (32%) patients received EFS. Prior to transplantation, it was not found to be associated with change in anthropomorphic growth. Post-transplantation exposure to EFS was associated with a faster increase in weight z-score (EST: +0.480 [0.231] z/yr, p = 0.04) and height z-score over time (EST: +0.366 [0.141] z/yr, p = 0.01). Normalization of weight and height z-scores was not achieved during the study follow-up period. This study suggests that further investigation into the role of EFS is warranted to identify strategies to improve growth in pediatric heart transplant recipients.

Comparison Between Different Speckle Tracking and Color Tissue Doppler Techniques to Measure Global and Regional Myocardial Deformation in Children

Myocardial deformation parameters obtained by speckle-tracking echocardiography (STE) and color Doppler tissue imaging (CDTI) using two different ultrasound systems and three different software packages were compared.

Usefulness of the Right Ventricular Systolic to Diastolic Duration Ratio to Predict Functional Capacity and Survival in Children with Pulmonary Arterial Hypertension

The objective of this study was to investigate the systolic to diastolic duration ratio (S:D ratio) in children with pulmonary arterial hypertension (PAH) and its association with right ventricular (RV) performance, hemodynamics, 6-minute walk test, clinical outcomes, and survival. We reviewed 503 serial echocardiograms in 47 children with PAH (mean pulmonary artery pressure >or=25 mm Hg) and compared the S:D ratio, assessed from Doppler flow of tricuspid valve regurgitation, to that in 47 age-matched controls. We reviewed echocardiograms, catheterization data, 6-minute walk tests, clinical data, lung transplantation, and death and used univariate linear regression models with a maximum likelihood algorithm for parameter estimation to investigate associations between S:D ratio and RV function, hemodynamics, functional capacity, and clinical outcomes. The S:D ratio was significantly higher in patients than in controls (1.38 +/- 0.61 vs 0.72 +/- 0.16, p <0.001). A higher S:D ratio was associated with worse echocardiographic RV fractional area of change, worse catheterization hemodynamics, shorter 6-minute walk distance, and worse clinical outcomes independent of pulmonary resistance or pressures. An increase of 0.1 in the S:D ratio was associated with a 13% increase in yearly risk for lung transplantation or death (hazard ratio 1.13, p <0.001). An S:D ratio 1.00 to 1.40 was associated with a moderate risk and an S:D ratio >1.40 was associated with a high risk of a negative outcome. In conclusion, in children with PAH, an increased S:D ratio is temporally associated with worse RV function, hemodynamics, exercise capability, clinical status, and survival.

Enalapril in Infants with Single Ventricle: Results of a Multicenter Randomized Trial

Angiotensin-converting enzyme inhibitor therapy improves clinical outcome and ventricular function in adults with heart failure. Infants with single-ventricle physiology have poor growth and are at risk for abnormalities in ventricular systolic and diastolic function. The ability of angiotensin-converting enzyme inhibitor therapy to preserve ventricular function and improve somatic growth and outcomes in these infants is unknown.

Parental Anxiety Associated with Kawasaki Disease in Previously Healthy Children

The objective of this study was to explore the lived experience of parents of children diagnosed with Kawasaki disease (KD) and to identify factors associated with increased levels of parental anxiety.

Cardiac Outcomes in Young Adult Survivors of the Arterial Switch Operation for Transposition of the Great Arteries

We sought to determine cardiac outcomes in young adults with complete transposition of the great arteries (TGA) after the arterial switch operation (ASO).

Functional Health Status in Children Following Surgery for Congenital Heart Disease: a Population-based Cohort Study

Functional health is becoming an important part of outcome assessment following congenital heart surgery.

Mitral Valve Replacement with the Quattro Stentless Pericardial Bioprosthesis: Mid-term Clinical and Echocardiographic Follow Up

Preservation of the subvalvar apparatus during mitral valve replacement (MVR) is associated with improved ventricular function and patient outcome. The Quattro valve is a chordally supported stentless mitral valve bioprosthesis that undergoes anticalcification treatment and is sutured to both annulus and papillary muscles heads, thus preserving annuloventricular continuity. The study aim was to assess the mid-term hemodynamic and functional results following MVR using the Quattro valve.

Superior Results Following the Ross Procedure in Patients with Congenital Heart Disease

The Ross procedure is a versatile operation that can be applied for aortic valve replacement (AVR) in patients with congenital heart disease (CHD), including small infants and those with complex left ventricular outflow tract (LVOT) obstruction. Herein, the clinical outcome is reported following the Ross procedure in patients with CHD at the authors' institution.

Intravenous Immunoglobulin Preparation Type: Association with Outcomes for Patients with Acute Kawasaki Disease

To determine whether two different intravenous immunoglobulin (IVIG) preparations were equally efficacious in the treatment of Kawasaki disease (KD). Single centre retrospective review of all patients treated with IVIG for KD between January 1990 and April 2007. Comparison of IVIG (dose 2 g/kg) from two commercial preparations; Iveegam stabilized with sugar (lyophilized, 5 g/ml glucose, pH 6.4-7.2, IgA 10 microg/ml, 5% IgG/ml) and Gamimune stabilized through acidification (no sugar, pH 4.0-4.5, IgA 270 microg/ml, 5% 1990-1999, 10% 1999-2007 IgG/ml). Propensity-adjusted differences in duration of fever after treatment initiation, frequency of retreatment with IVIG, hospital stay and maximum coronary artery z-score. A total of 954 patients were included, 862 (90%) were treated with Iveegam and 92 (10%) were treated with Gamimune. Patients' demographic, clinical and laboratory characteristics were similar between the two groups. In propensity-adjusted models, Iveegam was found to be associated with higher probability of non-response to IVIG (12% vs. 5%, p = 0.05) and longer median duration of fever after IVIG [1 (1-27) vs. 1 (1-8) days, p = 0.02] than Gamimune. Nevertheless, Gamimune was found to be associated with longer median duration of hospital stay [5 (2-49) vs. 4 (2-76) days, p < 0.0001] and higher median maximum coronary artery z-score both at the end of the acute phase (+1.4 vs. +0.8, p < 0.0001) and 6-8 weeks after the acute phase (+0.7 vs. +0.4, p < 0.0001). IVIG preparations with lower IgA content and stabilized with glucose appear to be associated with improved coronary artery outcomes for patients with KD.

Outcomes with Ventricular Assist Device Versus Extracorporeal Membrane Oxygenation As a Bridge to Pediatric Heart Transplantation

Extracorporeal membrane oxygenation (ECMO) has long been the sole means of mechanical support for pediatric patients with end-stage cardiac failure, but has a high waitlist mortality and a reported survival to hospital discharge of less than 50%. The purpose of this study was to compare waitlist mortality and survival for ECMO versus ventricular assist device (VAD) support. A review was conducted of all patients listed for heart transplantation (HTx) since 2002 and requiring mechanical support. VAD support has been available from 2004 (Berlin Heart Excor Pediatrics). Competing risks analysis was used to model survival to one of four outcomes (HTx, death on waitlist, delisting, improvement). Thirty-six patients were on mechanical support while awaiting HTx (21 ECMO, 12 VAD, three both). Median age at listing was 1.2 years (birth-16.6 years) for ECMO and 11.3 years (0.3-14.6 years) for VAD. Diagnosis was cardiomyopathy in 33% for ECMO and 93% for VAD. Median time to HTx was 37 days (1-930) overall, 20 days (1-85) for ECMO, and 39 days (5-108) for VAD. Mechanical support was associated with increased odds of HTx (hazard ratio [HR] 2.4 [1.7-3.3], P < 0.0001) but also delisting or death waiting (HR 3.0 [1.1-7.8], P = 0.03). Waitlist mortality of 38% on ECMO was reduced to 13% with VAD use. Survival post-HTx to hospital discharge was better in the group on VAD support (92 vs. 80%). Pediatric patients requiring mechanical support as a bridge to HTx have short wait times but high waitlist mortality. Those patients who survived to be put on the Berlin Heart Excor Pediatric device based on individualized clinical decision making then had a lower waitlist mortality, a longer duration of support, and a higher survival to transplantation and hospital discharge.

Haemodynamic Changes After Delivery Room Surfactant Administration to Very Low Birth Weight Infants

Surfactant replacement therapy (SRT) reduces respiratory morbidity and mortality in premature infants. The goal of this study was to characterise the effects of delivery room SRT on the ductus arteriosus and early neonatal haemodynamics.

Comparison of Shunt Types in the Norwood Procedure for Single-ventricle Lesions

The Norwood procedure with a modified Blalock-Taussig (MBT) shunt, the first palliative stage for single-ventricle lesions with systemic outflow obstruction, is associated with high mortality. The right ventricle-pulmonary artery (RVPA) shunt may improve coronary flow but requires a ventriculotomy. We compared the two shunts in infants with hypoplastic heart syndrome or related anomalies.

Macrophage Activation Syndrome in the Acute Phase of Kawasaki Disease

Rare cases of macrophage activation syndrome (MAS) occurring during the acute phase of Kawasaki disease (KD) have been reported. We sought to characterize, review treatment, and outcomes of KD patients with clinical features of MAS. Medical histories of patients treated for KD and MAS between January 2001 and March 2008 were reviewed. Of 638 KD patients seen, 12 (1.9%) had additional clinical findings usually associated with MAS; 7 of them were males older than 5 years (6.1%; odds ratio: 6.8, P=0.002). Clinically, 9 patients had at least 4 of 5 KD clinical signs, and all patients had prolonged fever beyond initial intravenous immunoglobulin treatment. Hepatosplenomegaly, cytopenia in two or more cell lines, hypertriglyceridemia and/or hypofibrinogenemia, and increased D-dimers were seen in 11 patients. Hyperferritinemia and elevated hepatic enzymes were seen in all patients. Four patients had biopsy-proven evidence of hemophagocytosis. All but 2 patients met at least 5 of 8 criteria necessary for MAS diagnosis. Treatment beyond the standard KD protocol (aspirin + intravenous immunoglobulin) was necessary in all but 1 patient. All patients eventually recovered with no long-term sequelae. A high index of suspicion for clinical features associated with MAS is warranted for KD patients to provide appropriate and timely treatment.

Management and Monitoring of Anticoagulation for Children Undergoing Cardiopulmonary Bypass in Cardiac Surgery

Cardiopulmonary bypass (CPB) creates a pro-coagulant state by causing platelet activation and inflammation leading to thrombin generation and platelet dysfunction. It is associated with severe derangements in normal homeostasis resulting in both thrombotic and hemorrhagic complications. This derangement is greater in children with congenital heart disease than in adults because of the immaturity of the coagulation system, hemodilution of coagulation factors, hyperreactive platelets, and in some patients, physiologic changes associated with cyanosis. During CPB, an appropriate amount of heparin is given with the goal of minimizing the risk of thrombosis and platelet activation and at the same time reducing the risk of bleeding from over anticoagulation. In young children, this balance is more difficult to achieve because of inherent characteristics of the hemostatic system in these patients. Historically, protocols for heparin dosing and monitoring in children have been adapted from adult protocols without re-validation for children. Extreme hemodilution of coagulation factors and platelets in young children affects the accuracy of anticoagulation monitoring in children. The activated clotting time does not correlate with plasma levels of heparin. In addition, recent studies suggest that children need larger doses of heparin than adults, because they have lower antithrombin levels, and they metabolize heparin more rapidly. Preliminary studies demonstrated that the use of individualized heparin and protamine monitoring and management in children is associated with reduced platelet activation and dysfunction and improved clinical outcomes. However, this review article clearly establishes that further studies are necessary to obtain evidence-based protocols for the proper management of anticoagulation of children undergoing cardiopulmonary bypass.

Long-term Anticoagulation in Kawasaki Disease: Initial Use of Low Molecular Weight Heparin is a Viable Option for Patients with Severe Coronary Artery Abnormalities

Patients with severe coronary artery involvement after Kawasaki disease (KD) require long-term systemic anticoagulation. We sought to compare our experience with thrombotic coronary artery occlusions, safety profile, and degree of coronary artery aneurysm regression in KD patients treated with low molecular weight heparin (LMWH) versus warfarin. Medical records of all KD patients diagnosed between January 1990 and April 2007 were reviewed. Of 1374 KD patients, 38 (3%) received systemic anticoagulation, 25 patients received LMWH from diagnosis onward, 12 of whom were subsequently switched to warfarin, and 13 received warfarin from onset. The frequency of thrombotic coronary artery occlusions was similar between drugs. Severe bleeding was more frequent in patients on warfarin, but minor bleeding was more frequent for patients on LMWH. Patients on warfarin were at greater risk of underanticoagulation or overanticoagulation (defined as achieving an anti-activated factor X level or an international normalized ratio below or above target level) than patients on LMWH (P < 0.05). Maximum coronary artery aneurysm z-scores diminished with time for patients on LMWH (P = 0.03) but not for those on warfarin (P = 0.55). This study suggests that LMWH is a potentially viable alternative for patients, especially young ones, with severe coronary artery involvement after KD.

Recurrent Exacerbations of Protein-losing Enteropathy After Initiation of Growth Hormone Therapy in a Fontan Patient Controlled with Spironolactone

Protein-losing enteropathy (PLE) is a rare, but serious complication in single ventricle patients after Fontan palliation, and is associated with a 5-year mortality of 46%. We describe a patient with PLE after Fontan palliation who achieved remission with high-dose spironolactone (an aldosterone antagonist), but had three exacerbations each temporally correlated with the use of growth hormone (an aldosterone agonist). Because of the opposing mechanisms of action of these two medications, caution might be indicated when using growth hormone for patients with PLE who are successfully treated with spironolactone.

The Ross Procedure in Children: Preoperative Haemodynamic Manifestation Has Significant Effect on Late Autograft Re-operation

The Ross procedure is the aortic valve-replacement procedure of choice in children. Nonetheless, late autograft re-operation for dilatation and/or valve regurgitation is of concern. We examined whether preoperative haemodynamic manifestation (e.g., stenosis, regurgitation and mixed aortic valve disease) affected late re-operation risk.

Percutaneous Pulmonary Valve Implantation in the Young 2-year Follow-up

The aim of this study was to investigate physiological and clinical consequences of percutaneous pulmonary valve implantation (PPVI) in patients with chronic right ventricular outflow tract (RVOT) obstruction and volume overload.

The Prevalence and Clinical Impact of Obesity in Adults with Marfan Syndrome

Patients with Marfan syndrome characteristically have an asthenic body habitus and are considered to be exempt from the obesity epidemic.

Intermediate Results Following Complex Biventricular Repair of Left Ventricular Outflow Tract Obstruction in Neonates and Infants

Although the majority of infants with severe left ventricular outflow tract obstruction (LVOTO) can be managed with balloon or surgical aortic valvotomy, a more complex biventricular repair may be required in a subset of infants with multi-level obstruction, failure of or complication to prior intervention. In the presence of normal left ventricle size and inflow, the Ross procedure is applied in patients with/without ventricular septal defect (VSD), while the Yasui procedure is applied only in those with VSD. We report mid-term outcomes in a single institution.

Post-transplant Lymphoproliferative Disorder in Pediatric Heart Transplant Recipients

Post-transplantation lymphoproliferative disorder (PTLD) is a major cause of morbidity and mortality after pediatric heart transplantation.

Results After Mitral Valve Replacement with Mechanical Prostheses in Young Children

We examined outcomes after mitral valve replacement in children younger than 8 years.

Efficacy and Safety of Rosuvastatin Therapy for Children with Familial Hypercholesterolemia

This study was undertaken to evaluate the efficacy and safety of rosuvastatin therapy for children with familial hypercholesterolemia.

Report of the National Heart, Lung, and Blood Institute's Working Group on Obesity and Other Cardiovascular Risk Factors in Congenital Heart Disease

Rheumatic Disease and Carotid Intima-media Thickness: a Systematic Review and Meta-analysis

To perform a systematic review and meta-analysis to examine whether rheumatic disease is associated with an increased carotid intima-media thickness (CIMT; increasingly used as a surrogate marker for atherosclerosis) when compared with healthy control subjects.

MEALTRAIN: What Do Inpatient Hospitalized Children Choose to Eat?

Data from the menu ordering system was used to calculate servings/child/day of food groups ordered by pediatric inpatients. The children ordered a mean of 5.0 +/- 0.56 servings of fruits and vegetables/day, which decreased to 3.5 +/- 0.27 servings when juice was removed. "Foods-to-limit" were ordered 3.8 +/- 0.31 times/day. Interventions to improve healthier choices are warranted.

Repeated Systematic Surveillance of Kawasaki Disease in Ontario from 1995 to 2006

Rising incidences of Kawasaki disease (KD) have been reported worldwide. Reported herein are the results of 4 triennial KD surveillances conducted in Ontario.

Outcomes of Prenatally Diagnosed Tetralogy of Fallot: Implications for Valve-sparing Repair Versus Transannular Patch

To assess outcomes of prenatally diagnosed tetralogy of Fallot and determine factors associated with the choice to undergo a valvesparing repair versus transannular patch, and the use of prostaglandins at birth.

Metal and Anion Composition of Two Biopolymeric Chemical Stabilizers and Toxicity Risk Implication for the Environment

The objective of this study was to (1) measure the concentration of four anions (Cl(-), F(-), [image omitted], and [image omitted]) and nine other elements (Al, Ba, Ca, K, Mg, Mn, Fe, Ni, and Si) in two nontraditional biopolymeric chemical stabilizers (EBCS1 and EBCS2), (2) investigate consequent environmental toxicity risk implications, and (3) create awareness regarding environmental health issues associated with metal concentration levels in enzyme-based chemical stabilizers that are now gaining widespread application in road construction and other concrete materials. Potential ecotoxicity impacts were studied on aqueous extracts of EBCS1 and EBCS2 using two thermodynamic properties models: the Pitzer-Mayorga model (calculation of the electrolyte activity coefficients) and the Millero-Pitzer model (calculation of the ionic activity coefficients). Results showed not only high concentrations of a variety of metal ions and inorganic anions, but also a significant variation between two chemical stabilizing mixtures. The mixture (EBCS2) with the lower pH value was richer in all the cationic and anionic species than (EBCS1). Sulfate (SO(2-)(4)) concentrations were found to be higher in EBCS2 than in EBCS1. There was no correlation between electrolyte activity and presence of the ionic species, which may be linked to a possible high ionic environmental activity. The concentrations of trace metals found (Mn, Fe, and Ni) were low compared to those of earth metals (Ba, Ca, K, and Mg). The metal concentrations were higher in EBCS1 than in EBCS2. Data suggest that specific studies are needed to establish "zero" permissible metal ecotoxicity values for elements and anions in any such strong polyelectrolytic enzyme-based chemical stabilizers.

Assessment and Management of Hypertension in Children and Adolescents

The epidemic of overweight and obesity in youth is increasing the prevalence of prehypertension and hypertension among children and adolescents. The younger the child is at presentation and the more severe the blood pressure abnormality, the more likely a secondary cause of hypertension is to be present. Measurement of blood pressure in children requires adaptation to the age and size of the child. Interpretation must be related to normative values specific for age, sex, and height. Evaluation is primarily aimed at identifying secondary causes of hypertension, associated comorbidities, additional risk factors, and evidence of target-organ damage. Ambulatory blood pressure monitoring is emerging as a useful tool for evaluation of some patients, particularly for those with suspected 'white coat' hypertension. Management of prehypertension and hypertension is directed at the underlying cause, exacerbating factors, and the magnitude of the blood pressure abnormality. Healthy behavioral changes are a primary management tool for treating hypertension and, more particularly, prehypertension and for addressing other cardiovascular risk factors, such as obesity. Pharmacological management is reserved for patients with hypertension who do not respond to behavioral changes, have additional cardiovascular risk factors or diabetes, are symptomatic, or have developed target-organ damage.

Equivalent Outcomes for Pediatric Heart Transplantation Recipients: ABO-blood Group Incompatible Versus ABO-compatible

ABO-blood group incompatible infant heart transplantation has had excellent short-term outcomes. Uncertainties about long-term outcomes have been a barrier to the adoption of this strategy worldwide. We report a nonrandomized comparison of clinical outcomes over 10 years of the largest cohort of ABO-incompatible recipients. ABO-incompatible (n = 35) and ABO-compatible (n = 45) infant heart transplantation recipients (< or =14 months old, 1996-2006) showed no important differences in pretransplantation characteristics. There was no difference in incidence of and time to moderate acute cellular rejection. Despite either the presence (seven patients) or development (eight patients) of donor-specific antibodies against blood group antigens, in only two ABO-incompatible patients were these antibodies implicated in antibody-mediated rejection (which occurred early posttransplantation, was easily managed and did not recur in follow-up). Occurrence of graft vasculopathy (11%), malignancy (11%) and freedom from severe renal dysfunction were identical in both groups. Survival was identical (74% at 7 years posttransplantation). ABO-blood group incompatible heart transplantation has excellent outcomes that are indistinguishable from those of the ABO-compatible population and there is no clinical justification for withholding this lifesaving strategy from all infants listed for heart transplantation. Further studies into observed differing responses in the development of donor-specific isohemagglutinins and the implications for graft accommodation are warranted.

Laboratory Measures of Exercise Capacity and Ventricular Characteristics and Function Are Weakly Associated with Functional Health Status After Fontan Procedure

Patients after the Fontan procedure are at risk for suboptimal functional health status, and associations with laboratory measures are important for planning interventions and outcome measures for clinical trials.

Compounds Structurally Related to Dechlorane Plus in Sediment and Biota from Lake Ontario (Canada)

The historical occurrence of Dechlorane Plus (DP) and detection of novel compounds structurally related to DP is described in a dated Lake Ontario sediment core. Our core was collected near the mouth of the Niagara River, which is known to be a major source of DP to the lake. Maximum DP concentrations (920 ng g(-1), dry weight) were observed between 1976 and 1980, the highest reported to date. Following that time, we observed a dramatic decrease in DP concentration which coincided with the enactment of United States federal and state laws to mitigate free release of chemicals into the Niagara River and installation of an industrial wastewater treatment facility. During the course of our research, four new substances structurally related to DP were also identified. These compounds were thought to arise from the Diels-Alder reactions resulting from impurities present in 1,5-cyclooctadiene, a feedstock used in production of DP. To confirm our hypothesis, Diels-Alder reactions were performed on the individual impurities. Using different stationary-phase capillary gas chromatography columns and high-resolution mass spectrometry, we were able to positively identify some of these novel compounds in the core. Interestingly, we also were able to identify a monoadduct compound, formed by addition of 1 mol of hexachlorocyclopentadiene to 2 mol of 1,3-cyclooctadiene, in lake trout. The concentration of this monoadduct was approximately 2 orders of magnitude greater than that of DP, suggesting that it is more bioaccumulative.

Improved Classification of Coronary Artery Abnormalities Based Only on Coronary Artery Z-scores After Kawasaki Disease

Competing definitions and classifications of coronary artery abnormalities (CAAs) after Kawasaki disease (KD) have been arbitrarily defined based on clinical experience. We sought to propose a classification system for CAAs based only on coronary artery z-scores. All echocardiograms performed between 1990 and 2007 on patients with a previous history of KD were reviewed. Coronary artery luminal dimensions were converted to body-surface-area-adjusted z-scores and compared to current classification systems. A total of 1356 patients with a previous history of KD underwent 4379 echocardiograms. There was important overlap in the distributions of coronary artery z-scores between the different CAA classes as defined by the American Heart Association (AHA). The AHA classification underestimated the severity of CAAs in 19-32% of small CAAs and 35-78% of medium CAAs. We determined the optimal definition of CAA to be small if the z-score is >or=2.5 to <5.0, large if the z-score is >or=5.0 to <10.0, and giant if the z-score is >or=10.0. This classification seems to appropriately apply to the circumflex branch despite a lack of normal values for this branch. The current AHA classification might not accurately classify CAAs in KD patients. Accurate classification is important for defining management and prognosis consistently across patient age and size.

Lesion-specific Outcomes in Neonates Undergoing Congenital Heart Surgery Are Related Predominantly to Patient and Management Factors Rather Than Institution or Surgeon Experience: A Congenital Heart Surgeons Society Study

To identify the role of institution and surgeon factors, including case volume and experience, on survival of neonates with complex congenital heart disease.

Ross and Yasui Operations for Complex Biventricular Repair in Infants with Critical Left Ventricular Outflow Tract Obstruction

To define the outcomes following Ross and Yasui procedures for complex biventricular repair of critical left ventricular outflow tract obstruction (LVOTO).

Management Recommendations for Metabolic Complications Associated with Second-generation Antipsychotic Use in Children and Youth

BACKGROUND: Second-generation antipsychotics are commonly associated with metabolic complications. These medications are being used more frequently for the treatment of mental health disorders in children, which has stimulated the need for creating formal guidelines on monitoring their safety and effectiveness. Previous guidelines have been developed for monitoring metabolic and neurological complications. To assist practitioners who perform these monitoring procedures, a complementary set of treatment recommendations have been created for situations in which abnormal measurements or results are encountered. OBJECTIVE: To create evidence-based recommendations to assist in managing metabolic complications in children being treated with second-generation antipsychotics. METHODS: A systematic review of the literature on metabolic complications of second-generation antipsychotic medications in children was conducted. Members of the consensus group evaluated the information gathered from the systematic review of the literature and used a nominal group process to reach a consensus on treatment recommendations. Wherever possible, references were made to existing guidelines on the evaluation and treatment of metabolic abnormalities in children. RESULTS: Evidence-based recommendations are presented to assist in managing metabolic complications including weight gain; increased waist circumference; elevation in prolactin, cholesterol, triglyceride and glucose levels; abnormal liver function tests and abnormal thyroid studies. CONCLUSION: The use of second-generation antipsychotics requires proper monitoring procedures. The present treatment guideline provides guidance to clinicians on the clinical management of metabolic complications if they occur.

The 'Golden Keys' to Health - a Healthy Lifestyle Intervention with Randomized Individual Mentorship for Overweight and Obesity in Adolescents

To conduct a pilot study designed to measure the impact of a healthy lifestyle intervention with or without individualized mentorship on adiposity, metabolic profile, nutrition and physical activity in overweight teens.

Novel Methoxylated Polybrominated Diphenoxybenzene Congeners and Possible Sources in Herring Gull Eggs from the Laurentian Great Lakes of North America

An increasing number of brominated flame retardants and other brominated substances are being reported in herring gull eggs from the Laurentian Great Lakes basin. Yet, in extracts from gulls' eggs, numerous bromide anion response peaks in electron capture negative ion (ECNI) mass chromatograms remain unidentified. Using archived herring gull egg homogenates, we characterize the structures of three major and three minor, new and unique brominated substances. After extensive cleanup and separation to isolate these substances from the extracts, high-quality ECNI and electron impact (EI) mass spectra revealed fragmentation patterns consistent with congeners of methoxylated polybrominated diphenoxybenzene (MeO-PBDPB), where four congeners contained five bromines and the other two contain four and six bromines, respectively. Optimized, semiquantitative analysis revealed sum concentrations of the MeO-PBDBP congeners ranged from <0.2 to 36.8 ng/g ww in pooled egg homogenates (collected in 2009) from fourteen herring gull colony sites across the Great Lakes, with the highest concentration being for Channel-Shelter Island in Saginaw Bay (Lake Huron). To our knowledge, there are no published reports on the environmental presence and sources of MeO-PBDPBs. We hypothesize that these MeO-PBDPBs are degradation products of the polybrominated diphenoxybenzenes, for example, tetradecabromodiphenoxybenzene (currently marketed as SAYTEX 120) or polybromo 3P2E. MeO-PBDPBs in Great Lakes herring gull eggs indicates their bioaccumulation potential, and raises concerns about their origin, environmental behavior and influences on wildlife and environmental health.

A Growing Problem: Maternal Death and Peripartum Complications Are Higher in Women with Grown-up Congenital Heart Disease

As patients with grown-up congenital heart disease (GUCH) increase, more women with GUCH will become pregnant. Heart surgeons may be involved in maternal GUCH care, yet the prevalence, characteristics, and outcomes for these women are unknown. We determined the national prevalence of GUCH parturients, their diagnostic makeup, and whether they have increased risk of peripartum complications, maternal or fetal death.

Comparison of Transplacental Treatment of Fetal Supraventricular Tachyarrhythmias with Digoxin, Flecainide, and Sotalol: Results of a Nonrandomized Multicenter Study

Fetal tachyarrhythmia may result in low cardiac output and death. Consequently, antiarrhythmic treatment is offered in most affected pregnancies. We compared 3 drugs commonly used to control supraventricular tachycardia (SVT) and atrial flutter (AF).

Feasibility of Very-high Resolution Ultrasound to Assess Elastic and Muscular Arterial Wall Morphology in Adolescents Attending an Outpatient Clinic for Obesity and Lipid Abnormalities

Atherosclerosis begins during early life and is accelerated in individuals with cardiovascular risk factors. We hypothesized that very-high resolution ultrasound (VHRU, 25-55 MHz) could feasibly detect early arterial changes in adolescents with risk factors.

Risk, Clinical Features, and Outcomes of Thrombosis Associated with Pediatric Cardiac Surgery

Thrombosis, usually considered a serious but rare complication of pediatric cardiac surgery, has not been a major clinical and/or research focus in the past.

Pulmonary Arterial Capacitance in Children with Idiopathic Pulmonary Arterial Hypertension and Pulmonary Arterial Hypertension Associated with Congenital Heart Disease: Relation to Pulmonary Vascular Resistance, Exercise Capacity, and Survival

Pediatric pulmonary arterial hypertension (PAH), whether idiopathic PAH (iPAH) or PAH associated with congenital heart disease (aPAH), carries high morbidity and mortality. Low pulmonary arterial capacitance (PAC), defined as right ventricular stroke volume/pulmonary artery pulse pressure, is a risk factor for mortality in adults with PAH. However, the relation of PAC to pulmonary vascular resistance (PVR), exercise endurance, and survival is poorly defined in children.

Surgical Interventions for Atrioventricular Septal Defect Subtypes: the Pediatric Heart Network Experience

The influence of atrioventricular septal defect (AVSD) subtype on outcomes after repair is poorly understood.

Recommendations for Physical Activity, Recreation Sport, and Exercise Training in Paediatric Patients with Congenital Heart Disease: a Report from the Exercise, Basic & Translational Research Section of the European Association of Cardiovascular Prevention and Rehabilitation, the European Congenital Heart and Lung Exercise Group, and the Association for European Paediatric Cardiology

All children have a natural need to move, play, and perform activities. Physical activity is necessary for optimal physical, emotional, and psychosocial development for healthy children as well as children with congenital heart disease (CHD). In this paper we provide recommendations for physical activity, recreational sport, and exercise training in children and adolescents with CHD. In general, children with CHD should be advised to comply with public health recommendations of daily participation in 60 min or more of moderate-to-vigorous physical activity that is developmentally appropriate and enjoyable and involves a variety of activities. While all patients with CHD can participate and benefit from physical activity and exercise, those with specific lesions or complications may require counselling regarding precautions and recommendations.

Management Recommendations for Metabolic Complications Associated with Second Generation Antipsychotic Use in Children and Youth

Second generation antipsychotics (SGAs) are commonly associated with metabolic complications. These medications are being used more frequently for the treatment of mental health disorders in children, which has stimulated the need for creating formal guidelines on monitoring their safety and effectiveness. Previous guidelines have been developed for monitoring for metabolic and neurological complications. In order to assist practitioners who perform these monitoring procedures, we have created a complementary set of treatment recommendations if abnormal measurements or results are encountered.

A Multicenter, Randomized Trial Comparing Heparin/warfarin and Acetylsalicylic Acid As Primary Thromboprophylaxis for 2 Years After the Fontan Procedure in Children

The purpose of this study was to compare the safety and efficacy of acetylsalicylic acid (ASA) and warfarin for thromboprophylaxis after the Fontan procedure.

Practice Variability and Outcomes of Coil Embolization of Aortopulmonary Collaterals Before Fontan Completion: a Report from the Pediatric Heart Network Fontan Cross-Sectional Study

The practice of coiling aortopulmonary collaterals (APCs) before Fontan completion is controversial, and published data are limited. We sought to compare outcomes in subjects with and without pre-Fontan coil embolization of APCs using the Pediatric Heart Network Fontan Cross-Sectional Study database which enrolled survivors of prior Fontan palliation.

Preparation and X-ray Structural Characterization of Further Stereoisomers of 1,2,5,6,9,10-hexabromocyclododecane

Technical 1,2,5,6,9,10-hexabromocyclododecane (HBCD) consists largely of three diastereomers (α-, β-, and γ-HBCD) produced by the trans addition of bromine to cis,trans,trans-cyclododeca-1,5,9-triene (CDT). However, another seven diastereomers are theoretically possible and may be produced by trans addition of bromine across the double bonds of the other three isomers of 1,5,9-CDT. There are indications that small amounts of the minor HBCD isomers may be present in commercial HBCD mixtures or in products containing this brominated flame retardant (BFR). Such minor components may indeed derive from traces of other 1,5,9-CDTs in the cis, trans, trans starting material, however their formation may also be possible through isomerizations during the processing of this BFR or by bioisomerization subsequent to its release into the environment. Two of the seven additional diastereomers (δ- and ε-HBCD) were synthesized previously from trans,trans,trans-CDT. We now report the preparation of the remaining five diastereomers, ζ-, η-, and θ-HBCD from cis,cis,trans-CDT and ι- and κ-HBCD from cis,cis,cis-CDT, and their characterization by (1)H NMR spectroscopy and X-ray crystallography. The availability of these further diastereomers of HBCDshould aid in determining if the minor isomers are present in commercial samples of this BFR, in products containing HBCDs, or in environmental samples. We have also carried out an X-ray crystal structure determination on ε-HBCD, so that crystal structures are now available for all 10 HBCD diastereomers.

Long-term Tricuspid Valve Function After Norwood Operation

Long-term functional outcomes after Norwood palliation are likely to depend on tricuspid valve performance in the systemic role. We therefore aimed to characterize features associated with tricuspid valve intervention.

A Comparison of Cardiac Output by Thoracic Impedance and Direct Fick in Children with Congenital Heart Disease Undergoing Diagnostic Cardiac Catheterization

To evaluate the measurement of cardiac output (CO) using continuous electrical bioimpedance cardiography (Physioflow; Neumedx, Philadelphia, PA) (CO(PF)) with a simultaneous direct Fick measurement (CO(FICK)) in children with congenital heart disease.

Motivational Interviewing As an Intervention to Increase Adolescent Self-efficacy and Promote Weight Loss: Methodology and Design

Childhood obesity is associated with serious physiological and psychological consequences including type 2 diabetes, higher rates of depression and low self-esteem. With the population of overweight and obese youth increasing, appropriate interventions are needed that speak to the issue of readiness to change and motivation to maintain adherence to healthy behavior changes. Motivational Interviewing (MI) is a method of therapy found to resolve ambivalence, enhance intrinsic motivation and promote confidence in a person's ability to make behavior changes. While MI has shown promise in the adult obesity literature as effecting positive lifestyle change, little is known about the effectiveness of MI with overweight and obese youth. This study aims to: 1) demonstrate that MI is an effective intervention for increasing a person's self-efficacy; 2) demonstrate that exposure to MI will facilitate healthy behavior changes; 3) explore psychological changes related to participation in MI and 4) compare physiological and anthropometric outcomes before and after intervention.

Late Status of Fontan Patients with Persistent Surgical Fenestration

This study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes.

Early Determinants of Atherosclerosis in Paediatric Systemic Lupus Erythematosus

To assess traditional and non-traditional cardiovascular risk factors and to determine the prevalence and correlates of early vascular markers of atherosclerosis in paediatric systemic lupus erythematosus (pSLE).

Nontraditional Risk Factors and Biomarkers for Cardiovascular Disease: Mechanistic, Research, and Clinical Considerations for Youth: a Scientific Statement from the American Heart Association

The rapid increase in the prevalence and severity of obesity in children is likely to lower the age of onset and increase the incidence of cardiovascular disease worldwide. Understanding the pathophysiology and improving the clinical management of cardiovascular disease involve a knowledge of novel risk factors and biomarkers. The clinical and mechanistic roles of these novel biological factors during childhood are currently being investigated. The goals of this scientific statement are to present the existing knowledge and theoretical framework of nontraditional risk factors for cardiovascular disease as they relate to children and adolescents, to describe the relevance and weight of available experimental and clinical evidence and the therapeutic implications pertaining to nontraditional risk factors in the pediatric population, and to stimulate further research with a goal of developing valid and reliable approaches to identify and validate novel risk factors that will aid in the clinical evaluation and perhaps prediction of cardiovascular disease in the pediatric population. Although several biomarkers are promising, substantial research is required before nontraditional risk factors can be used to identify and reduce cardiovascular disease risk in children and adolescents.

Toxoplasma in Animals, Food, and Humans: an Old Parasite of New Concern

All hosts, including humans, can be infected by any one of the three forms of the parasite Toxoplasma gondii that correspond to three morphological stages: tachyzoite, bradyzoite, and sporozoite form. Felids are definitive hosts for T. gondii, which is an intracellular pathogen that infects a wide range of warm-blooded intermediate hosts. Toxoplasmosis is a disease where the interest of the diverse medical and veterinary specialties converge. Awareness needs to be increased that toxoplasmosis can induce clinical disease not only in immunocompromised patients or through congenital infections, but also in healthy patients. This is a review article that aims at illustrating why toxoplasmosis should be regarded a veterinary public health issue and how veterinary practitioners can contribute in controlling the infection.

The Myocardium of Fetuses with Endocardial Fibroelastosis Contains Fewer B and T Lymphocytes Than Normal Control Myocardium

The observation that endocardial fibroelastosis (EFE) can result from an immune response to maternal autoantibody deposition in the fetal myocardium raises the possibility that the fetal immune system may contribute to the pathogenesis of idiopathic EFE and dilated cardiomyopathy (DCM). This study sought to characterize myocardial immune cell presence in fetuses and neonates with idiopathic EFE + DCM, in those with EFE + structural heart disease, and in normal control subjects. Paraffin tissue sections from fetuses identified from the pathology database were stained for B cell, T cell, macrophage, and general hematopoietic cell surface markers. Of the 14 fetuses included in the study, 5 had EFE + DCM, 4 had EFE + structural heart disease, and 5 were normal control fetuses. The EFE + DCM group had fewer B cells than the control group (0.15 vs. 0.44 cells/mm(2); p = 0.005). The EFE + heart disease group had both fewer B cells (0.18 vs. 0.44 cells/mm(2); p = 0.08) and T cells (0.29 vs. 0.80 cells/mm(2); p = 0.04) than the control group. The CD4/CD8 ratio was similar in the EFE + DCM and EFE + heart disease groups (1.0 vs. 0.9; p = 0.17) but higher in the EFE + DCM group than in the control group (0.9 vs. 0.3; p = 0.03). The myocardium of fetuses with EFE contains fewer B and T lymphocytes than normal control fetuses.

Environmental Implications of Material Leached from Coal

Samples of coal were collected from different seams at a South African coal mine and comparative leaching experiments were carried out under various pH conditions and times to investigate the leaching behavior and potential environmental impact of possibly hazardous elements such as As, Cd, Co, Cr, Mn, Ni, Pb, Th and U. The calculated leaching intensities, sequential extraction results and cumulative percentages demonstrate that the leaching behavior of the elements is strongly influenced by the pH, the leaching time and the properties and occurrences of the elements. The leached concentrations of As, Cd, Co, Cr, Mn, Ni and Pb exceeded the maximum concentrations recommended by the Environmental Protection Agency (EPA) for surface water.

Older Children at the Time of the Norwood Operation Have Ongoing Mortality Vulnerability That Continues After Cavopulmonary Connection

Delayed first-stage palliation of children with hypoplastic left heart syndrome and related pathologies can be associated with poor outcomes because of development of progressive pulmonary vascular disease and volume load effects on the systemic ventricle and atrioventricular valve. We examine the current era's survival in this subgroup.

Parental Factors Associated with Screen Time in Pre-school Children in Primary-care Practice: a TARGet Kids! Study

To identify child and parental factors associated with screen time in 3-year-old children.

Improved Outcomes Associated with Intraoperative Steroid Use in High-risk Pediatric Cardiac Surgery

Corticosteroids are commonly administered perioperatively in pediatric cardiac surgery to reduce cardiopulmonary bypass induced inflammation. However, their effects on outcomes and potential for adverse events are not well defined.

Factors Associated with Serum Brain Natriuretic Peptide Levels After the Fontan Procedure

Although a useful marker of heart failure in adults, the utility of brain natriuretic peptide concentration (BNP) for children after the Fontan procedure is not well studied.

Usefulness of Mitral Regurgitation As a Marker of Increased Risk for Death or Cardiac Transplantation in Idiopathic Dilated Cardiomyopathy in Children

In adults with idiopathic dilated cardiomyopathy (IDC), mitral regurgitation (MR) is associated with adverse prognosis and is often addressed by surgery or intervention. MR is commonly found in children with IDC, but its prognostic relevance has not been defined, and interventions to reduce MR are not routinely performed in this population. In this study, it was hypothesized that MR is an independent risk factor for death or transplantation. This was a single-center, retrospective study of sequential patients with IDC or familial IDC (left ventricular end-diastolic dimension z score >2 and ejection fraction <50%). Patients with acute myocarditis or previous mitral surgery were excluded. MR severity was graded according to American Society of Echocardiography guidelines as mild, moderate, or severe on the basis of MR jet vena contracta width. Left ventricular end-diastolic volume, end-systolic volume, and ejection fraction were measured by biplane Simpson's method. Forty-two children with IDC were studied. The mean follow-up period was 25 months. At initial assessment, 34 children (82%) were taking angiotensin-converting enzyme inhibitors, 25 (60%) furosemide, 27 (65%) β blockers, and 7 (17%) intravenous inotropes. The mean indexed end-systolic volume was 91 ± 51 ml/m(2). The mean ejection fraction was 27 ± 16%. MR was mild in 42%, moderate in 19%, severe in 2%, and absent in 35% of patients. MR severity progressed from initial to last evaluation. MR severity was an independent risk factor for lower freedom from death or transplantation. Progression in MR severity increased the annual hazard of death or transplantation by a factor of 2.4 (p = 0.003). In conclusion, MR severity is independently associated with worse clinical status and decreased freedom from death or transplantation in children with IDC.

The Role of Atorvastatin in Regulating the Immune Response Leading to Vascular Damage in a Model of Kawasaki Disease

Superantigens have been implicated in a number of diseases including Kawasaki disease (KD), a multi-system vasculitis resulting in coronary artery aneurysms. We have characterized a murine disease model in which coronary arteritis is induced by a novel superantigen found in Lactobacillus casei cell wall extract (LCWE). Using this animal model of KD, we have identified three pathogenic steps leading to coronary artery aneurysm formation. These steps include T cell activation and proliferation, production of the proinflammatory cytokine tumour necrosis factor (TNF)-α and up-regulation of matrix metalloproteinase 9 (MMP-9), an elastolytic protease. In addition to their cholesterol-lowering effects, 3-hydroxy-3-methylglutaryl (HMG) coenzyme A (CoA) reductase inhibitors (statins) have pleotropic immunomodulatory properties. Thus, we examined the effect of atorvastatin in modulating each of these three critical pathogenic processes leading to aneurysm formation in the disease model. Atorvastatin inhibited lymphocyte proliferation in response to superantigen stimulation in a dose-dependent manner. This inhibition was also observed for production of soluble mediators of inflammation including interleukin (IL)-2 and TNF-α. The inhibitory effect on proliferation was rescued completely by mevalonic acid, confirming that the mechanism responsible for this inhibitory activity on immune activation was inhibition of HMG-CoA reductase. Similarly, TNF-α-induced MMP-9 production was reduced in a dose-dependent manner in response to atorvastatin. Inhibition of extracellular-regulated kinase (ERK) phosphorylation appears to be the mechanism responsible for inhibition of MMP-9 production. In conclusion, atorvastatin is able to inhibit critical steps known to be important in the development of coronary aneurysms, suggesting that statins may have therapeutic benefit in patients with KD.

Outcomes and Associated Risk Factors for Mitral Valve Replacement in Children

We aim to report time-related outcomes following mitral valve replacement (MVR) in children and to identify factors affecting outcomes.

Factors Associated with the Physical Activity Level of Children Who Have the Fontan Procedure

Children with complex heart defects are sedentary, with activity level unrelated to exercise capacity. We sought to identify factors associated with physical activity level for children who have the Fontan procedure.

Effectiveness of Serial Increases in Amino-terminal Pro-B-type Natriuretic Peptide Levels to Indicate the Need for Mechanical Circulatory Support in Children with Acute Decompensated Heart Failure

We sought to determine prospectively whether serial assessment of the natriuretic peptide prohormone, amino-terminal pro-B-type natriuretic peptide (NT-pro-BNP), correlated with clinical severity and outcomes in children hospitalized for acute decompensated heart failure (ADHF). Patients (>1 month of age) admitted from 2005 to 2007 with ADHF requiring intravenous vasoactive/diuretic therapy for ADHF were eligible. Serum NT-pro-BNP levels were obtained within 24 hours of admission and at prespecified intervals, and clinical caregivers were blinded to these levels. End points included hospital discharge, death or cardiac transplantation, and care escalation including the need for mechanical circulatory support (MCS) was noted. Twenty-four patients were enrolled: 22 survived to hospital discharge and 2 died. Ten required MCS (of which 6 underwent cardiac transplantation). Two patients underwent transplantation without MCS. For the entire cohort, NT-pro-BNP levels peaked at days 2 to 3 after admission, with a subsequent gradual decrease until discharge. However, for those who did require MCS, NT-pro-BNP failed to decrease consistently until after MCS initiation. At discharge, NT-pro-BNP levels were significantly decreased from admission levels but remained well above normal for all patients. Single-point NT-pro-BNP levels on admission did not correlate with independently assessed clinical scores of heart failure severity or predict the need for MCS in this cohort. In conclusion, serial NT-pro-BNP levels demonstrated an incremental trend after 48 hours in patients who went on to require MCS but decreased in all other patients and may therefore assist the decision to initiate or avoid MCS after admission for pediatric ADHF.

Survival Implications: Hypertrophic Cardiomyopathy in Noonan Syndrome

To understand relationships and survival implications between structural heart disease and hypertrophic cardiomyopathy in Noonan syndrome (Noonan syndrome-HCM), we reviewed the clinical course of 138 children with Noonan syndrome diagnosed with cardiovascular abnormalities and compared survival with the 30 children with Noonan syndrome-HCM with 120 contemporaneous children with nonsyndromic HCM.

Toxicokinetics of Tetrabromoethylcyclohexane (TBECH) in Juvenile Brown Trout (Salmo Trutta) and Effects on Plasma Sex Hormones

Technical 1,2-dibromo-4-(1,2 dibromoethyl)cyclohexane or tetrabromoethylcyclohexane (TBECH) used primarily as an additive flame retardant in polystyrene foams, contains two diastereoisomers, α- and β- present in equimolar amounts. At temperatures in excess of 125°C, isomerization to two other isoforms, δ- and γ- is possible. The recent detection of TBECH in the environment and studies suggesting that isomers are androgenic prompted us to examine the toxicokinetics and biochemical effects of one of the isomers, β-, in a controlled laboratory environment. Juvenile brown trout (Salmo trutta) were exposed to three different amounts of the β-isomer (low, medium and high) via the food followed by a period in which they were exposed to unfortified food. A fourth group of fish was exposed to unfortified food for the duration of the experiment. On days 0, 7, 14, 21, 35, 49, 56, 63, 77, 91, 105, and 133, eight fish from each treatment group were euthanized and liver, plasma, lower jaw (i.e., thyroid tissue) and gonad were collected and the remaining tissue ('whole-fish') was retained. β-Isomer content was measured in whole-fish and in liver while estradiol (E2), 11-ketotestosterone (11-KT) and testosterone (T) were measured in plasma. Based on liver and gonad somatic indices, no apparent effects on liver or gonad development in fish from any of the treatment groups were observed. The bioaccumulation of β-isomer was similar in fish from all treatment groups with steady-state occurring before the end of the uptake phase. Depuration of the β-isomer from fish obeyed first order kinetics and there were no statistically significant differences in the depuration half life (t(1/2)) among the treatment groups: 22.5 ± 10.4 (low), 13.5 ± 5.9 (med) and 13.8 ± 2.2 (high) days. Steady-state biomagnification factors were much smaller than 1 for fish in all treatment groups. Debrominated metabolites were not detected in composite liver or whole-fish extracts and there was no evidence of isomerization of the β-isomer to other isoforms in vivo. While there were occasional differences among treatment groups in circulating plasma E2, T and 11-KT levels there was no clear, temporal trend or dose-response.

Evaluation of Kawasaki Disease Risk-scoring Systems for Intravenous Immunoglobulin Resistance

To assess the performance of 3 risk scores from Japan that were developed to predict, in children with Kawasaki disease, resistance to intravenous immunoglobulin (IVIG) treatment.

Clinical Research Careers: Reports from a NHLBI Pediatric Heart Network Clinical Research Skills Development Conference

Wyman W. Lai, MD, MPH, and Victoria L. Vetter, MD, MPH. The Pediatric Heart Network (PHN), funded under the U.S. National Institutes of Health-National Heart, Lung, and Blood Institute (NIH-NHLBI), includes two Clinical Research Skills Development (CRSD) Cores, which were awarded to The Children's Hospital of Philadelphia and to the Morgan Stanley Children's Hospital of New York-Presbyterian. To provide information on how to develop a clinical research career to a larger number of potential young investigators in pediatric cardiology, the directors of these two CRSD Cores jointly organized a one-day seminar for fellows and junior faculty from all of the PHN Core sites. The participants included faculty members from the PHN and the NHLBI. The day-long seminar was held on April 29, 2009, at the NHLBI site, immediately preceding the PHN Steering Committee meeting in Bethesda, MD.

Surgical Management of Complete Atrioventricular Septal Defect: Associations with Surgical Technique, Age, and Trisomy 21

We sought to evaluate the contemporary results after repair of a complete atrioventricular septal defect and to determine the factors associated with suboptimal outcomes.

Dechloranes 602, 603, 604, Dechlorane Plus, and Chlordene Plus, a Newly Detected Analogue, in Tributary Sediments of the Laurentian Great Lakes

A chlorinated compound (Chlordene Plus, CP), structurally related to Dechloranes (Dec) 602, 603, 604, and Dechlorane Plus (DP), was identified, and concentrations and spatial trends of Dec 602, 603, 604, CP, and DP in tributary sediments of the Laurentian Great Lakes are reported. The dechloranes were widely detected with their concentrations varying considerably across the Great Lakes basin. Spatial trends of Dec 602, 604, and DP in Canadian tributary sediments were similar to that of BDE 209, which suggested these flame retardant chemicals in tributaries were associated with industrial and urban areas. The highest concentrations of Dec 602, 604, and DP observed in tributaries of the Niagara River confirmed that past or ongoing manufacturing of these compounds at plants along the river were important sources to Lake Ontario. Dec 603 was detected in technical products of aldrin and dieldrin, and its spatial trend was consistent with historic pesticide usage. Similarly, CP was detected in technical products of chlordene and chlordane, and it was found in higher concentrations in sediments near urban areas, possibly related to past chlordane use in home termite control.

Remote Preconditioning Improves Maximal Performance in Highly Trained Athletes

Remote ischemic preconditioning (RIPC) induced by transient limb ischemia releases a dialysable circulating protective factor that reduces ischemia-reperfusion injury. Exercise performance in highly trained athletes is limited by tissue hypoxemia and acidosis, which may therefore represent a type of ischemia-reperfusion stress modifiable by RIPC.

Is BDE-175 an Important Enough Component of Commercial Octabromodiphenyl Ether Mixtures to Be Listed in Annex A of the Stockholm Convention?

Commercial octabromodiphenyl ether mixtures, containing hexabromodiphenyl ethers and heptabromodiphenyl ethers were listed in Annex A of the Stockholm Convention on May 2009 (Fourth Conference of the Parties) (UNEP, 2009a). Four compounds are specifically mentioned: 2,2',4,4',5,5'-hexabromodiphenyl ether (BDE-153), 2,2',4,4',5,6'-hexabromodiphenyl ether (BDE-154), 2,2',3,3',4,5',6-heptabromodiphenyl ether (BDE-175), and 2,2',3,4,4',5',6-heptabromodiphenyl ether (BDE-183). Presumably they were identified as key components of commercial mixtures and found to be present in environmental samples. However, since BDE-175 and BDE-183 co-elute on common HRGC columns, the presence of BDE-175 as an important component in technical octa-BDE mixtures has not been illustrated. The successful HRGC/LRMS separation of a 1:1 mixture of BDE-175 and BDE-183, as well as (1)H NMR analysis of technical material, has allowed us to confirm that this congener is not present in technical products (e.g. Great Lakes DE-79â„¢) in quantifiable amounts.

Assessment of Myocardial Deformation in Children Using Digital Imaging and Communications in Medicine (DICOM) Data and Vendor Independent Speckle Tracking Software

Analysis of myocardial deformation from data stored in Digital Imaging and Communications in Medicine format using vendor-independent software may be useful for clinical and research purposes but has not been evaluated in children.

Hyperglycemia After Pediatric Cardiac Surgery: Impact of Age and Residual Lesions

We evaluated the effect of patient age and significant residual cardiac lesions on the association between hyperglycemia and adverse outcomes in children after cardiac surgery. The incidence, severity, and duration of hyperglycemia in this patient population and perioperative factors predisposing to hyperglycemia were also delineated.

Non-high-density Lipoprotein Cholesterol Concentration is Associated with the Metabolic Syndrome Among US Youth Aged 12-19 Years

To test the hypothesis that the concentration of non-high-density lipoprotein cholesterol (non-HDL-C) is associated with the metabolic syndrome (MetS) in youth.

Long-term Results of Aortic Root Repair Using the Reimplantation Technique

OBJECTIVES: Aortic valve sparing is frequently performed to treat patients with aortic root aneurysm, but there is an inadequate amount of information regarding its long-term durability. This study examines the long-term results of reimplantation of the aortic valve in patients with aortic root aneurysms. METHODS: From August 1989 to December 2010, 296 consecutive patients had reimplantation of the aortic valve into a tubular Dacron graft. Their mean age was 45 years (range, 11-79 years), and 78% were men. Of the patients, 36% had Marfan syndrome and 11% had bicuspid aortic valve. Patients were followed prospectively with periodic images of the aortic root and remaining aorta. The mean follow-up was 6.9 ± 4.5 years. There were 21 patients at risk at 15 years. RESULTS: There were 4 operative and 18 late deaths. The survival at 5, 10, and 15 years was 95.1% ± 3.5%, 93.1% ± 4.4%, and 76.5% ± 18%, respectively. Only 3 patients required reoperation on the aortic valve; all 3 patients had the Bentall procedure. Freedom from reoperation at 5, 10, and 15 years was 99.7% ± 2.0%, 97.8% ± 5.3%, and 97.8% ± 5.3%, respectively. During follow-up, moderate aortic insufficiency developed in 9 patients, and severe aortic insufficiency developed in 2 patients. Freedom from moderate or severe aortic insufficiency at 5, 10, and 15 years was 98.3% ± 3.5%, 92.9% ± 6.5%, and 89.4% ± 12%, respectively. CONCLUSIONS: The function of the aortic valve implanted inside a tubular Dacron graft remains normal at 15 years in most patients after this type of aortic valve-sparing operation.

Factors Associated With Thrombotic Complications After the Fontan Procedure: A Secondary Analysis of a Multicenter, Randomized Trial of Primary Thromboprophylaxis for 2 Years After the Fontan Procedure

OBJECTIVES: The study sought to identify factors associated with increased risk of thrombosis after Fontan. BACKGROUND: The Fontan procedure is the culmination of staged palliation for patients with univentricular physiology. Thrombosis is an important complication after this procedure. METHODS: An international multicenter randomized controlled trial of acetylsalicylic acid versus warfarin for thromboprophylaxis after the Fontan procedure was conducted in 111 patients, and did not show a significant difference regarding thrombotic complications. We performed a secondary analysis of this previously published manuscript to identify factors associated with thrombosis in this population. Standardized prospective data collection included independent adjudication of all events. RESULTS: At 2.5 years after randomization, time-related freedom from thrombosis was 69% (all venous, no arterial events), with 28% of thrombosis presenting with clinical signs or events. Hazard of thrombosis was highest immediately after Fontan with a gradual increase in risk during late follow-up. In multivariable models, factors associated with higher risk of thrombosis were pulmonary atresia with intact ventricular septum (hazard ratio [HR]: 3.64, 95% confidence interval [CI]: 1.04 to 12.70, p = 0.04), pulmonary artery distortion (HR: 2.35, 95% CI: 0.96 to 5.73, p = 0.06), higher pre-operative unconjugated bilirubin (HR: 1.19 μmol/l, 95% CI: 1.01 to 1.39, p = 0.04), use of central venous lines for >10 days or until hospital discharge (HR: 17.8, 95% CI: 3.97 to 79.30, p < 0.001), and lower FiO(2) 24 h after the procedure (HR: 1.49/10%, 95% CI: 1.00 to 2.22, p = 0.06). Patients on warfarin who consistently achieved minimum target international normalized ratio levels or those on acetylsalicylic acid had a decrease in risk of thrombosis compared with patients who often failed to meet target international normalized ratio level (HR: 3.53, 95% CI: 1.35 to 9.20, p = 0.01). CONCLUSIONS: More favorable thromboprophylaxis strategies are needed in light of the difficulties in controlling warfarin therapy and the high prevalence of thrombosis in this population (International Multi Centre Randomized Clinical Trial of Anticoagulation in Children Following Fontan Procedures; NCT00182104).

Physical Activity Interacts with Adiposity in Determining Cardiometabolic Risk in Adolescents

Background: Moderate-to-vigorous physical activity (MVPA) has been negatively associated with cardiometabolic risk. We sought to determine if MVPA interacts with body-mass index (BMI) and waist circumference (WC) in determining cardiometabolic risk in adolescents. Methods: This cross-sectional study included cardiometabolic risk (blood pressure [BP], nonfasting lipids) screening and a 7-day recall physical activity questionnaire in 4,104 adolescents (51% male; mean age: 14.6 ± 0.5 years old). WC- and BMI- percentiles were used to define anthropometric categories (including obese adolescents: 90th WC, 85th BMI). Results: Obesity in adolescents was associated with lower levels of high-density lipoprotein (HDL cholesterol (Estimate [EST]: -0.28(0.07) mmol/L, p < .001) and higher non-HDL cholesterol (EST: +0.38(0.14) mmol/L, p = .008). Each additional day with 20 min of MVPA was associated with lower non-HDL cholesterol (EST: -0.014(0.005) mmol/L/days/week, p = .003), independent of anthropometric category. Each additional day with 20 min of MVPA was associated with an increased odds ratio (OR) for higher BP category in obese adolescents (OR: 1.055, 95% CI: 1.028-1.084, p < .001) and a lower odds ratio for higher BP category in presumably-muscular adolescents (OR: 0.968, 95% CI: 0.934-0.989, p = .005). Conclusions: An increase in MVPA was associated with an increased likelihood for higher BP category in obese adolescents. The dose-response relationship between physical activity and cardiometabolic risk needs to be evaluated in adolescents of varying anthropometry categories.

Enhanced Physiology for Submaximal Exercise in Children After the Fontan Procedure

PURPOSE: After the Fontan procedure, children exhibit reduced peak exercise capacity; yet, their sub-maximal exercise response remains unclear. This study sought to determine the relationship between sub-maximal and peak exercise capacity, and physical activity in Fontan patients. METHODS: This cross-sectional study recruited 50 Fontan patients (59% males) with a median age of 9 years (range: 6-12). The median age at Fontan procedure was 2.9 years (range: 1.6-9.1). Study assessments included medical history, exercise testing, and accelerometry. RESULTS: Significantly lower sub-maximal oxygen consumption (VO2) and heart rate (HR) in response to a standardized workload than published values for healthy children, (mean±SD) of -1.72±5.24 (p<0.001) and -1.45±1.98 (p<0.001), respectively, suggest enhanced sub-maximal work efficiency in this group of patients after Fontan. Higher sub-maximal VO2 z-score was associated with higher sub-maximal HR z-Score (p=0.02) and lower body mass index z-score (p=0.01). Higher peakVO2 was associated with higher sub-maximal VO2 z-score (p<0.01), male sex (p=0.03), higher RER (p=0.02), lower sub-maximal HR z-score (p<0.01), and higher chronotropic responsiveness (p<0.0001). Exercise test duration z-score was associated with lower sub-maximal HR z-score (p=0.02) and higher chronotropic responsiveness (p=0.02). CONCLUSIONS: Fontan patients exhibit a lower sub-maximal VO2 and HR responsiveness at a given workload than healthy controls during standardized exercise testing; Thus, they may be better adapted to perform sub-maximal exercise. While peak exercise capacity is limited, Fontan patients are able to perform sub-maximal physical activities at the same level as their healthy peers.

The Fate of the Neoaortic Valve and Root After the Modified Ross-Konno Procedure

OBJECTIVES: In children with aortic valve disease associated with annular hypoplasia or complex multilevel left ventricular outflow tract obstruction, the Ross procedure, combined with a modified Konno-type aortoventriculoplasty, is advocated. We aim to examine the fate of the neoaortic apparatus and assess neoaortic valve function after the modified Ross-Konno procedure. METHODS: Forty-three patients, with a median age of 6 years, underwent the modified Ross-Konno procedure with a myectomy but without the use of a ventricular septal patch. Serial postoperative echocardiograms (n = 187) were analyzed, and regression models adjusted for repeated measures were used to model the longitudinal growth of the neoaortic annulus and root. RESULTS: There were 2 operative deaths (5%) and 1 late mortality. At 8 years, survival was 93% and freedom from autograft, homograft, and all-cause reoperation was 100%, 81%, and 72%, respectively. The median postprocedure diameter and z score were 14 mm (7-21 mm) and +1.3 (-3.0 to +6.1) for the neoaortic annulus and 21 mm (9-30 mm) and +1.6 (-1.3 to +4.1) for the neoaortic root, respectively. Serial echocardiograms showed a progressive increase in annular (+0.56 mm/year, P < .001) and root (+0.89 mm/year, P < .001) diameters but little change in annular (-0.07/year, P = .08) and root (-0.002/year, P = .96) z scores. Autograft regurgitation developed in 9 patients; however, the degree and progression of regurgitation over time were not significant (P = .22). CONCLUSIONS: After the modified Ross-Konno procedure, the neoaortic annulus and root increased in size proportionately to somatic growth. Autograft regurgitation, usually mild and stable, developed in few patients, and none required autograft reoperation. Our findings support the use of the modified Ross-Konno as the procedure of choice in children with aortic valve disease and complex left ventricular outflow tract obstruction.

Office-based Randomized Controlled Trial to Reduce Screen Time in Preschool Children

To determine if an intervention for preschool-aged children in primary care is effective in reducing screen time, meals in front of the television, and BMI.

Recommendations for Physical Activity, Recreation Sport, and Exercise Training in Paediatric Patients with Congenital Heart Disease: a Report from the Exercise, Basic & Translational Research Section of the European Association of Cardiovascular Prevention and Rehabilitation, the European Congenital Heart and Lung Exercise Group, and the Association for European Paediatric Cardiology

All children have a natural need to move, play, and perform activities. Physical activity is necessary for optimal physical, emotional, and psychosocial development for healthy children as well as children with congenital heart disease (CHD). In this paper we provide recommendations for physical activity, recreational sport, and exercise training in children and adolescents with CHD. In general, children with CHD should be advised to comply with public health recommendations of daily participation in 60 min or more of moderate-to-vigorous physical activity that is developmentally appropriate and enjoyable and involves a variety of activities. While all patients with CHD can participate and benefit from physical activity and exercise, those with specific lesions or complications may require counselling regarding precautions and recommendations.

Effects of an Acute, Outpatient Physiotherapy Exercise Program Following Pediatric Heart or Lung Transplantation

This prospective interventional study investigated the impact of a three-month, ambulatory HA or HB, semi-individualized, PT-prescribed exercise program following pediatric HTx or LTx. SMW distance, strength, and flexibility were assessed at start and completion of the program and one yr after enrollment. Subjects received either an HB or HA exercise program three times per week. The cohort demonstrated clinically and statistically significant improvements in SMW distances at three months (425.7 ± 109.4-500.6 ± 93.6 m, p < 0.001) and at one yr (528.5 ± 66.6 m, p = 0.001), although there was no difference between the two groups at any time. Similar improvements were also observed in strength and flexibility measures. Correlates with higher SMW distance at three months and one yr included older age, male gender, and underlying diagnosis other than CHD. Male gender and diagnosis other than CHD were associated with a slower improvement in the SMW distance. This is the first report of institutionally based, outpatient exercise rehabilitation in the recovery following pediatric thoracic transplantation. We found similar improvements to HB interventions up to one yr after surgery. Further study of the role of exercise rehabilitation and long-term fitness outcomes is needed.

Sleep Disturbance and Cardiovascular Risk in Adolescents

Evidence suggests that inadequate or disturbed sleep is associated with increased cardiovascular risk in adults. There are limited data on sleep quality and associated cardiovascular risk in children.

Predictive Value of Bronchoscopy After Infant Cardiac Surgery: a Prospective Study

Airway evaluation following infant cardiac surgery often reveals evidence of tracheobronchial narrowing. We studied the association between airway narrowing and extubation failure (EF) in this population.

Genetic Variations in Hypoxia Response Genes Influence Hypertrophic Cardiomyopathy Phenotype

Background:Risk factors for diastolic dysfunction in hypertrophic cardiomyopathy (HCM) are poorly understood. We investigated the association of variants in hypoxia-response genes with phenotype severity in pediatric HCM.Methods:A total of 80 unrelated patients <21 y and 14 related members from eight families with HCM were genotyped for six variants associated with vascular endothelial growth factor A (VEGFA) downregulation, or hypoxia-inducible factor A (HIF1A) upregulation. Associations between risk genotypes and left-ventricular (LV) hypertrophy, LV dysfunction, and freedom from myectomy were assessed. Tissue expression was measured in myocardial samples from 17 patients with HCM and 20 patients without HCM.Results:Age at enrollment was 9 ± 5 y (follow-up, 3.1 ± 3.6 y). Risk allele frequency was 67% VEGFA and 92% HIF1A. Risk genotypes were associated with younger age at diagnosis (P < 0.001), septal hypertrophy (P < 0.01), prolonged E-wave deceleration time (EWDT) (P < 0.0001) and isovolumic relaxation time (IVRT) (P < 0.0001), and lower freedom from myectomy (P < 0.05). These associations were seen in sporadic and familial HCM independent of the disease-causing mutation. Risk genotypes were associated with higher myocardial HIF1A and transforming growth factor B1 (TGFB1) expression and increased endothelial-fibroblast transformation (P < 0.05).Conclusion:HIF1A-upregulation and/or VEGFA-downregulation genotypes were associated with more severe septal hypertrophy and diastolic dysfunction and may provide genetic markers to improve risk prediction in HCM.

The Study of Antiarrhythmic Medications in Infancy (SAMIS): a Multicenter, Randomized Controlled Trial Comparing the Efficacy and Safety of Digoxin Versus Propranolol for Prophylaxis of Supraventricular Tachycardia in Infants

Supraventricular tachycardia (SVT) is one of the most common conditions requiring emergent cardiac care in children, yet its management has never been subjected to a randomized controlled clinical trial. The purpose of this study was to compare the efficacy and safety of the 2 most commonly used medications for antiarrhythmic prophylaxis of SVT in infants: digoxin and propranolol.

Superior Cavopulmonary Anastomosis Timing and Outcomes in Infants with Single Ventricle

OBJECTIVES: We sought to identify factors associated with the timing and surgical outcomes of the superior cavopulmonary anastomosis. METHODS: The Pediatric Heart Network's Infant Single Ventricle trial database identified participants who underwent superior cavopulmonary anastomosis. Factors potentially associated with age at superior cavopulmonary anastomosis, length of stay and death by 14 months of age were evaluated. Factors included subject demographics, cardiac anatomy, measures from neonatal hospitalization and pre-superior cavopulmonary anastomosis visit, adverse events, echocardiographic variables, intraoperative variables, superior cavopulmonary anastomosis type, and number of concurrent cardiac surgical procedures. Age at superior cavopulmonary anastomosis was analyzed using Cox proportional hazards regression. Natural log length of stay was analyzed by multiple linear regression. RESULTS: Superior cavopulmonary anastomosis was performed in 193 subjects at 5.2 months of age (interquartile range, 4.2, 6.2) and weight of 5.9 kg (interquartile range, 5.3, 6.6). The median length of stay was 7 days (interquartile range, 6, 10). There were 3 deaths and 1 transplant during the superior cavopulmonary anastomosis hospitalization, and 3 deaths and 3 transplants between discharge and 14 months of age. Age at superior cavopulmonary anastomosis was associated with center and interstage adverse events. A longer length of stay was associated with younger age and greater case complexity. Superior cavopulmonary anastomosis type, valve regurgitation, ventricular ejection fraction, and ventricular end-diastolic pressure were not independently associated with age at superior cavopulmonary anastomosis or the length of stay. CONCLUSIONS: Greater case complexity and more frequent interstage adverse events are associated with an earlier age at superior cavopulmonary anastomosis. Significant variation in age at superior cavopulmonary anastomosis among centers, independent of subject factors, highlights a lack of consensus regarding the optimal timing. Factors associated with length of stay could offer insights for improving presuperior cavopulmonary anastomosis care and surgical outcome.

Familial Hypercholesterolemia in Children and Adolescents

This review provides an update and highlights the controversies regarding recent advances and recommendations regarding screening, diagnosis and treatment of children and adolescents with familial hypercholesterolemia.

Newly Discovered Methoxylated Polybrominated Diphenoxybenzenes Have Been Contaminants in the Great Lakes Herring Gull Eggs for Thirty Years

We recently reported the discovery and identification of novel methoxylated polybrominated diphenoxybenzenes (MeO-PBDPBs) in herring gulls eggs from the Laurentian Great Lakes of North America. We presently investigated the temporal changes (1982-2010) in MeO-PBDPB concentrations and congener patterns, as well as chemical tracers of diet (ratios of carbon and nitrogen stable isotopes), in egg pool homogenates from five selected colony sites across the Great Lakes. Egg pool homogenates from the Channel-Shelter (C-S) Island (Lake Huron) contained ∑MeO-PBDPB concentrations orders of magnitude greater than those from other colonies, suggesting potential point contamination sources nearby. In the C-S Island egg pools, concentrations increased from the initial study year (31 ng/g wet weight) and peaked around the late 1990s, followed by a general decline until 2010. Over the period, concentrations generally increased in eggs from Fighting Island (Lake Erie), Toronto Harbour (Lake Ontario) and Big Sister Island (Lake Michigan) colonies, whereas the levels in Agawa Rock (Lake Superior) declined. Although other factors likely exist, changes over time in the carbon and nitrogen isotope tracers reflected a shift of the gull diet from aquatic to more terrestrial origins, and suggested this diet shift partially accounted for the temporal changes of ∑MeO-PBDPB levels in eggs from most colonies. The ratio of Br(6)- to Br(5)-MeO-PBDPB congeners generally decreased over time in the colonies at Channel-Shelter Island, Fighting Island and Agawa Rock. This suggested that Br(5)- versus Br(6)-MeO-PBDPB congeners and/or possibly their nonmethoxylated and higher brominated precursors may have been more abundant in diets of terrestrial origin. Notably, these MeO-PBDPB congeners are not "emerging" brominated substances, but rather "recently discovered" contaminants since, as of 2011, ∑MeO-PBDPB concentrations have been constantly in the range of 30-100 ng/g ww for at least the last 30 years.

A Predictive Model for Neurodevelopmental Outcome After the Norwood Procedure

Neurodevelopmental outcomes after the Norwood procedure for single right ventricular lesions are worse than those in the normal population. It would be valuable to identify which patients at the time of Norwood discharge are at greatest risk for neurodevelopmental impairment later in childhood. As such, this study sought to construct and validate a model to predict poor neurodevelopmental outcome using variables readily available to the clinician. Using data from the 14 month neurodevelopmental outcome of the Single-Ventricle Reconstruction (SVR) trial, a classification and regression tree (CART) analysis model was developed to predict severe neurodevelopmental impairment, defined as a Psychomotor Development Index (PDI) score lower than 70 on the Bayley Scales of Infant Development-II. The model then was validated using data from subjects enrolled in the Infant Single Ventricle (ISV) trial. The PDI scores were lower than 70 for 138 (44 %) of 313 subjects. Predictors of a PDI lower than 70 were post-Norwood intensive care unit (ICU) stay longer than 46 days, genetic syndrome or other anomalies, birth weight less than 2.7 kg, additional cardiac surgical procedures, and use of five or more medications at hospital discharge. Using these risk factors, the CART model correctly identified 75 % of SVR subjects with a PDI lower than 70. When the CART model was applied to 70 subjects from the ISV trial, the correct classification rate was 67 %. This model of variables from the Norwood hospitalization can help to identify infants at risk for neurodevelopmental impairment. However, given the overall high prevalence of neurodevelopmental impairment and the fact that nearly one third of severely affected children would not have been identified by these risk factors, close surveillance and assessment for early intervention services are warranted for all infants after the Norwood procedure.

Stroke Recurrence in Children with Congenital Heart Disease

Pediatric arterial ischemic stroke (AIS) carries an important morbidity and mortality burden. Congenital heart disease (CHD) is among the most important risk factors for pediatric AIS. Data on stroke recurrence in childhood CHD are lacking, resulting in uncertainty regarding optimal strategies for preventing recurrence.

Guidelines for Lipid Screening in Children and Adolescents: Bringing Evidence to the Debate

Cardiac Performance and Quality of Life in Patients Who Have Undergone the Fontan Procedure with and Without Prior Superior Cavopulmonary Connection

BACKGROUND: A superior cavopulmonary connection is commonly performed before the Fontan procedure in patients with a functionally univentricular heart. Data are limited regarding associations between a prior superior cavopulmonary connection and functional and ventricular performance late after the Fontan procedure. METHODS: We compared characteristics of those with and without prior superior cavopulmonary connection among 546 subjects enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. We further compared different superior cavopulmonary connection techniques: bidirectional cavopulmonary anastomosis (n equals 229), bilateral bidirectional cavopulmonary anastomosis (n equals 39), and hemi-Fontan (n equals 114). RESULTS: A prior superior cavopulmonary connection was performed in 408 subjects (75%); the proportion differed by year of Fontan surgery and centre (p-value less than 0.0001 for each). The average age at Fontan was similar, 3.5 years in those with superior cavopulmonary connection versus 3.2 years in those without (p-value equals 0.4). The type of superior cavopulmonary connection varied by site (p-value less than 0.001) and was related to the type of Fontan procedure. Exercise performance, echocardiographic variables, and predominant rhythm did not differ by superior cavopulmonary connection status or among superior cavopulmonary connection types. Using a test of interaction, findings did not vary according to an underlying diagnosis of hypoplastic left heart syndrome. CONCLUSIONS: After controlling for subject and era factors, most long-term outcomes in subjects with a prior superior cavopulmonary connection did not differ substantially from those without this procedure. The type of superior cavopulmonary connection varied significantly by centre, but late outcomes were similar.

Genetic Determinants of Right-ventricular Remodeling After Tetralogy of Fallot Repair

Hypoxia-inducible factor (HIF1A) regulates the myocardial response to hypoxia and hemodynamic load. We investigated the association of HIF1A variants with right-ventricular (RV) remodeling after tetralogy of Fallot (TOF) repair.

Identification and Determination of the Dechlorination Products of Dechlorane 602 in Great Lakes Fish and Arctic Beluga Whales by Gas Chromatography-high Resolution Mass Spectrometry

During the course of our studies of in-use chlorinated flame retardants, such as Dechlorane Plus(®) and Dechloranes 602 and 604, blubber of beluga whales from the Canadian Arctic and lake trout and whitefish from the North American Great Lakes were found to contain two novel dechlorination products of Dechlorane 602 (Dec602). The structures of these compounds were characterized by experiments performed using both gas chromatography-high resolution mass spectrometry and Fourier transform mass spectrometry with a prepared technical mixture of monohydro and dihydroDec602 derivatives. These Dec602 derivatives are analogous to the well-known monohydro and dihydro photochemical degradation products of Mirex. The ratio of the two monohydroDec602 diastereomers varied between Lake Ontario fish and those from the upper lakes, but only one isomer was found in Arctic beluga, indicating that one isomer is either more stable or more bioaccumulative. Dechlorane Plus(®), Dec603, and Dec 604 were not detected in Arctic beluga, but Dec602 and its monohydroDec602 derivative were measured in approximately equal concentrations, ranging from 25 to 300 pg/g lipid. In Great Lakes fish, concentrations of the monohydroDec602 derivatives were also close to those of Dec602, ranging from 2 to 67 ng/g lipid and were greatest in Lake Ontario. This study reports on the first measurements of dechlorane-related compounds in Arctic biota and the first detection of monohydroDec602 degradation products and their accumulation in biota.

Paediatric Obesity Research in Early Childhood and the Primary Care Setting: the TARGet Kids! Research Network

Primary paediatric health care is the foundation for preventative child health. In light of the recent obesity epidemic, paediatricians find themselves at the frontline of identification and management of childhood obesity. However, it is well recognized that evidence based approaches to obesity prevention and subsequent translation of this evidence into practice are critically needed. This paper explores the role of primary care in obesity prevention and introduces a novel application and development of a primary care research network in Canada--TARGet Kids!--to develop and translate an evidence-base on effective screening and prevention of childhood obesity.

Rate, Associated Factors and Outcomes of Recurrence of Kawasaki Disease in Ontario, Canada

Previous studies on recurrence of Kawasaki disease (KD) have mostly been limited to Japan, which has an incidence of KD 8-10-fold higher than North America. The aim of the present study was to determine the rate of KD recurrence for patients in Ontario, to identify factors potentially associated with increased odds of recurrence, and to compare the clinical course and outcomes of index and recurrent KD episodes.

Trends in Endocarditis Hospitalizations at US Children's Hospitals: Impact of the 2007 American Heart Association Antibiotic Prophylaxis Guidelines

In 2007, the American Heart Association recommended cessation of antibiotic prophylaxis for infective endocarditis (IE) before dental procedures for all but those at highest risk for adverse outcomes from IE. The impact of these guidelines is unclear. We evaluated IE hospitalizations at US children's hospitals during this period.

Factors Associated with Low Physical Activity Levels Following Pediatric Cardiac Transplantation

Banks L, Dipchand AI, Manlhiot C, Millar K, McCrindle BW. Factors associated with low physical activity levels following pediatric cardiac transplantation. Abstract:  Background: Objectively measured MVPA levels following pediatric cardiac transplantation are unknown despite physical health implications. We sought to determine factors associated with MVPA in a pediatric cohort who had undergone cardiac transplantation. Methods: Study assessments included maximal exercise testing (VO(2) max), accelerometry, and physical activity (HAES) and functional health status (CHQ-PF50) questionnaires. Results: Participants (n = 20, 60% male, age: 11.8 ± 3.0 yr old) had a VO(2) max of 28.5 ± 6.8 mL/kg/min (%-predicted: 65 ± 14%) and maximal heart rate of 154 ± 16 beats/min (%-predicted: 73 ± 7.5%). Participants performed a median of 7.6 min/day (Q1 4.0 min/day, Q3 11.0 min/day) of MVPA. Each additional year of age at transplantation was associated with a decrease of 1.9 [1.0] min/day of MVPA (p = 0.07). Predicted VO(2) max, maximal power output, male sex, and age at study enrollment were not associated with an increase in MVPA. Parents' perception of their child's functional health status (CHQ-PF50) was lower on general health (p < 0.01) and family activity (p < 0.01) domains relative to a population-based cohort of parents reporting on healthy children. Conclusion: Pediatric cardiac transplantation recipients may be indicated to participate in cardiac rehabilitation to optimize physical activity levels.

Neurodevelopmental Outcomes After Open Heart Operations Before 3 Months of Age

The purpose of this study was to monitor developmental progress and identify predictors of developmental outcomes at 2 years after operation in infants who underwent a surgical procedure with cardiopulmonary bypass (CPB) at less than 3 months of age.

Factors Associated With Low Moderate-to-Vigorous Physical Activity Levels in Pediatric Patients With Kawasaki Disease

Background/Methods. We sought to determine functional health status and physical activity determinants in 27 patients with Kawasaki disease (KD; 20 males, 11 ± 3 years old). Patient physical activity data were compared with a population-based study of healthy children (Canadian Health Measures Survey). Results. KD patients performed less moderate-to-vigorous physical activity (MVPA) than healthy children (males, 27 vs 61 min/d, P < .001; females, 10 vs 47 min/d, P < .001). Male KD patients performed more MVPA than female KD patients (median = 27; quartiles [Q1 15, Q3 26] min/d vs 10 [Q1 7, Q3 11] min/day, P = .009). Lower MVPA in KD patients was significantly associated with female gender; lower child self-efficacy score; lower Child Health Questionnaire (CHQ-PF50) scores for role functioning behavioral issues, physical functioning, and family cohesion; and higher CHQ-PF50 scores for self-esteem and family activity limitations. Conclusion. Physical activity counseling should be a focus of management for children with a history of KD.

Thrombotic Complications and Thromboprophylaxis Across All Three Stages of Single Ventricle Heart Palliation

To describe the incidence of thrombotic complications across all 3 stages of single ventricle palliation and the association between thromboprophylaxis use and thrombotic risk.

Enhanced Exercise Performance and Survival Associated with Evidence of Autonomic Reinnervation in Pediatric Heart Transplant Recipients

Following heart transplantation (HTx), loss of autonomic input to the allograft results in elevated resting heart rate (HR) and decreased chronotropic reserve. As enhanced exercise capacity and HR recovery post exercise are suggestive of reinnervation in pediatric cohorts, we used heart rate variability (HRV) analysis to assess autonomic reinnervation in pediatric HTx recipients. Pediatric patients transplanted between 1996 and 2010 and with serial 24-hour Holter recordings post-HTx were analyzed for HRV using time and frequency domain measures. Of 112 patients, 68 (57%) showed evidence of autonomic reinnervation that was not associated with age at HTx. Evidence of reinnervation was associated with a significant increase in low-frequency power spectrum (p<0.001), suggesting sympathetic reinnervation. Patients with evidence of reinnervation showed higher percent-predicted maxVO(2) on performing an exercise test (+10.2 ± 3.6%, p = 0.006) and improved HR recovery at 3 minutes (-11.4 ± 3.9 bpm, p = 0.004), but no difference in percent-predicted maximal HR. Cox hazards modeling using presumed sinus reinnervation criteria at last Holter recording as a time-dependent covariate was associated with decreased hazard of mortality and/or retransplantation (HR: 0.2, 95% CI 0.04-1.0, p = 0.05). In conclusion, a majority of pediatric HTx recipients demonstrate evidence of reinnervation that is associated with functional outcomes. Studies to assess graft reinnervation as a marker of long-term prognosis are warranted.

Foetal Echocardiographic Assessment of Borderline Small Left Ventricles Can Predict the Need for Postnatal Intervention

BACKGROUND: We sought to prospectively determine foetal echocardiographic factors associated with neonatal interventions in borderline hypoplastic left ventricles. METHODS: Foetuses were included who had a left ventricle that was 2-4 standard deviations below normal for length or diameter and had forward flow across the mitral and aortic valves. Factors associated with an intervention in the first month of life or no need for intervention were sought using univariate and multivariate logistic regression models. RESULTS: From 2005 to 2008, 47 foetuses meeting the criteria had an additional diagnosis (+foetal coarctation/+transverse arch hypoplasia): atrioventricular septal defect 7 (+2/+0), double outlet right ventricle 2 (+0/+0), Shone's complex 19 (+9/+4), and ventricular disproportion 19 (+13/+11; 4 both). There were seven pregnancies terminated, three foetal demises, and five had compassionate care. There were 32 livebirths that either had a biventricular repair (n = 20, n = 2 dead), univentricular palliation (n = 2, both alive), or no intervention (n = 9). Overall survival of livebirths to 6 months of age was 79%. Factors associated with early intervention on first foetal echocardiogram were: obstructed or retrograde arch flow (p = 0.08, odds ratio 3.3), coarctation (p = 0.05, odds ratio 11.4), and left ventricle outflow obstruction (p = 0.05, odds ratio 12.5). Neonatal factors included: Shone's diagnosis (p = 0.02, odds ratio 4.9), bicuspid aortic valve (p = 0.005, odds ratio 11.7), and larger tricuspid valve z-score (p = 0.05, odds ratio 3.6). A neonatal factor associated with no intervention was a larger mitral valve z-score (mean -3.8 versus -4.2 intervention group, p = 0.04, odds ratio 2.8).DiscussionThe need for early intervention in foetuses with borderline hypoplastic left ventricle can be predicted by foetal echocardiography.

Role of Waist Measures in Characterizing the Lipid and Blood Pressure Assessment of Adolescents Classified by Body Mass Index

To determine if the interaction of waist circumference percentile and waist to height ratio(WHtR) with body mass index (BMI) may serve to provide further risk specification in the lipid and blood pressure assessment of adolescents beyond BMI classification.

Impaired Right and Left Ventricular Diastolic Myocardial Mechanics and Filling in Asymptomatic Children and Adolescents After Repair of Tetralogy of Fallot

After tetralogy of Fallot (TOF) repair patients have right ventricular (RV) dysfunction and reduced exercise tolerance. Diastolic dysfunction may be important but is as yet poorly characterized. The early diastolic strain rate (SR) is a measure of ventricular relaxation, and may be useful to assess diastolic mechanics in TOF. We hypothesized that children after TOF repair have diastolic dysfunction and dyssynchrony by this measure, and sought to determine their relationship with pulmonary regurgitation (PR), RV enlargement, and aerobic exercise capacity.

Rate, Associated Factors and Outcomes of Recurrence of Kawasaki Disease in Ontario, Canada

Background:  Previous studies on recurrence of Kawasaki disease (KD) have mostly been limited to Japan which has an incidence of KD 8-10 fold higher than North America. We sought to determine the rate of KD recurrence for patients in Ontario, to identify factors potentially associated with increased odds of recurrence, and to compare the clinical course and outcomes of index and recurrent KD episodes. Methods:  Review of all patients with recurrence of KD identified in Ontario, Canada, from 1995 to 2006. All patients with recurrence of KD (defined as at least 3 clinical signs of KD in addition to fever >5 days), presenting >14 days after the return to baseline from the index episode were included. Results:  A total of 1,010 patients were followed for 5,786 patient-years. During this period a total of 17 recurrent episodes in 16 patients were identified at a median of 1.5 years after the initial episode (2 weeks-5 years). Rate of recurrence of KD was 2.9 episodes/1000 patient-years, which is higher than the expected annual incidence of KD in the same age group (26.2/100,000 per year). No factors associated with increased risk of recurrence were identified, perhaps due to the small number of events. Clinical course and outcomes of the index and recurrent KD episodes were similar. Conclusions:  A previous history of KD should increase the index of suspicion for future episodes of KD to allow for rapid recognition, treatment and to achieve optimal outcomes. © 2010 The Authors. Pediatrics International © 2010 Japan Pediatric Society.

Functional Health Status of Adults with Tetralogy of Fallot: Matched Comparison with Healthy Siblings

Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings.

Intermediate-term Mortality and Cardiac Transplantation in Infants with Single-ventricle Lesions: Risk Factors and Their Interaction with Shunt Type

The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt.

Biventricular Strategies for Neonatal Critical Aortic Stenosis: High Mortality Associated with Early Reintervention

To characterize the risk of reintervention after biventricular strategies to treat neonatal critical aortic stenosis, and the effect of reintervention on survival.

Impaired Left Ventricular Myocardial Mechanics and Their Relation to Pulmonary Regurgitation, Right Ventricular Enlargement and Exercise Capacity in Asymptomatic Children After Repair of Tetralogy of Fallot

Left ventricular (LV) dysfunction is common in adults late after repair of tetralogy of Fallot (TOF). The early detection of myocardial dysfunction may be important, but LV myocardial strain and dyssynchrony are not well studied in children with TOF. The objective of this study was to investigate LV strain and dyssynchrony in asymptomatic children and adolescents after contemporary repair of TOF. The hypothesis was that impaired LV myocardial mechanics are related to pulmonary regurgitation, right ventricular (RV) enlargement, and exercise capacity.

Poor Accuracy of Noninvasive Cardiac Output Monitoring Using Bioimpedance Cardiography [PhysioFlow(R)] Compared to Magnetic Resonance Imaging in Pediatric Patients

Identification of low cardiac output (CO) states in anesthesia is important because preoperative hemodynamic optimization may improve outcome in surgery. Accurate real-time CO measurement would be useful in optimizing "goal-directed" therapy. We sought to evaluate the reliability and accuracy of CO measurement using bioimpedance cardiography (PhysioFlow®, NeuMeDx, Bristol, PA) in pediatric patients with and without cardiac disease undergoing anesthesia for magnetic resonance imaging (MRI).

The Importance of Nutrition and Physical Activity for Children

Current Outcomes of the Glenn Bidirectional Cavopulmonary Connection for Single Ventricle Palliation

The Glenn bidirectional cavopulmonary connection (BCPC) is an established procedure in multistage palliation of various single ventricle (SV) anomalies. We aimed to report the current outcomes following BCPC and to examine risk factors affecting survival and progression to the next palliation stage.

Hypertrophic Cardiomyopathy in Childhood: Disease Natural History, Impact of Obstruction, and Its Influence on Survival

We investigated the natural history, outcomes of myectomy, and impact on survival of obstructive hypertrophic cardiomyopathy (HCM) in childhood.

Cross-sectional Study of Motor Development Among Children After the Fontan Procedure

OBJECTIVES: To determine the gross motor skills of school-aged children after the Fontan procedure and compare the locomotor and object control skills with normative data.Study designThis study followed a cross-sectional design. SETTING: This study was based on hospital outpatient visit, with accelerometry conducted at home.PatientsThis study included 55 patients, including 22 girls in the age group of 6-10 years, 5.1 years after Fontan.Main outcome measuresTest of Gross Motor Development - Version 2, daily activity by accelerometer, medical history review, child and parent perceptions of activity. RESULTS: Being involved in active team sports increased locomotor percentile score by 10.3 points (CI: 4.4, 16.1). Preference for weekend outdoor activities (6.9, CI: 2.0, 11.8), performing at least 30 minutes of moderate-to-vigorous physical activity daily (24.5, CI: 7.3, 41.8), and reporting that parents seldom criticise the child's physical activity (21.8, CI: 8.9, 34.8) were also associated with higher locomotor percentile scores (p < 0.01). Object control percentile scores were higher (p < 0.03) with involvement in formal instruction (5.9, CI: 1.1, 10.6) and being restricted to "activities within comfortable limits" (27.6, CI: 7.7, 47.5). Older chronological age (r = 0.28), a more complicated medical history (r = 0.36), and older age at Fontan (r = 0.28) were associated with greater skill delay (p < 0.04). CONCLUSIONS: Children after Fontan attain basic motor skills at a later age than their peers, and deficits continue for more complex skills as age increases, suggesting a need for longitudinal monitoring of gross motor skill development through the elementary school years. Future research might investigate whether a gross motor skill rehabilitation programme can provide these children with the motor skills needed to successfully participate in a physically active lifestyle with peers.

Interaction Between Myocardial and Vascular Changes in Obese Children: a Pilot Study

Changes in vascular and myocardial structure and function have been demonstrated in obese children, but limited data are available on how these changes are related. The aims of this study were to investigate vascular and myocardial changes in obese children with lipid abnormalities and to study the interactions between vascular and myocardial parameters.

Functional Health Status in Adult Survivors of Operative Repair of Tetralogy of Fallot

We aimed to determine late functional health status of the growing adult population with repaired tetralogy of Fallot (TOF). We studied all 840 patients with TOF born from 1927 through 1984 who survived to adulthood (> 18 years of age). Clinical follow-up was by chart review, telephone interview (n = 706), and echocardiographic reports (n = 339). Functional health status was assessed using Short Form-36 (SF-36) surveys (n = 396) indexed to normative data. Risk of reoperation was low (≈ 1%/year) but increased beyond age 40 years. At latest follow-up moderate or severe pulmonary regurgitation was common (54%) and right ventricular outflow tract stenosis presented in 1/3. Consequently, evidence of right ventricular dilatation and dysfunction and tricuspid regurgitation was typical. Left-sided abnormalities were also common: hypertrophy (p < 0.0001) and outflow tract dilation (p < 0.0001) with at least mild aortic regurgitation in > 50%. Cardiorespiratory symptoms were reported in 45% (palpitations 27%, dyspnea 21%, chest pain 17%). SF-36 scores were significantly below normal for 4 physical domains (p < 0.001). Decrements in physical functioning were associated particularly with older age at follow-up (p < 0.0001), associated syndromes/lesions, reoperations, ventricular dysfunction, tricuspid regurgitation, residual septal defects, and cardiorespiratory symptomatology. Echocardiographic abnormalities were more common in older patients (p < 0.0001). All 3 SF-36 domains specific to psychosocial well-being were normal. In conclusion, despite excellent survival prospects, physical compromise is common in adults with repaired TOF. Greater decrements in older patients may reflect late deterioration with advancing age or cohort effects related to historical management. Efforts to limit ventricular and outflow tract dysfunction may translate into improved late functional status.

Development of Donor-specific Isohemagglutinins Following Pediatric ABO-incompatible Heart Transplantation

Graft acceptance following pediatric ABO-incompatible heart transplantation has been associated with a deficiency of donor-specific isohemagglutinins (DSI) due to B-cell elimination. Recent observations suggest that some of these patients do produce DSI. The purpose of this study was to examine the pattern of, risk factors for development and clinical impact of DSI. All children who underwent an ABO-incompatible heart transplant (1996-2009) were included. Serial postheart transplantation DSI titers and clinical outcomes were reviewed. DSI were produced in 27% of the patients (n = 11/41). Anti-A production was significantly greater in "at risk" patients than Anti-B (39% vs. 8%; p = 0.04). Risk factors associated with the development of DSI included: older age at transplantation (HR: 1.15/month, p = 0.04), pretransplant Anti-B level ≥ 1:8 (HR: 9.61, p = 0.004) and HLA sensitization (HR: 2.80, p = 0.11). The presence of DSI did increase the risk of cellular rejection but not antibody-mediated rejection, allograft vasculopathy, graft loss or death. Although these antibodies do not result in any significant clinical consequences, their presence suggests that B-cell tolerance is not the sole mechanism of graft acceptance.

Heparin Brand is Associated with Postsurgical Outcomes in Children Undergoing Cardiac Surgery

We sought to determine whether the use of specific unfractionated heparin brands during cardiopulmonary bypass for pediatric cardiac surgery was associated with differences in postoperative outcomes, especially regarding the incidence of bleeding and thromboembolic complications.

Left Ventricular Hypoplasia: a Spectrum of Disease Involving the Left Ventricular Outflow Tract, Aortic Valve, and Aorta

"Hypoplastic left heart syndrome" is an unsatisfactory term describing lethal underdevelopment of the left ventricle (LV). It represents the more severe end of a spectrum of LV hypoplasia, mandating single-ventricle palliation or cardiac transplantation. Less severe "borderline" ventricular hypoplasia may instead allow various biventricular therapeutic strategies and better long-term outcomes. In this review, we consider factors causing and modifying the abnormal development of the LV. LV hypoplasia is typically seen in association with left ventricular outflow tract obstruction, itself part of a spectrum of related defects with common etiologies. Secondary responses to outflow obstruction are complex but involve abnormal flow dynamics and shear stresses that result in compromised and poorly orchestrated ventricular growth and development. Subsequent remodeling is likely influenced by genetic modifiers, including intrinsic myocardial growth signaling pathways, possibly including those of HAND transcription factors. In addition, during the latter stages of gestation, cardiomyocytes undergo a switch in myogenic potential and lose the ability to undergo mitosis. Ventricular hyperplasia can therefore no longer occur; remodeling is instead limited to muscular hypertrophy. Subtle differences in this switch in myogenic potential--and modulators thereof--are likely to be of clinical and therapeutic importance, especially in children with "borderline LVs" being considered for fetal interventions or post-natal biventricular repair strategies. Finally, by more clearly understanding the initiators and propagators of abnormal ventricular development, we can hope to lean away from grouping a heterogeneous group of infants together under the unsatisfactory term "hypoplastic left heart syndrome."

Longer Blood Storage is Associated with Suboptimal Outcomes in High-risk Pediatric Cardiac Surgery

The negative effects of long-term storage of allogeneic red blood cells (RBCs) on outcomes in adult cardiac surgery have been established, but evidence of a similar effect in pediatric cardiac surgery is limited.

Complete and Incomplete Kawasaki Disease: Two Sides of the Same Coin

We sought to determine differences in clinical presentation, treatment response and coronary artery outcomes between complete and incomplete KD. All patients treated for KD between January 1990 and April 2007 were reviewed. Patients were classified as having an incomplete presentation if presenting with fever ≥ 5 days and <4 "classic" KD clinical signs. A total of 955 patients were included. Incomplete clinical presentation was seen in 217 cases (23%). Patient's demographic, clinical and laboratory characteristics were similar between groups with few exceptions. Patients presenting with incomplete KD had a longer median interval from symptom onset to diagnosis (7 [4-17] versus 6 [6-13] days, p < 0.001) and were less likely to be treated with intravenous immunoglobulin (86% versus 96%, p < 0.001). No significant difference between groups were seen in regard to coronary artery abnormalities (incomplete 13% versus complete 11%, p = 0.58). CONCLUSION: Complete and incomplete KD appear to be different sides of the same coin, differing only in the number of signs and symptoms at presentation. Similar laboratory findings and coronary artery outcomes between the two groups support this conclusion.

Feasibility of Improving the Motor Development of Toddlers with Congenital Heart Defects Using a Home-based Intervention

This study investigated the feasibility of administering motor assessments, delivering rehabilitation via parent-led activities, and enhancing motor function in children with complex congenital heart defects. Gross and fine motor development were evaluated in 20 toddlers ages 12 to 26 months after either a superior cavopulmonary connection (SCPC) procedure or an arterial switch operation (ASO) using the Peabody developmental scale, version 2 (PDMS-2). Feasibility of assessment and program delivery were examined using open-ended interviews with parents. The ASO group scored consistently higher than the SCPC group in every subscore of the PDMS-2 (ASO gross motor quotient, 96.78 ± 7.396 vs SCPC gross motor quotient, 77.56 ± 7.715 [P < 0.001]; ASO fine motor quotient, 101.20 ± 6.512 versus SCPC fine motor quotient, 87.70 ± 9.945 [P = 0.002]; ASO total motor quotient, 98.78 ± 6.515 versus SCPC total motor quotient, 79.56 ± 8.095 [P < 0.001]). A lower total motor quotient was associated with the use of anticoagulant medication (-20.3 ± 4.6; P < 0.001), longer and more frequent hospital stays (respectively, -3.6 ± 1 .4; P = 0.01 and -0.8 ± 0.4; P = 0.02), and shorter times between the most recent surgery and the assessment date (2.1 ± 0.5; P < 0.001). Age-standardized scores were constant between baseline and follow-up evaluation (baseline gross motor quotient, 87 ± 12 vs. post-intervention gross motor quotient, 88 ± 15 [P = 0.89]; baseline fine motor quotient, 94 ± 11 vs. post-intervention fine motor quotient, 94 ± 12 [P = 0.55]; baseline total motor quotient, 89 ± 12 vs. post-intervention total motor quotient, 90 ± 14 [P = 0.89]), indicating achievement of the expected rate of development. The most common barrier to home activity completion was illness in the SCPC group and lack of interest in the ASO group. Providing enjoyable activities and incorporating the activities into the participants' schedules were keys to compliance. All the children were able to complete the assessments, and the parents reported a positive impact of the intervention on family life. Children who have had the SCPC procedure experience significant motor delays early in life. However, toddlers after ASO have age-appropriate motor skills. Completion of the rehabilitation program enables post-SCPC children to increase their rate of development to age-appropriate norms.

Challenges in Echocardiographic Assessment of Mitral Regurgitation in Children After Repair of Atrioventricular Septal Defect

The validity and reproducibility of echocardiographic methods used to quantify mitral regurgitation (MR) in children with congenital heart disease are unknown. We evaluated the usefulness of methods used to quantify MR in children enrolled in a multicenter trial of enalapril 6 months after surgical repair of an atrioventricular septal defect (AVSD). MR severity in this trial was assessed using body surface area (BSA)-adjusted vena contracta lateral (i-VCW(lat)) and anterior-posterior (i-VCW(ap)) dimensions and cross-sectional area (i-VCA), regurgitant volume/BSA, regurgitant fraction, and qualitative MR grade. For each method, association with left ventricular end-diastolic volume (LVEDVz) and end-diastolic dimension (LVEDDz) z-scores and interobserver agreement were assessed. In 149 children (median age 1 year), i-VCW(lat), i-VCW(ap), and i-VCA were best associated with LVEDVz (r (2) = 0.54, r (2) = 0.24, and r (2) = 0.46, respectively; p < 0.001 for all) and showed the highest interobserver agreement (intraclass correlation coefficient = 0.62, 0.73, and 0.68, respectively). Qualitative MR grade was also associated with LVEDVz (r (2) = 0.31, p < 0.001) and showed modest interobserver agreement (kappa 0.56). Regurgitant volume/BSA and regurgitant fraction were associated with LVEDVz (r (2) = 0.45 and r (2) = 0.45, p < 0.001 for both) but showed poor interobserver agreement [ICC = 0.28 (n = 91) and ICC = 0.17 (n = 76), respectively], and their values were negative in 75% of subjects. In conclusion, echocardiographic assessment of MR severity after AVSD remains challenging. Among the quantitative methods used in this trial, i-VCW and i-VCA performed the best but offered little advantage compared with qualitative MR grade. The utility of regurgitant volume and fraction was severely limited by poor interobserver agreement and frequently negative values.

Corticosteroid Administration for Patients with Coronary Artery Aneurysms After Kawasaki Disease May Be Associated with Impaired Regression

Corticosteroid administration in Kawasaki disease (KD) is controversial but accepted as treatment for patients who do not respond to initial treatment. The impact of corticosteroids on evolving coronary artery aneurysms (CAA) and future vascular remodelling is unknown.

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