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Objective Pediatric heart transplant recipients are at risk of posttransplant coronary artery disease known as cardiac allograft vasculopathy (CAV), and also may develop diastolic dysfunction. As CAV begins with a process of progressive intimal thickening, these occult diffuse changes may be detected using optical coherence tomography (OCT). We hypothesized that the development of CAV, as identified via OCT, may be a mechanism of declining ventricular function. Accordingly, the purpose of this study was to assess coronary artery intimal thickening and LV strain in children who have undergone heart transplantation. Methods In 17 children, we analyzed OCT images for coronary intima and media thickness, and cross-sectional area (CSA). We also performed speckle tracking imaging (STI) of the LV to determine longitudinal strain and strain rate, in addition to standard echocardiographic measures. Results Longitudinal diastolic strain rate was associated with maximum intima thickness (r = -.497, P = .042), intima CSA, (r = -.489, P = .047), maximum media thickness (r = -.503, P = .039), and media CSA (r = -.614, P = .009). The intima maximum thickness, intima/media, and intima/lumen ratios were associated with stroke volume index (Std. β = -0.487, P = .023 and Std. β = -0.488, P = .022, respectively). Conclusions These findings suggest coronary artery intimal thickening may be mechanistically linked to changes in ventricular function following cardiac transplantation.

BACKGROUND: Respiratory Syncytial Virus (RSV) is a leading cause of hospital admission for acute lower respiratory tract infections (LRTI) in young children. Infants with congenital heart disease (CHD) are at a higher risk of severe infection, specifically those with hemodynamically significant intra-cardiac shunts, impaired cardiac function or pulmonary hypertension. Palivizumab prevents RSV-related hospitalizations in children with higher risk indications, including CHD, however the number of doses required to achieve optimal protection remains controversial. We report the clinical outcomes of children with CHD who received an abbreviated Palivizumab 4-dose schedule, with an increased inter-dose interval in our region. OBJECTIVES: To report hospitalization outcomes in children with CHD who received an abbreviated 4-dose Palivizumab dose schedule through the centrally managed regional RSV Immunoprophylaxis Program. DESIGN/METHODS: Retrospective population-based study of children with CHD enrolled into the regional RSV Program between 2012–2016. Children received palivizumab between November and April. Eligibility was defined according to our provincial guidelines and this defined our sample size. The primary outcome was in-season hospitalizations for RSV-confirmed or potentially RSV-related (unknown) LRTI determined by cross referencing the RSV Program database and the Canadian Institute of Health Informatics (CIHI) discharge abstract database (DAD) using seven RSV-related ICD-10 diagnostic codes. Children were categorized according to CHD subtype. Analysis was by intention-to-treat. RESULTS: A total of 325 children ([mean ± SD] gestational age 37.9±6.2 weeks; birth weight 2870±871 grams) were approved to receive 406 palivizumab courses. Between November 2012 and April 2016, 89 hospitalizations occurred in CHD patients. Of this, seventeen were RSV-confirmed hospitalizations (median age 5.9 [IQR 4 to 10]months); 8 additional cases were not tested for RSV, for a maximum rate of hospitalizationsfor RSV-confirmed or unknown of 6.2 per 100 approvals (95%CI: 4.0 to 9.0%). 24/25 RSV-confirmed or potentially RSV-related hospitalizations occurred before the 4(th)Palivizumab dose. Only one RSV-confirmed hospitalization occurred (52 days) after the 4th Palivizumab dose. Clinical characteristics of children with RSV-confirmed hospitalizations (n=17) were similar to those with RSV-unrelated hospitalizations (n=40). CONCLUSION: Our data provide population-based evidence of the protection achieved by a 4-dose Palivizumab dose schedule in infants with CHD. This clinical practice yields substantial benefits to children, their families, and to the health care system, such as reduced visits, injections, and costs, compared to a standard 5-dose schedule. Our results warrant confirmation in other geographic areas.

BACKGROUND:Acetylsalicylic acid (ASA) is part of the recommended treatment of Kawasaki disease (KD). Controversies remain regarding the optimal dose of ASA to be used. We aimed to evaluate the noninferiority of ASA at an antiplatelet dose in acute KD in preventing coronary artery (CA) abnormalities.METHODS:This is a multicenter, retrospective, nonrandomized cohort study including children 0 to 10 years of age with acute KD between 2004 and 2015 from 5 institutions, of which 2 routinely use low-dose ASA (3–5 mg/kg per day) and 3 use high-dose ASA (80 mg/kg per day). Outcomes were CA abnormalities defined as a CA diameter with a z score ≥2.5. We assessed the risk difference of CA abnormalities according to ASA dose. All subjects received ASA and intravenous immunoglobulin within 10 days of fever onset.RESULTS:There were 1213 subjects included, 848 in the high-dose and 365 in the low-dose ASA group. There was no difference in the risk of CA abnormalities in the low-dose compared with the high-dose ASA group (22.2% vs 20.5%). The risk difference adjusted for potential confounders was 0.3% (95% confidence interval [CI]: −4.5% to 5.0%). The adjusted risk difference for CA abnormalities persisting at the 6-week follow-up was −1.9% (95% CI: −5.3% to 1.5%). The 95% CI of the risk difference of CA abnormalities adjusted for confounders was within the prespecified 5% margin considered to be noninferior.CONCLUSIONS:In conjunction with intravenous immunoglobulin, low-dose ASA in acute KD is not inferior to high-dose ASA for reducing the risk of CA abnormalities.

Acquired left ventricular aneurysm is extremely rare in children. This report describes an infant with acquired left ventricular aneurysm after percutaneous aortic balloon valvuloplasty for critical aortic stenosis. The potential risk factors for myocardial injury during cardiac catheterization and potential complications are discussed.

Yeung JP, Human DG, Sandor GGS, De Souza AM, Potts JE. Serial measurements of exercise performance in pediatric heart transplant patients using stress echocardiography. Pediatr Transplantation 2011: 15: 265–271. © 2011 John Wiley & Sons A/S. Abstract: Heart transplantation is an increasingly acceptable therapeutic option for children with end-stage and complex congenital heart disease. With advances in surgery, immunosuppression, and follow-up care, functional outcomes need to be evaluated. We report the results of serial exercise testing performed using stress echocardiography in a cohort of pediatric HTP. HTP (n = 7) exercised on a semi-recumbent ergometer to volitional fatigue. Echocardiography-Doppler measurements, HR, and blood pressure were taken at rest and during staged exercise. Results were compared with healthy CON (n = 12). HTP did significantly less work during exercise (940 vs. 1218 J/kg, p

Costello syndrome is characterized by constitutional mutations in the proto-oncogene HRAS, causing dysmorphic features, multiple cardiac problems, intellectual disability, and an increased risk of neoplasia. We report a male infant with dysmorphic features, born prematurely at 32 weeks, who, during his 3-month life span, had an unusually severe and ultimately fatal manifestation of hypertrophic cardiomyopathy and hyperinsulinemic hypoglycemia. Molecular studies in this patient demonstrated the uncommon Q22K mutation in the HRAS gene, diagnostic of Costello syndrome. The major autopsy findings revealed hypertrophic cardiomyopathy, congenital myopathy, and a 1.4-cm pancreatic nodule that was positive for insulin expression and morphologically identical to a focal lesion of congenital hyperinsulinism. Sequencing of KCNJ11 and ABCC8, the 2 most commonly mutated genes in focal lesion of congenital hyperinsulinism, revealed no mutations. While hyperinsulinism is a recognized feature of RASopathies, a focal proliferation of endocrine cells similar to a focal lesion of hyperinsulinism is a novel pathologic finding in Costello syndrome.

We present a case series of pediatric blunt cardiac trauma with a variety of cardiac abnormalities, occurring immediately and after the initial insult. The range of complications and importance of serial evaluations are emphasized.

To describe the clinical features, diagnosis, treatment and outcome of children with Kawasaki disease (KD) treated at a large tertiary care Canadian paediatric hospital and to try to identify correlations between clinical features and the development of coronary artery abnormalities.The charts of 176 patients diagnosed with typical, atypical or incomplete KD between 1992 and 2000 at British Columbia's Children's Hospital were reviewed.The male to female ratio was 1.8:1. The median age was 2.5 years (range two months to 14 years), with 8% nine years or older (42% Caucasian, 43% Asian). Cases occurred steadily throughout the year. One hundred two (58%) patients had typical, 18 (10%) patients had atypical and 56 (32%) patients had incomplete KD. The median time from fever onset to first intravenous immunoglobulin (IVIG) was seven days (range two to 49 days), and treatment began within 10 days of fever onset in 134 (76%) patients. All patients received one or more doses of 2 g/kg IVIG. Forty-two (24%) patients received a second dose for nonresponsiveness, of whom 10 (6%) remained nonresponsive. Eight (5%) patients received intravenous methylprednisolone. Forty-eight (27%) patients developed coronary artery abnormalities, with 10 (6%) echogenic abnormalities, 25 (14%) dilatations and 13 (7%) aneurysms (seven giant). No patient with a normal echocardiogram at four to eight weeks developed an abnormality on subsequent study. Fourteen (8%) patients had persistent abnormalities at last follow-up (median 447 days, range 62 to 3272 days): seven dilations and seven aneurysms (six giant). Five of 13 children (39%) who developed aneurysms failed to meet diagnostic criteria for typical KD, and three of those five aneurysms were present at less than one year after diagnosis. Four of eight (50%) patients receiving intravenous methyl-prednisolone for IVIG nonresponsiveness had or developed aneurysms. One patient died.Some children diagnosed with KD who fail to meet the diagnostic description develop coronary artery abnormalities. There is a need for a more accurate means of diagnosis to more appropriately use IVIG, an expensive and increasingly scarce resource. The role of corticosteroids remains unclear and a randomized controlled clinical trial to determine their role is needed.

Sixty-six patients operated on previously for coarctation of the aorta were included in the study. There were 50 children and 16 adults. Invasive and non-invasive gradients were recorded. Anatomical obstruction was evaluated by angiography in 54 patients and the numbers and size of collaterals were quantified. The closest correlation was found between Doppler-estimated gradients and invasive peak-instantaneous gradients (r = 0.87). The relation between anatomical obstruction and invasive peak-to-peak gradients, Doppler gradients and diastolic flow ere discouraging (r = 0.64, 0.54 and 0.35). Liberal indications for angiography or magnetic resonance tomography are recommended.

We describe and illustrate a rare association of hypoplastic left heart syndrome, absent hilar left pulmonary artery, and an unusual bronchopulmonary malformation. This case highlights the utility of combination imaging of echocardiography and CT angiography in diagnosing a cyanotic newborn with a chest radiograph that is suspicious for lung hypoplasia.

A child with complex congenital heart disease in 2008 is very likely to survive a series of surgical and medical interventions, and confront an array of medical and psychosocial stressors that are presently poorly understood. As approaches to medical problems change, careful assessment of those results is essential, and the initial work of the multicentre Pediatric Heart Network is a huge step in the right direction, setting the stage for proper controlled trials of therapies. Major complications, notably ventricular failure, rhythm problems and thromboembolism, will affect nearly one-quarter of survivors, necessitating further interventions. Appropriate educational and psychosocial support for these children and their families is the next challenge for all of us in the field of paediatrics. How ironic would it be to have invested so much in early survival, only to allow the child to fail in life itself.

Enzyme replacement therapy (ERT) has been increasingly used as an interim treatment in severe mucopolysaccharidosis type I (MPSI)/Hurler patients prior to hematopoietic stem cell transplantation (HSCT).We present the outcome of a patient with MPSI/Hurler after 14 months of ERT prior to HSCT.Urinary glucosaminoglycan excretion decreased by 70% after one month of ERT. Liver volume decreased by 14% of baseline after 12 months of ERT. Pre-existing thoracolumbar kyphosis progressed to thoracolumbar dislocation with complete displacement of facets after 12 months of ERT. New development of mitral valve thickening was found by echocardiography and mild hearing loss progressed to severe sensorineural hearing loss after 13 months of ERT.ERT over a period of 14 months did not prevent progression of organ manifestations in our patient. Patients should be monitored every 6 months for cardiac, skeletal and audiological involvement on ERT.

Fifteen children with aortic stenosis were examined with Echo-Doppler cardiography in order to evaluate the use of the continuity equation to calculate the aortic valve area. The results were compared to invasive estimates of valve area calculated with the Gorlin's equation. A close correlation between the two methods, r = 0.94, was found and it is concluded that even in children an accurate estimate of valve area can be expected with the use of the continuity equation.

Pulmonary arterial and intracranial calcifications are rarely found in children. A female infant, the recipient of a twin–twin transfusion syndrome was found, by ultrasound and computed tomography, to have both pulmonary arterial and intracerebral calcification. A rare condition, termed idiopathic arterial calcification of infancy, is the likely cause. This condition carries a poor prognosis and is usually fatal.

Palliation of complex congenital heart disease, requiring reconstruction of the right ventricular outflow tract (RVOT), is standard practice. Survival of the homograft is a limiting factor. We examined the role of balloon angioplasty (BAP) in prolonging conduit life. Twelve patients underwent 15 BAP procedures between February 1989 and October 1997. The median age at conduit insertion was 28 months with detection of a significant echo gradient 42 months later. Calcification of homografts, with attendant obstruction and valve dysfunction, was present in all patients. BAP was performed within 1 month of echocardiography and reduced the gradient from a median of 57 to 38 mmHg (p < 0.0005). Echocardiographic follow-up showed persistent gradients (median 68 mmHg) and 11/12 patients went on to conduit replacement after BAP. Only one patient had replacement deferred as a result of BAP. Complications requiring intervention occurred in 20% of the procedures and included bleeding and an unusual balloon fracture. Although BAP can reduce the pressure gradient across the RVOT conduit, the effect is transient and the delay of surgery is not due to improved hemodynamic function. Approximately 10% of cases will benefit from BAP alone, but given the high rate of complications, we do not recommend this procedure as a means of prolonging conduit life.

Objectives. To determine the prevalence of Kawasaki disease in older children and to evaluate its clinical presentation, time to diagnosis, and outcome in comparison with younger patients with the disease. Methodology. A retrospective analysis of all patients discharged with a diagnosis of Kawasaki disease at a pediatric tertiary care hospital over a 12-year period. Results. A total of 133 patients were included in this study; 7.5% were 9 years of age or older at the time of illness. Patients were grouped by age: infants included children age 1 to 8 years of age and children 9 years of age or older. Older children had a higher frequency of abnormal cardiovascular physical examination (50%) versus children (6%) and infants (10%). The older age group and the infants had a higher prevalence of coronary artery abnormalities and poor left ventricular function than did the 1- to 8-year-olds. Eighty percent of the older children had coronary arteries that were either dilated or aneurysmal, and 30% demonstrated left ventricular dysfunction on initial echocardiography. The number of days to diagnosis after meeting the diagnostic criteria was 5.8 ± 2.3 for infants, 5.2 ± 1.5 for older children, and 1.9 ± 0.3 for children. Older children had a complicated course of Kawasaki disease compared with younger patients. Conclusion. We found a higher prevalence of older children with Kawasaki disease at our center than has previously been reported. Older patients, as well as infants, had a higher rate of coronary artery abnormalities than did the children between 1 and 8 years of age. Older age at the time of illness or a delay in treatment may be important factors in determining cardiac involvement in Kawasaki disease.

Objectives: To report the clinical outcomes (early death, late death, and rate of reintervention) and performance of the Contegra conduit as a right ventricle outflow tract implant and to determine the risk factors for early reintervention. Methods: Forty-nine Contegra conduits were implanted between January 2002 and June 2009. Data collection was retrospective. The mean age and follow-up duration of Contegra recipients was 3.5 ± 4.6 years and 4.2 ± 2.0 years, respectively. Results: There were three deaths (two early, one late), giving a survival rate of 93.9%. The rate of conduit-related reintervention was 19.6% and was most often due to distal conduit stenosis. Age at implantation of 22 mm) remains the first choice of implant in older children. The rates of reintervention are significantly higher with a diagnosis of truncus arteriosus, age at implantation of

Kingella kingae , a HACEK (Haemophilus parainfluenzae, Aggregatibacter actinomycetemcomitans, Aggregatibacter aphrophilus, Cardiobacterium hominis, Eikenella corrodens, Kingella kingae) organism, is a common resident of the upper airway in children; it has been associated with endocarditis in children with pre-existing heart conditions. This case report describes K. kingae endocarditis leading to valvular damage in a previously healthy 18-month-old child. Our patient developed a K. kingae bacteremia that was later complicated by meningitis, septic embolic stroke, and endocarditis of the mitral valve, leading to perforation of the posterolateral leaflet. The patient was initially treated conservatively with cefotaxime but, subsequently, required a mitral valve repair with a pericardial patch and annuloplasty. This report draws attention to the need for clinicians to be aware of the potentially serious complications of K. kingae infection in young children. If K. kingae infection is suspected then therapy should be initiated promptly with a β-lactam, followed by early echocardiographic assessment. This case also highlights the lack of specific guidelines available for K. kingae endocarditis.

Thirty-one patients with ventricular septal defect were evaluated with Echo Doppler cardiography and colour flow mapping. The ventricular septal defect area was estimated by measurement of the jet diameter by colour flow mapping. The ventricular septal defect flow was calculated as the product of the cross-sectional area of the ventricular septal defect and the velocity time integral of flow through the defect. In ten patients the diameter obtained by colour flow mapping was compared with the diameter obtained at angiography, r = 0.88 (SD = 0.14 cm). The aortic flow was calculated as the product of left ventricular outflow tract cross-sectional area and the corresponding velocity time integral. QP/QS ratio was estimated as (ventricular defect flow + aortic flow)/aortic flow and compared to estimates by a radionuclide or oximetric method. A correlation of r = 0.95 (SD = 0.23) was found.

A 6-month-old infant developed a left ventricular thrombus in association with low antithrombin-III activity following the repair of a truncus arteriosus. Conservative management was associated with spontaneous resolution of the clot. The significance of this association is discussed.

DOPPLER ASSESSMENT OF AORTIC IMPEDENCE FOLLOWING ANTIFAILURE THERAPY FOR CARDIAC FAILURE IN CHILDREN. 202

The clinical and laboratory findings in 29 children with infective endocarditis over the past 10 years are reviewed retrospectively. Twenty children (70 per cent) had congenital heart disease, five (16 per cent) had rheumatic heart disease, and four (14 per cent) had no underlying cardiac abnormality prior to presentation. Twelve (41 per cent) patients had undergone cardiac surgery prior to developing endocarditis. The most common causative organisms were Staphylococcus aureus, Staphylococcus epidermidis, and viridans streptococci none of which was associated with a significantly greater mortality. There was a high case fatality rate of 35 per cent of children below the age of 6 months and children without an underlying cardiac abnormality were particularly at risk. There is a need for standardization of diagnostic criteria for infective endocarditis to facilitate accurate collaborative epidemiological investigation.

In kwashiorkor the heart is clinically and radiologically small. This study utilises echocardiography, a tool not previously used in this disease, to show that this is due to decreased muscle mass.

A trial was conducted using sequential analysis by pairs to compare the efficacy of corticosteroids and salicylates in the treatment of acute rheumatic carditis. The results show a significantly favourable effect of steroid treatment both in clinical response and in reduction of the erythrocyte sedimentation rate. In addition, patients receiving steroids usually had a shorter hospital stay. The use of steroids in acute rheumatic fever with carditis is recommended.