Carotid Intima Media Thickness and Arterial Stiffness in Children With Acute Rheumatic Fever

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) constitute important public health problems in developing countries. Inflammation is present both in the early and late stages of the diseases. Chronic inflammation is known to be associated with atherosclerosis. We hypothesize that subclinical atherosclerosis and arterial stiffness may increase due to the ongoing inflammation as well as the increased pulse pressure and left-ventricular systolic dysfunction in RHD. The purpose of the present study was to investigate carotid intima media thickness (CIMT) and carotid artery stiffness in patients with ARF. Forty patients in follow-up due to ARF in the age group of 7–16 years (disease duration 1–10 years) and 36 volunteered subjects with similar body mass index were included in the study. The subjects included in the present study were compared regarding M-mode echocardiographic parameters and CIMT as well as carotid arterial strain (CAS), carotid artery distensibility (CAD), beta stiffness index (βSI), and pressure-strain elasticity modulus (Ep) as carotid artery stiffness parameters. CIMT (0.52 ± 0.08 and 0.48 ± 0.07 mm, p = 0.01), βSI (5.29 ± 2.98 and 3.02 ± 1.30, p < 0.001), and Ep (426.53 ± 210.50 and 254.44 ± 104.69 p < 0.001) were increased, whereas CAS (0.11 ± 0.01 and 0.19 ± 0.09, p < 0.001) and CAD (10.27 ± 4.69 and 17.76 ± 14.41, p < 0.001) were decreased in patients with ARF compared with the control group. There was a positive correlation between pulse pressure and βSI (r = 0.25, p = 0.02) and Ep (r = 0.28, p = 0.01) in addition to a correlation between left atrial dilatation and CIMT (r = 0.55 p < 0.001) in patients with ARF. CIMT and carotid artery stiffness were increased in patients with ARF. Patients with ARF may have an increased risk of subclinical atherosclerosis and cardiovascular events.     

Prediction of Residual Valvular Lesions in Rheumatic Heart Disease: Role of Adhesion Molecules

Rheumatic heart disease (RHD) is a chronic condition characterized by fibrosis and scarring of the cardiac valves and damage to the heart muscle, leading to congestive heart failure and death. This prospective cohort study was conducted to investigate the possible relation between the levels of serum adhesion molecules and acute rheumatic fever (ARF) carditis, valvular insult severity, and residual valvular lesion after improvement of rheumatic activity. Serum levels of intercellular adhesion molecule 1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), and E-selectin were assayed by enzyme-linked immunoassay (ELISA) for 50 children with ARF carditis during activity and after improvement and for 50 healthy children as control subjects. After the acute attack, patients were followed up regularly to detect residual valvular lesion. The serum levels of these adhesion molecules were significantly higher in the patients than in the control group (p < 0.001). In addition, the levels of serum adhesion molecules were significantly higher in the patients with severe carditis than in the patients with mild to moderate carditis (p < 0.001). Among the severe carditis group, the level of serum adhesion molecules was significantly higher among the patients with heart failure than among the patients without heart failure (p < 0.001). Furthermore, the pretreatment serum levels of ICAM-1 and VCAM-1 were significantly higher among the patients with residual valve lesion (p = 0.002) than among those without the lesion (p < 0.001). The cutoff values were obtained for the prediction of residual valvular lesion (ICAM-1, >1,032.3 μg/ml; VCAM-1, >3,662.3 μg/ml; E-selectin, >104.8 μg/ml). Finally, by combining the three adhesion molecules in a single prediction model, the highest area under the curve (AUC) ± standard error (SE) was obtained (0.869 ± 0.052), and the positive likelihood ratio for having a residual valvular lesion was increased (17.33). Levels of serum adhesion molecules could predict residual valvular lesions in RHD patients. The authors recommend that the serum level of adhesion molecules be measured in all cases of ARF carditis.     

<Emphasis Type="Italic">IL10</Emphasis> Promoter Polymorphisms are Associated with Rheumatic Heart Disease in Saudi Arabian Patients

Rheumatic heart disease (RHD) is an inflammatory disease that develops following streptococcal infections. IL10 helps to balance immune responses to pathogens. IL10 polymorphisms have been associated with RHD, although results remain inconclusive. Our aim was to investigate the association between IL10 polymorphisms and RHD in Saudi Arabian patients. IL10 promoter polymorphisms (-1082A/G, -829C/T, and -592C/A) were genotyped in 118 RHD patients and 200 matched controls using the TaqMan allelic discrimination assay. There was a significant difference in IL10-1082 genotype frequency between patients and controls (p = 0.01). -1082G allele carriage (GG+GA vs AA) and the (-1082, -819, -592) GCC haplotype carriage were associated with an increased risk of RHD (p = 0.004, OR 2.1, 95 % CIs 1.7–3.4 and p = 0.004, OR 2, 95 % CIs 1.3–3.4, respectively). The ACC haplotype was associated with a decrease in RHD risk (p = 0.015, OR 0.6, 95 % CIs 0.4–0.9). IL10 promoter polymorphisms may play an important role in the development of RHD and provide an opportunity for therapeutic stratification.     

Acute rheumatic fever in the 1980s

Acute rheumatic fever (ARF) is an inflammatory sequela which occurs in 1-3% of children afflicted with group A beta-hemolytic streptococcal pharyngitis (strep throat). The major manifestations are carditis, migratory polyarthritis and chorea. ARF recurs with repeated strep throats and frequently leads to rheumatic heart disease (RHD), usually mitral and aortic regurgitation and mitral stenosis. ARF likely results from an abnormal host immune response with a host-antibody/streptococcal antigen production in pharyngeal tissue and subsequent cross-reaction of host antibodies with host end organs. Treatment includes eradication of the streptococcus, use of high doses of salicylates and adrenal corticosteroids, and prolonged bed rest with gradual ambulation after clinical and laboratory signs of the disease are gone. While the incidence and mortality of ARF and RHD have decreased drastically in the affluent industrialized countries of Europe, North America, and in Japan, the disease is a major health problem in the less affluent, 'developing' countries of Latin America, the Middle East, Africa, India and Southeast Asia. The major risk factors for ARF are believed to be genetic or familial, inadequate medical care and crowded conditions. The last two factors are socioeconomic but may also be influenced by ethnic cultural behavior. Genetic propensity for ARF is supported by recent evidence of a specific DR-HLA marker in the majority of people with ARF or RHD. Finally, while ARF appears to be vanishing in most areas of the country, it is still prevalent in some affluent populations and in some disadvantaged minorities.     

Obstructive Left Heart Disease in Neonates With a “Borderline” Left Ventricle: Diagnostic Challenges to Choosing the Best Outcome

In most newborns with left heart obstruction, the choice between a single-ventricle or biventricular management pathway is clear. However, in some neonates with a “borderline” left ventricle, this decision is difficult. Existing criteria do not reliably identify neonates who will have a good long-term outlook after biventricular repair (BVR). The objective of this study was prospective assessment of the outcome after BVR for newborns in whom the left ventricle (LV) was considered “borderline” by an expert group. This study was a prospective follow-up evaluation of neonates with obstructive left heart disease related to a “borderline” LV who underwent biventricular management between January 2005 and April 2011. Of 154 neonates who required intervention for left heart obstruction, 13 (7.8 %) met the echocardiographic (echo) inclusion criteria. At the first and last echo, the z-scores were respectively ?1.76 ± 1.37 and ?0.66 ± 1.47 (p = 0.013) for the mitral valve, ?1.02 ± 1.57 and ?0.23 ± 1.78 (p = 0.056) for the aortic valve, and 13.77 ± 5.8 and 20.85 ± 8.9 ml/m² (p = 0.006) for the LV end-diastolic volume. At this writing, all 12 survivors are clinically well. However, LV diastolic dysfunction and pulmonary artery hypertension was present in 5 (36 %) of 12 patients. Endocardial fibroelastosis (EFE) was detected in five patients at the last follow-up echo, but only in two patients preoperatively. Cardiac magnetic resonance imaging did not confirm EFE in any of assessed patients. The study authors could not reliably predict the outcome after BVR for neonates with left heart obstruction and a “borderline” LV. The presence of EFE with consequent diastolic dysfunction is more important than LV volume in determining the outcome. Prospective identification of EFE remains challenging.     

Genetic and Clinical Determinants Influencing Warfarin Dosing in Children With Heart Disease

Warfarin is a common anticoagulant with narrow therapeutic window and variable anticoagulation effects. Single gene polymorphisms in cytochrome P450 2C9 (CYP2C9) and vitamin K epoxide reductase (VKORC1) have been shown to impact warfarin dosing in adults. Insufficient data exists on genetic and clinical factors which influence warfarin dosing in children. Pediatric patients with heart disease who received long-term warfarin therapy were tested for VKORC1 and CYP2C9 polymorphisms. Clinical and demographic data were reviewed in those children who achieved stable therapeutic international normalized ratio (INR). Multiple linear regression modeling was used to assess relationships between stable warfarin doses and genetic or clinical variables. Fifty children were tested for VKORC1 and CYP2C9 polymorphisms; 37 patients (M 26: F 11) had complete data, achieved stable therapeutic INR, and were included in dose variability analysis. There were predominance of white race 73% and male sex 70.3%. The mean age was 9.6 years (1.8–18.6 years). The mean weight was 37.8 kg (7.7–95 kg). Fontan physiology and mechanical cardiac valves were two most common indications for chronic warfarin therapy (25/37 or 67.6%). Twelve patients (32.4%) had ≥2 indications for warfarin therapy. Three patients had documented venous or arterial clots, and 5 patients had strokes. Congenital heart disease was present in 29 patients (78.4%), including Fontan physiology (20), complex biventricular physiology (4), and congenital mitral valve disease (5). Acquired heart disease was present in 8 patients (21.6%), including Kawasaki disease with coronary aneurysms (3), acquired mitral valve disease (3), and Marfan syndrome (2). Stable warfarin dose (mg/kg/day) was strongly associated with VKORC1 polymorphism (p < 0.0001) and goal therapeutic INR (p = 0.009). Negative correlations were observed between stable warfarin dose and age, weight, height, and BSA (p = 0.04, 0.02, 0.02, and 0.02 respectively). Factors which did not influence warfarin dose included CYP2C9 polymorphism (p = 0.17), concurrent medications (p = 0.85), sex (p = 0.4), race (p = 0.14), congenital heart disease (p = 0.09), and Fontan physiology (p = 0.76). The gene-dose effect was observed in children with homozygous wild type VKORC1 CC, who required higher warfarin dose compared to those carrying heterozygous TC or homozygous TT (p = 0.028 and 0.0004 respectively). The full multiple linear regression model revealed that VKORC1 genotypes accounted for 47% of dosing variability; CYPC29 accounted for 5%. Overall, the combination of VKORC1, CYP2C9, age, and target INR accounted for 82% of dosing variability. In children with heart disease, VKORC1 genotypes, age, and target INR are important determinants influencing warfarin dosing in children with heart disease. Future warfarin dosing algorithm in children should factor both genetic and clinical factors.     

Rhythm and Conduction Analysis of Patients With Acute Rheumatic Fever

Various rhythm and conduction abnormalities can develop in acute rheumatic fever. This study investigated rhythm and conduction abnormalities in children with acute rheumatic fever using a standard 12-lead electrocardiogram and 24-h rhythm Holter recordings. This multicenter retrospective study, performed between August 2011 and March 2012, enrolled 73 consecutive children with acute rheumatic fever. Standard electrocardiography was used to measure PR and corrected QT intervals. Holter recordings were evaluated for all the patients, and 52 of the patients (71.2 %) had carditis that was either isolated or together with other major criteria. A positive correlation was detected between carditis and the mean PR interval on standard electrocardiography, but this was not significant (p > 0.05). Standard electrocardiography showed a significant positive correlation between PR and corrected QT intervals (p = 0.03; r = 0.55). Standard electrocardiography showed only three patients (4.2 %) with premature contractions, whereas 24-h electrocardiography showed 26 patients (35.6 %) with premature contractions. Carditis was positively correlated with premature contractions (p < 0.01; r = 0.57). One patient with junctional rhythm and one patient with left bundle block were detected by standard electrocardiography. Whereas some patients with carditis exhibited no arrhythmic evidence on standard electrocardiograms, complete atrioventricular block, supraventricular tachycardia, and Mobitz type 1 block were observed on 24-h Holter recordings. A positive correlation also was observed between the presence of premature contractions and serum levels of acute-phase reactants (p = 0.03; r = 0.62). These findings led to the conclusion that rhythm and conduction disorders in acute rheumatic fever are more common than previously thought.     

Pediatric RIFLE for Acute Kidney Injury Diagnosis and Prognosis for Children Undergoing Cardiac Surgery: A Single-Center Prospective Observational Study

This study evaluated the performance of the pediatric RIFLE (pRIFLE) score for acute kidney injury (AKI) diagnosis and prognosis after pediatric cardiac surgery. It was a single-center prospective observational study developed in a pediatric cardiac intensive care unit (pCICU) of a tertiary children’s hospital. The study enrolled 160 consecutive children younger than 1 year with congenital heart diseases and undergoing cardiac surgery with cardiopulmonary bypass. Of the 160 children, 50 (31 %) were neonates, and 20 (12 %) had a univentricular heart. Palliative surgery was performed for 53 patients (33 %). A diagnosis of AKI was determined for 90 patients (56 %), and 68 (42 %) of these patients achieved an “R” level of AKI severity, 17 patients (10 %) an “I” level, and 5 patients (3 %) an “F” level. Longer cross-clamp times (p = 0.045), a higher inotropic score (p = 0.02), and a higher Risk-Adjusted Classification for Congenital Heart Surgery score (p = 0.048) but not age (p = 0.27) correlated significantly with pRIFLE class severity. Patients classified with a higher pRIFLE score required a greater number of mechanical ventilation days (p = 0.03) and a longer pCICU stay (p = 0.045). Renal replacement therapy (RRT) was needed for 13 patients (8.1 %), with two patients receiving continuous hemofiltration, and 11 patients receiving peritoneal dialysis. At the start of dialysis, the distribution of RRT patients differed significantly within pRIFLE classes (p = 0.015). All deceased patients were classified as pRIFLE “I” or “F” (p = 0.0001). The findings showed that pRIFLE is easily and feasibly applied for pediatric patients with congenital heart disease. The pRIFLE classification showed that AKI incidence in pediatric cardiac surgery infants is high and associated with poorer outcomes.     

Change in Pediatric Functional Classification During Treatment and Morbidity and Mortality in Children with Pulmonary Hypertension

Despite advances in therapy, outcomes for children with pulmonary hypertension remain poor. We sought to assess the validity of a pediatric-specific functional classification system for pulmonary hypertension (PH) in a heterogeneous population of children with PH diagnosed by echocardiogram or cardiac catheterization. A single-center, retrospective study of 65 infants and children with PH was performed. Pediatric Functional Class (FC) at diagnosis, at last visit, and change in FC over time were evaluated for their association with mortality and PH-associated morbidity in univariate, time-to-event, and multivariate regression analyses. Median age at PH diagnosis was 5.3 months (0 days–12.7 years). Twenty-five children (38 %) had idiopathic PH or PH secondary to congenital heart disease, one (2 %) had left heart disease, and 39 (60 %) had PH secondary to respiratory disease. Mortality was 25 % (16/63), primarily in the first year of follow-up. FC at diagnosis was not significantly associated with survival (p = 0.22), but higher FC (more impaired) at last visit (p < 0.001) and change in FC over time (HR 2.3, 95 % confidence interval 1.3–4, p = 0.0003) were associated with mortality. Higher FC at last visit was associated with greater days of hospitalization in the intensive care unit per year (p = 0.006) and history of cardiac arrest (p = 0.012) and syncope (p = 0.02). Although pediatric FC at diagnosis was not predictive of mortality, response to therapy (as assessed by change in FC over time and FC at last visit) was associated with morbidity and mortality in this heterogeneous cohort. Multicenter prospective studies are necessary to further validate these findings.     

Efficacy and Predictors of Success of Noninvasive Ventilation for Prevention of Extubation Failure in Critically Ill Children With Heart Disease

The study aimed primarily to evaluate the efficacy of noninvasive ventilation (NIV) and to identify possible predictors for success of NIV therapy in preventing extubation failure in critically ill children with heart disease. The secondary objectives of this study were to assess the efficacy of prophylactic NIV therapy initiated immediately after tracheal extubation and to determine the characteristics, outcomes, and complications associated with NIV therapy in pediatric cardiac patients. A retrospective review examined the medical records of all children between the ages 1 day and 18 years who sustained acute respiratory failure (ARF) that required NIV in the cardiovascular intensive care unit (CVICU) at Lucile Packard Children’s Hospital between January 2008 and June 2010. Patients were assigned to a prophylactic group if NIV was started directly after extubation and to a nonprophylactic group if NIV was started after signs and symptoms of ARF developed. Patients were designated as responders if they received NIV and did not require reintubation during their CVICU stay and nonresponders if they failed NIV and reintubation was performed. The data collected included demographic data, preexisting conditions, pre-event characteristics, event characteristics, and outcome data. The outcome data evaluated included success or failure of NIV, duration of NIV, CVICU length of stay (LOS), hospital LOS, and hospital mortality. The two complications of NIV assessed in the study included nasal bridge or forehead skin necrosis and pneumothorax. The 221 eligible events during the study period involved 172 responders (77.8 %) and 49 nonresponders (22.2 %). A total of 201 events experienced by the study cohort received continuous positive airway pressure (CPAP), with 156 responders (78 %), whereas 20 events received bilevel positive airway pressure (BiPAP), with 16 responders (80 %). In the study, 58 events (26.3 %) were assigned to the prophylactic group and 163 events (73.7 %) to the nonprophylactic group. Compared with the nonprophylactic group, the prophylactic group experienced significantly shorter CVICU LOS (median, 49 vs 88 days; p = 0.03) and hospital LOS (median, 60 vs 103 days; p = 0.05). The CVICU LOS and hospital LOS did not differ significantly between the responders (p = 0.56) and nonresponders (p = 0.88). Significant variables identifying a responder included a lower risk-adjusted classification for congenital heart surgery (RACHS-1) score (1–3), a good left ventricular ejection fraction, a normal respiratory rate (RR), normal or appropriate oxygen saturation, prophylactic or therapeutic glucocorticoid therapy within 24 h of NIV initiation, presence of atelectasis, fewer than two organ system dysfunctions, fewer days of intubation before extubation, no clinical or microbiologic evidence of sepsis, and no history of reactive airway disease. As a well-tolerated therapy, NIV can be safely and successfully applied in critically ill children with cardiac disease to prevent extubation failure. The independent predictors of NIV success include lower RACHS-1 classification, presence of atelectasis, steroid therapy received within 24 h after NIV, and normal heart rate and oxygen saturations demonstrated within 24 h after initiation of NIV.     

Evaluation of the WHO clinical decision rule for streptococcal pharyngitis

Aims: To prospectively assess the WHO clinical decision rule (CDR) for group A beta haemolytic streptococcal (GABHS) pharyngitis in three countries. Methods: A prospective, observational cohort study in urban outpatient clinics in Rio de Janeiro, Cairo, and Zagreb. There were 2225 children aged 2–12 years with cough, rhinorrhoea, red or sore throat; 1810 of these with sore throat were included in the analysis. Results: The proportion of children presenting with sore throat and found to have GABHS pharyngitis ranged from 24.6% (Brazil) to 42.0% (Croatia). WHO CDR sensitivity was low for all sites in both age groups. In children age 5 or older, sensitivity ranged from 3.8% in Egypt to 10.8% in Brazil. In children under 5, sensitivity was low (0.0–4.6%) Specificity was high in both age groups in all countries (93.8–97.4%). Conclusions: In these populations, the current WHO CDR has high specificity, but low sensitivity; it did not detect up to 96.0% of children who have laboratory confirmed GABHS pharyngitis. A CDR with higher sensitivity should be developed for use in regions where rheumatic fever and rheumatic heart disease are still major health problems.     

Progression of Aortic Regurgitation After Different Repair Techniques for Congenital Aortic Valve Stenosis

We sought to characterize the incidence of AR progression and determine risk factors for AR progression in a consecutive series of infants and children after surgical correction of congenital aortic valvular and supravalvular stenosis. N = 30 patients underwent repair of the aortic valve for isolated congenital aortic valve stenosis (n = 14, 47 %) or combined with aortic regurgitation (AR, n = 16, 53 %). N = 27 (90 %) had a valvular and n = 3 patients (10 %) presented with supravalvular pathology of their aortic valve. In n = 16 patients (53 %) a bicuspid and in n = 2 (6 %) patients, a unicuspid valve was present. Comparative survival was analyzed using the Cox model and log-rank calculations. Log-rank calculations were performed for variables reaching statistical significance in order to identify differences in survival between groups. Commissurotomy was performed in n = 20 patients, patch implantation in n = 4, cusp shaving in n = 8, cusp prolapse correction in n = 3, and cusp augmentation in n = 4 patients. In patients with combined dysfunction and preoperative AR, AR was successfully reduced by the initial procedure, and postoperatively the overall median AR grade was 1+ (range 0–2.5+, p = 0.001, for AR reduction among patients with any grade of preoperative AR). By the time of follow-up echocardiography, the median AR grade had significantly progressed toward 1.5+ (p = 0.004). At the time of mid-term follow-up at 3.2 years, none of the patients had moderate or severe AR grades >2.5+. Patients with a monocuspid aortic valve and patients who had some kind of patch implantation into their cusps or commissures or shaving of thickened cusps were more likely to present with progression of aortic regurgitation. Monocuspid aortic valve and patch implantation, as well as cusp shaving, are probably linked to AR progression. The standard procedure of commissurotomy results in an absolute rate of AR progression of 40 % over a medium-term follow-up period.     

Evaluation of the WHO clinical decision rule for streptococcal pharyngitis.

AIMS: To prospectively assess the WHO clinical decision rule (CDR) for group A beta haemolytic streptococcal (GABHS) pharyngitis in three countries. METHODS: A prospective, observational cohort study in urban outpatient clinics in Rio de Janeiro, Cairo, and Zagreb. There were 2225 children aged 2-12 years with cough, rhinorrhoea, red or sore throat; 1810 of these with sore throat were included in the analysis. RESULTS: The proportion of children presenting with sore throat and found to have GABHS pharyngitis ranged from 24.6% (Brazil) to 42.0% (Croatia). WHO CDR sensitivity was low for all sites in both age groups. In children age 5 or older, sensitivity ranged from 3.8% in Egypt to 10.8% in Brazil. In children under 5, sensitivity was low (0.0-4.6%) Specificity was high in both age groups in all countries (93.8-97.4%). CONCLUSIONS: In these populations, the current WHO CDR has high specificity, but low sensitivity; it did not detect up to 96.0% of children who have laboratory confirmed GABHS pharyngitis. A CDR with higher sensitivity should be developed for use in regions where rheumatic fever and rheumatic heart disease are still major health problems.     

Clinical score card for diagnosis of group A streptococcal sore throat

Objective: Group A β hemolytic streptococcus (GAS) sore throat primarily occurs among children in 5–15 years age group, and if not treated appropriately causes rheumatic fever/.rheumatic heart disease (RF/RHD). Present study was aimed at validation of a clinical scoring system for diagnosis of GAS.Methods: Five hundred and thirty six children in 5–15 years age group were enrolled by systematic random selection of households from a peri-urban slum of Chandigarh. They were visited fortnightly at their home for one year to record signs and symptoms of cough and cold. Throat swabs were collected in 918 episodes, of which 123 (13.4%) were GAS culture positive.Result : Significant association of GAS was found with pain in the throat, enlarged tonsils, pharyngeal erythema and tender cervical lymphadenopahty. According to the percentage positivity of GAS culture, weighted scores were assigned to age of the child, season of occurrence, fever, size of tonsil, pharyngeal erythema and exudate, lymphadenopathy and pain in throat. Combinations of various symptoms and signs gave sensitivity of 86–89% and specificity of 83–89% whereas clinical score of 15 or more had 91% sensitivity and 98% specificity for diagnosis of GAS pharyngitis.Conclusion: As the level of clinical acumen and prevalence of GAS may differ in different primary care settings of the country, the proposed scoring system should be validated and adapted to suit local conditions before establishing it in the primary prophylaxis strategy to prevention of RF/RHD.     

Mortality and hospitalisation costs of rheumatic fever and rheumatic heart disease in New Zealand

Aims: To estimate the annual mortality and the cost of hospital admissions for acute rheumatic fever (ARF) and rheumatic heart disease (RHD) for New Zealand residents. Methods: Hospital admissions in 2000–2009 with a principal diagnosis of ARF or RHD (ICD9_AM 390‐398; ICD10‐AM I00‐I099) and deaths in 2000–2007 with RHD as the underlying cause were obtained from routine statistics. The cost of each admission was estimated by multiplying its diagnosis‐related group (DRG) cost weight by the national price for financial year 2009/2010. Results: There were on average 159 RHD deaths each year with a mean annual mortality rate of 4.4 per 100 000 (95% confidence limit 4.2, 4.7). Age‐adjusted mortality was five‐ to 10‐fold higher for Māori and Pacific peoples than for non‐Māori/Pacific. The mean age at RHD death (male/female) was 56.4/58.4 for Māori, 50.9/59.8 for Pacific and 78.2/80.6 for non‐Māori, non‐Pacific men and women. The average annual DRG‐based cost of hospital admissions in 2000–2009 for ARF and RHD across all age groups was $12.0 million (95% confidence limit $11.1 million, $12.8 million). Heart valve surgery accounted for 28% of admissions and 71% of the cost. For children 5–14 years of age, valve surgery accounted for 7% of admissions and 27% of the cost. Two‐thirds of the cost occurs after the age of 30. Conclusions: ARF and RHD comprise a burden of mortality and hospital cost concentrated largely in middle age. Māori and Pacific RHD mortality rates are substantially higher than those of non‐Māori/Pacific.     

Measurement of Cardiac Output in Children by Pressure-Recording Analytical Method

We evaluated two pressure-recording analytical method (PRAM) software versions (v.1 and v.2) to measure cardiac index (CI) in hemodynamically stable critically ill children and investigate factors that influence PRAM values. The working hypothesis was that PRAM CI measurements would stay within normal limits in hemodynamically stable patients. Ninety-five CI PRAM measurements were analyzed in 47 patients aged 1–168 months. Mean CI was 4.1 ± 1.4 L/min/m² (range 2.0–7.0). CI was outside limits defined as normal (3–5 L/min/m²) in 53.7 % of measurements (47.8 % with software v.1 and 69.2 % with software v.2, p = 0.062). Moreover, 14.7 % of measurements were below 2.5 L/min/m², and 13.6 % were above 6 L/min/m². CI was significantly lower in patients with a clearly visible dicrotic notch than in those without (3.7 vs. 4.6 L/min/m², p = 0.004) and in children with a radial arterial catheter (3.5 L/min/m²) than in those with a brachial (4.4 L/min/m², p = 0.021) or femoral catheter (4.7 L/min/m², p = 0.005). By contrast, CI was significantly higher in children under 12 months (4.2 vs. 3.6 L/min/m², p = 0.034) and weighing under 10 kg (4.2 vs. 3.6 L/min/m², p = 0.026). No significant differences were observed between cardiac surgery patients and the rest of children. A high percentage of CI measurements registered by PRAM were outside normal limits in hemodynamically stable, critically ill children. CI measured by PRAM may be influenced by the age, weight, location of catheter, and presence of a dicrotic notch.     

Provision of Transition Education and Referral Patterns from Pediatric Cardiology to Adult Cardiac Care

ACC/AHA guidelines recommend a structured preparation for and transfer to adult-oriented cardiac care for adult survivors of pediatric onset heart disease (POHD). Given this, we sought to describe the transition and transfer practices for a cohort of young adults with POHD and to determine factors associated with successful transfer to adult-oriented cardiac care. We performed a single-center, retrospective chart review on patients ≥18 years of age, with POHD likely to require lifelong cardiac care, who were seen in outpatient pediatric cardiology (PC) between 2008 and 2011. Successful transfer was defined as the subsequent attendance at adult cardiology (AC) within 2 years of PC visit. We identified 118 patients who met study criteria. Mean age 22.4 ± 2.0 years, 59 % male, 64 % white and 40 % Hispanic. Mean transition education topics noted was 3.3 ± 1.8 out of 20 and covered the underlying cardiac disease (89 %), follow-up and current medications (56 %) and exercise limitations (34 %). Recommendations for follow-up were AC (57 %) and PC (33 %). Of those told to transfer to AC, 79 % successfully transferred. Characteristics of successful transfer included: prior cardiac surgery (p = 0.008), cardiac medication use (p = 0.006) and frequency of follow-up ≤1 year (p = 0.037). One-quarter of all subjects did not follow-up within at least 2 years. Despite published guidelines, transition education appears lacking and the approach to transfer to adult cardiac care is not consistent. Given the increased risk of morbidity and mortality in this patient population, standardization of transition education and transfer processes appear warranted.     

Control of rheumatic fever in developing countries

Steps which need to be taken for control of rheumotic fever keeping in mind the available resources in developing countries are the establishment of a national rheumatic fever control programme, which should be linked with school health care programme. Cases of rheumatic heart disease should be detected and referred to RHD registries for secondary prophylaxis programme. In the high risk age group of 5–15 years the parents and teachers should be vigilant about sore throat, which should be diagnosed and treated vigorously. The establishment of laboratory competence in streptococcal bacteriology and serology all over the country is of prime importance.     

Infective Endocarditis in Previously Healthy Children With Structurally Normal Hearts

Structural heart disease, intravascular catheters, and invasive procedures are predisposing factors for infective endocarditis (IE). Data on IE in children with structurally normal hearts and no predisposing factors are limited. We aim to characterize IE (definite or possible by Duke criteria) in such a subgroup of pediatric patients (age <18 years) who were treated at our medical center. Of 51 events of IE in 50 children, 9 (18 %) had no predisposing factors. These infections were all community-acquired and presented with fever, a newly detected heart murmur, diagnostic echocardiographic findings, and left-sided infection. Clinical course was characterized by acute onset (n = 8 of 9) with a 100 % complication rate (heart failure or embolic phenomena). Emergency cardiac surgery was performed in 7 children (Ross surgery [n = 4], mitral valve replacement [MVR; n = 2], and valve repair [n = 1]). Causative organisms were S. aureus (n = 3), S. pneumoniae (n = 2), H. parainfluenzae (n = 1), and K. kingae (n = 1). In contrast, IE in children with predisposing factors (42 of 51 [82 %]) was frequently health care–associated (30 of 42), right-sided (20 of 42, p = 0.041), and with lower rates of diagnostic echocardiographic findings (28 of 42, p = 0.041), complications (16 of 42, p < 0.001), and surgical intervention (9 of 42, p = 0.002). Causative organisms were mainly viridans streptococci (n = 9), Candida species (n = 8), coagulase-negative staphylococci (n = 6), enteric Gram-negative bacilli (n = 6), S. aureus (n = 5), and K. kingae (n = 3). Mortality was 11 % in both groups. We conclude that pediatric IE in children with and without predisposing factors differs significantly. Due to the acute and complicated course of the latter, high awareness among pediatricians and prompt diagnosis are crucial.     

The changing incidence of juvenile mitral stenosis and natural history of rheumatic mitral valvulitis in Al Baha, Saudi Arabia

This study aimed to evaluate the impact of improved socio-economic conditions and health care on chronic mitral valve complications of rheumatic heart disease in Al Baha in the Kingdom of Saudi Arabia. Altogether, 190 cases of acute rheumatic fever (ARF) seen between 1982 and 1996 and 146 cases of symptomatic mitral stenosis seen between 1984 and 1996 were analysed. 2-D/Doppler echocardiography was used to evaluate the severity and progression of mitral regurgitation (MR)/mitral stenosis (MS). Of 90 cases of ARF who presented initially with MR, the murmur remained unchanged in 40%, increased in 30% and had decreased or disappeared in 30%. In cases with recurrence of ARF, mitral incompetence deteriorated in 16 of 26 cases (61.5%) and remained stable in ten (38%). Severe MS developed during follow-up in three cases of ARF, all aged less than 20 years. Seven of 50 cases (14%) of severe MS studied were aged 20 years or less. The 43% (25 of 58 cases) of severe cases of MS aged 20 years or less reported from King Faisal Hospital, Riyadh in 1981 compared with 14% of severe cases aged 20 years or less in this study indicates a significant slowing down of the rate of progression of MS following an attack of ARF in this region. Among individuals with MR during an ARF attack who reliably take their antibiotic prophylaxis, 70% will lose that murmur within 5 years of follow-up. In this series, only 30% showed a decrease or loss of MR and this probably reflects the degree of non-compliance with secondary antibiotic prophylaxis in our cases. The rate of default from antibiotic prophylaxis was 32.6%.