Kawasaki disease: patients' transition journey and recommendations for adult care.

BACKGROUND: Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation. OBJECTIVE: The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults' post-health care transition. METHODS: A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed. RESULTS: Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations. CONCLUSIONS: The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.

The Healthy Eating Assessment Tool (HEAT): A Simplified 10-Point Assessment of CHILD-2 Dietary Compliance for Children and Adolescents with Dyslipidemia.

Traditional dietary assessment tools used to determine achievement of cholesterol-lowering dietary targets, defined in the Cardiovascular Health Integrated Lifestyle Diet (CHILD-2), are time intensive. We sought to determine the utility of the Healthy Eating Assessment Tool (HEAT), a simplified 10-point dietary assessment tool, in relation to meeting dietary cut points of the CHILD-2, as well as its association with markers of adiposity and lipid variables. We performed a 2-year single-center, prospective cross-sectional study of pediatric patients with dyslipidemia. HEAT score associations with meeting CHILD-2 fat targets were modest. Only patients with the highest HEAT scores (good 43%, excellent 64%) met the CHILD-2 cut point of <25% total fat calories (p = 0.03), with a non-significant trend for limiting the percentage of daily saturated fat to <8% (excellent 64%), and no association with cholesterol intake. There were more consistent associations with markers of adiposity (body mass index z-score r = -0.31, p = <0.01 and waist-to-height ratio r = -0.31, p = <0.01), and there was no independent association with lipid levels. While fat-restricted diets are safe, they are not particularly effective for treatment of dyslipidemia or for weight management alone. The HEAT may be a more useful and simplified way of assessing and tracking broader dietary goals in clinical practice.

Socioeconomic Status and Kawasaki Disease Outcomes in a Single-Payer Health Care System.

Background: For patients with Kawasaki disease (KD), lower socioeconomic status (SES) may adversely affect the timeliness of presentation and initiation of intravenous immune globulin, and coronary artery outcomes. Multipayer systems have been shown to affect health care equity and access to health care negatively. We sought to determine the association of SES with KD outcomes in a single-payer health care system. Methods: Patients with KD presenting from 2007 to 2017 at a single institution were included. SES data were obtained by matching patient postal code district with data from the 2016 Census Canada. Results: SES data were linked for 1018 patients. The proportion of households living below the after-tax low-income cutoff in the patient's postal code district was 13% for not treated, 13% for delayed intravenous immune globulin treatment, and 12% for prompt treatment (P = 0.58). Likewise, the average median annual household income was unrelated to delayed or no treatment. The percentage >15 years of age with advanced education differed between groups at 33%, 29%, and 31% for delayed treatment, prompt treatment, and missed groups, respectively (P = 0.004). SES variables were not significantly different for those with vs without coronary artery aneurysms (max Z-score: >2.5), including the proportion of households living below low-income cutoff (12% vs 13%; P = 0.37), average median annual household income (CAD$81,220 vs $82,055; P = 0.78), and proportion with a university degree (33% vs 31%; P = 0.49), even after adjusting for sex, age, year, and KD type. Conclusions: Timeliness of treatment for KD and coronary artery outcomes were not associated with SES variables within a single-payer health care system.

Contexte: Chez les patients atteints de la maladie de Kawasaki (MK), un statut socioéconomique (SSE) plus difficile pourrait retarder le moment de la première consultation et le début du traitement par immunoglobuline intraveineuse (IgIV) ainsi que peser sur les résultats associés aux artères coronaires. Il a été démontré que les systèmes à payeurs multiples compromettent l'équité en matière de soins de santé et l'accès à ces derniers. Nous avons cherché à déterminer s'il existait un rapport entre le SSE et les résultats associés à la MK au sein d'un système de soins de santé à payeur unique. Méthodologie: L'étude comprenait des patients atteints de la MK qui se sont présentés à un même établissement entre 2007 et 2017. Les données sur le SSE ont été obtenues en associant le code postal des patients aux données du recensement canadien de 2016. Résultats: Les données sur le SSE de 1 018 patients ont été répertoriées. La proportion des foyers qui étaient sous le seuil de faible revenu (SFR) après impôt dans la circonscription correspondant à leur code postal était la suivante : 13 % pour les patients non traités, 13 % pour les patients chez qui le traitement par IgIV a été tardif et 12 % pour les patients qui ont rapidement reçu un traitement (p = 0,58). De même, aucune relation n'a été établie entre le revenu annuel médian des ménages et un traitement tardif ou une absence de traitement. Le pourcentage de personnes âgées de plus de 15 ans ayant un niveau de scolarité élevé différait d'un groupe à l'autre, soit respectivement 33 %, 29 % et 31 % pour les groupes à traitement tardif, à traitement rapide et sans traitement (p = 0,004). Les variables en matière de SSE n'étaient pas significativement différentes chez les patients présentant des anévrismes coronariens et chez ceux n'en présentant pas (score z maximal > 2,5), peu importe la proportion des foyers qui étaient sous le SFR après impôt (12 % contre 13 %; p = 0,37), le revenu annuel médian des ménages (81 220 $ CA contre 82 055 $; p = 0,78) ou le taux de diplomation universitaire (33 % contre 31 %; p = 0,49), et ce, même après ajustement en fonction du sexe, de l'âge et du type de MK. Conclusions: Aucune corrélation n'a été établie entre le SSE et le délai avant l'instauration d'un traitement contre la MK ou les résultats liés aux artères coronaires dans le contexte d'un système de soins de santé à payeur unique.

Understanding the Educational Support and Psychosocial Needs of Parents and Adolescents With Kawasaki's Disease and Coronary Artery Aneurysms.

INTRODUCTION: Kawasaki disease (KD) with coronary artery aneurysms (complex KD) presents relentless challenges for families. Psychosocial experiences and needs were explored. METHOD: A descriptive, exploratory study of adolescents and parents using a needs survey and psychosocial instruments (anxiety, depression, and functional impairment). RESULTS: Fifty-one parents and 38 adolescents participated. Predominant interests were for information sheets, newsletters, Web sites, and phone applications. Gaps in disease-specific knowledge and awareness of coronary artery aneurysms between parents and adolescents were identified. Psychosocial concerns for adolescents included symptoms of anxiety (22%), depression (13%), and functional impairment (22%). Multivariable analyses indicated higher depression scores associated with longer travel distance from specialists (p = .04). Parent-reported social concerns for their adolescent were associated with higher anxiety (p = .005) and functional impairment (p = .005). Written commentary complemented the findings. DISCUSSION: Care protocols require psychosocial assessment/referral and the use of virtual platforms. The groundwork was laid for developing patient and family-centered strategies.

Effectiveness and Safety of Statin Therapy in Children: A Real-World Clinical Practice Experience.

BACKGROUND: Statin use for hypercholesterolemia in children is predominantly reported from short-term clinical trials. In this study, we assess the efficacy and safety of statin treatment in clinical pediatric practice. METHODS: Records of all patients who began statin treatment at age <18 years and remained on statins for >6 months from 5 pediatric lipid clinics were reviewed. Information at baseline and from all clinic evaluations after statin initiation was recorded, including lipid measurements, statin drug/dose, safety measures (anthropometry, hepatic enzymes, creatine kinase levels), and symptoms. Lipid changes on statin therapy were assessed from baseline to 6 ± 3 months and from 6 ± 3 months to last follow-up with a mixed-effects model, using piecewise linear splines to describe temporal changes, controlling for repeated measures, sex, and age. RESULTS: There were 289 patients with median low-density lipoprotein cholesterol (LDL-C) of 5.3 mmol/L (interquartile range [IQR]:4.5-6.5) and mean age of 12.4 ± 2.9 years at statin initiation. Median duration of therapy was 2.7 years (IQR: 1.6-4.5) with 95% on statins at last evaluation. There were significant decreases in total cholesterol, LDL-C, and non-high-density lipoprotein cholesterol (non-HDL-C) from baseline to 6 ± 3 months (P < 0.001) and from 6 ±3 months to last follow-up (P < 0.001). Triglycerides and HDL-C were unchanged but the triglyceride to HDL-C ratio decreased significantly by late follow-up. At final evaluation, median LDL-C had decreased to 3.4 mmol/L (IQR:2.8-4.2). No patient had statins discontinued for safety measures or symptoms. CONCLUSIONS: In real-world clinical practice, intermediate-term statin treatment is effective and safe in children and adolescents with severe LDL-C elevation.

CONTEXTE: Les statines sont fréquemment employées pour traiter l'hypercholestérolémie chez les enfants dans le cadre d'essais cliniques de courte durée. Dans l'étude présentée ici, nous évaluons l'efficacité et l'innocuité de l'emploi de statines dans la pratique clinique en pédiatrie. MÉTHODOLOGIE: Nous avons passé en revue les dossiers de tous les patients de cinq cliniques pédiatriques des lipides qui ont commencé à prendre une statine avant l'âge de 18 ans et qui ont poursuivi le traitement pendant plus de six mois. Les valeurs mesurées au départ et à chacune des évaluations cliniques après l'instauration d'un traitement par une statine ont été consignées, notamment la lipidémie, le type et la dose de la statine prescrite, les paramètres d'évaluation de l'innocuité (anthropométrie, enzymes hépatiques, taux de créatine kinase) et les symptômes. La variation de la lipidémie chez les patients recevant une statine a été évaluée sur deux périodes, soit entre le début du traitement et l'évaluation effectuée à 6 ± 3 mois ainsi qu'entre l'évaluation effectuée à 6 ± 3 mois et la dernière évaluation de suivi, à l'aide d'un modèle à effets mixtes et de splines linéaires par morceaux pour décrire les changements temporels, en contrôlant pour les mesures répétées, le sexe et l'âge. RÉSULTATS: L'étude portait sur 289 patients ayant un taux de cholestérol des lipoprotéines de basse densité (C-LDL) médian de 5,3 mmol/l (intervalle interquartile [IIQ] : 4,5 à 6,5) et âgés de 12,4 ± 2,9 ans en moyenne au moment de l'instauration du traitement par une statine. La durée médiane du traitement était de 2,7 ans (IIQ : 1,6 à 4,5), 95 % des sujets étant toujours sous statine à la dernière évaluation. Les taux de cholestérol total, de C-LDL et de cholestérol des lipoprotéines non de haute densité (C-non-HDL) avaient diminué de manière significative entre le début du traitement et l'évaluation à 6 ± 3 mois (p < 0,001) et entre l'évaluation à 6 ± 3 mois et la dernière évaluation de suivi (p < 0,001). Les taux des triglycérides et du C-HDL n'avaient pas bougé, mais le rapport triglycérides/C-HDL avait diminué considérablement vers la fin du suivi. À l'évaluation finale, le taux de C-LDL avait diminué à 3,4 mmol/l (IIQ : 2,8 à 4,2). Aucun patient n'avait abandonné le traitement par une statine en raison de problèmes d'innocuité ou des symptômes. CONCLUSIONS: En situation réelle dans la pratique clinique, le traitement à moyen terme par une statine est efficace et sûr chez les enfants et les adolescents présentant une élévation grave du taux de C-LDL.

Readiness for Transition to Adult Health Care for Young Adolescents with Congenital Heart Disease.

This study evaluates transition readiness, medical condition knowledge, self-efficacy, and illness uncertainty in young adolescents (ages 12 to 15 years) with congenital heart disease (CHD), and medical, patient, and parental factors associated with transition readiness. We enrolled 82 patients with moderate or complex CHD (n = 36, 44% male; mean age 13.6 ± 1.3 years), and their parents. Patients completed standardized self-report measures: Transition Readiness Assessment Questionnaire (TRAQ), MyHeart scale, General Self-Efficacy scale, and Children's Uncertainty in Illness Scale. Parents completed the MyHeart scale and demographic information. Many young adolescents had not discussed transfer with a health care provider (n = 20, 24%) or parent (n = 34, 41%). Transition readiness was higher among patients who were older, more knowledgeable about their condition, had a history of primary cardiac repair and greater self-efficacy, and was lower for boys and patients on cardiac medications. Transition readiness was unrelated to CHD diagnosis and patients' illness uncertainty. Patients' self-advocacy skills were superior to their chronic disease self-management skills. Increased parental medical condition knowledge was positively correlated with patient knowledge, and patient-parent discussion of transfer was associated with increased patient's self-management skills. Transition is not uniformly discussed with young adolescent CHD patients. Parental involvement is correlated with increased transition readiness and patient disease self-management skills. Young adolescent transition programs should focus on education around improving patient medical condition knowledge, promote chronic disease self-management skills development, and include parental involvement.

Factors associated with development of coronary artery aneurysms after Kawasaki disease are similar for those treated promptly and those with delayed or no treatment.

BACKGROUND: While the risk is reduced, patients may develop coronary artery (CA) aneurysms after Kawasaki disease (KD) despite receiving intravenous immunoglobulin (IVIG) within 10days of symptom onset. Risk factors for CA aneurysms may differ compared to those patients with delayed or no treatment. METHODS: Patients diagnosed with KD between 1990 and 2013 were included. Patients with maximum coronary artery z-scores>5 were classified as having CA aneurysms. Separate multivariable regression models were used to determine factors associated with CA aneurysms for those with versus without prompt treatment. RESULTS: Of 1358 patients included, 83% (n=1126) were treated with IVIG within 10days and 5% (n=53) developed CA aneurysms. Patients who had delayed (>10days) or no IVIG treatment were at increased odds of developing CA aneurysms (OR: 3.1,95% CI: 1.9-5.1, p<0.001). For patients with prompt treatment with IVIG, factors associated with increased odds of CA aneurysms were: longer duration of fever prior to treatment (OR: 1.2/day, p=0.04), age<1year (OR: 3.9, p=0.001), higher pre-IVIG white blood cell count (OR: 1.05/×109/L, p=0.007), lower hemoglobin (OR: 1.4/g/L, p=0.004) and non-response to initial IVIG treatment (OR: 2.5, p<0.001). For patients with delayed or no treatment, factors associated with increased odds of CA aneurysms were: males (OR: 5.4, p=0.009), age<1year (OR: 29.9, p<0.001), and higher platelet count (OR: 1.4/100×1012/L, p=0.001). Delayed treatment with IVIG did not reduce the risk of CA aneurysms (OR: 1.9, p=0.28), and total duration of fever was not associated with CA aneurysms for this group (OR: 1.04/day, p=0.16). CONCLUSIONS: Factors associated with the development of CA aneurysms are generally similar for those treated promptly versus those with delayed or no treatment. For those with delayed diagnosis, treatment with IVIG does not appear to be effective to prevent CA aneurysms.

Variability in Response to Intravenous Immunoglobulin in the Treatment of Kawasaki Disease.

OBJECTIVES: To characterize the pattern of temperature response to intravenous immunoglobulin (IVIG) infusion in patients with Kawasaki disease (KD). STUDY DESIGN: Patients nonresponsive to IVIG (axillary temperature ≥37.5°C >24 hours after end of IVIG) were identified. Each patient with IVIG-nonresponsive KD was matched to a control patient with IVIG-responsive KD of the same age, sex, and duration of fever before IVIG. Hourly temperature profiles were obtained from immediately before the start of IVIG infusion until complete defervescence. RESULTS: A total of 182 patients nonresponsive to IVIG were matched (total n = 364). Nonresponders were further classified as partial nonresponders (68%) (axillary temperature decreased to <37.5°C but fever recurred) and complete nonresponders (32%) (axillary temperature consistently ≥37.5°C throughout IVIG treatment). The temperature profile during IVIG infusion was similar between responders and partial nonresponders (EST: -0.061 [0.007]°C/h, P < .001 for responders vs EST: -0.027 (0.012)°C/h, P = .03 for partial nonresponders [responders vs partial nonresponders, P = .65]), where EST is the parameter estimate from the regression model, representing the change in degrees Celsius for each hour since start of IVIG. In complete nonresponders, IVIG was not associated with significant decreases in temperature (EST: -0.008 [0.010]°C, P = .42). Factors associated with complete (vs partial) nonresponse included laboratory-confirmed infection, greater C-reactive protein, and IVIG brand. Defervescence in partial nonresponders was achieved with a second IVIG dose for 72% of patients compared with 58% of complete nonresponders (P = .001). Complete nonresponders were more likely to develop coronary artery aneurysms vs partial nonresponders (OR: 2.4 [1.1-5.4], P = .03) or responders (OR: 3.2 [1.5-6.9], P = .002). CONCLUSIONS: Nonresponse to initial IVIG can be further characterized by temperature profile, and complete nonresponders may require more aggressive second-line therapy.

Management and Outcomes of Patients with Occlusive Thrombosis after Pediatric Cardiac Surgery.

OBJECTIVES: To evaluate management and outcomes of thrombosis after pediatric cardiac surgery and stratify thrombi according to risk of short- and long-term complications to better guide therapeutic choices. STUDY DESIGN: Retrospective review was performed of 513 thrombi (400 occlusive) diagnosed after 213 pediatric cardiac operations. Long-term outcomes over time were assessed with the use of parametric hazard regression models. RESULTS: Serious complications and/or high-intensity treatment occurred with 17%-24% of thrombi depending on location, most commonly in thrombi affecting the cardiac and cerebral circulation. Bleeding complications affected 13% of patients; associated factors included thrombolytics (OR 8.7, P < .001), greater daily dose of unfractionated heparin (OR 1.25 per 5 U/kg/day, P = .03), and extracorporeal support (OR 4.5, P = .007). Radiologic thrombus persistence was identified in 30% ± 3% at 12 months; associated factors included extracorporeal support (hazard ratio [HR] 1.9, P = .003), venous (HR 1.7, P = .003), and occlusive thrombi at presentation (HR 1.8, P = .001); greater oxygen saturation before surgery (HR 1.13/10%, P = .05) and thrombi in femoral veins (HR 1.9, P = .001) were associated with increased hazard of resolution. Freedom from postthrombotic syndrome was 83% ± 4% at 6 years, greater number of persistent vessel segment occlusions (HR 1.8/vessel, P = .001) and greater fibrinogen at diagnosis (HR 1.1 per g/L, P = .02) were associated with increased hazard. CONCLUSIONS: Thrombosis outcomes after pediatric cardiac surgery remain suboptimal. Given that more intensive treatment would likely increase the risk of bleeding, the focus should be on both thrombosis-prevention strategies, as well as in tailoring therapy according to a thrombosis outcome risk stratification approach.