Exploring Health-Related Quality of Life in Children With Hypertrophic Cardiomyopathy and Relationship to Physical Activity.

BACKGROUND: Hypertrophic cardiomyopathy is a burdensome condition that inflicts both physical and psychological impairment on those with the disease, negatively impacting health-related quality of life (HRQoL). Given the abundance of evidence suggesting a role of physical activity (PA) in modulating HRQoL in healthy populations of children, we sought to determine the relationship between HRQoL and PA in children diagnosed with hypertrophic cardiomyopathy. METHODS AND RESULTS: A multicenter prospective observational cohort study was conducted, with patients with hypertrophic cardiomyopathy aged 10 to 19 years being provided a wrist-worn activity tracker (Fitbit Charge HR) to wear for 14 days. Patients self-reported on Pediatric Quality of Life 4.0 quality of life inventory items, which were associated with PA metrics following covariate adjustment using linear regression. A total of 56 participants were recruited to the study. The median age at enrollment was 15.5 years (interquartile range, 13.8-16.8), and 16 out of 56 (29%) of the cohort were girls. The cohort reported decreased metrics of physical, psychosocial, and total summary scores compared with health reference populations, with scores comparable with that of published populations with chronic disease. Increased physical HRQoL scores were significantly associated with increased daily steps taken, distance traveled, and flights of stairs climbed. CONCLUSIONS: These results show that impaired PA correlates with reduced HRQoL in children with hypertrophic cardiomyopathy, suggesting PA may partially mediate HRQoL in this population.

Unique Aspects of Hypertrophic Cardiomyopathy in Children.

Hypertrophic cardiomyopathy (HCM) is a primary heart muscle disease characterized by left ventricular hypertrophy that can be asymptomatic or with presentations that vary from left ventricular outflow tract obstruction, heart failure from diastolic dysfunction, arrhythmias, and/or sudden cardiac death. Children younger than 1 year of age tend to have worse outcomes and often have HCM secondary to inborn errors of metabolism or syndromes such as RASopathies. For children who survive or are diagnosed after 1 year of age, HCM outcomes are often favourable and similar to those seen in adults. This is because of sudden cardiac death risk stratification and medical and surgical innovations. Genetic testing and timely cardiac screening are paving the way for disease-modifying treatment as gene-specific therapies are being developed.

Administrative Databases: Friend or Foe in Paediatric Cardiomyopathy.

Background: Cardiomyopathy (CM) is a rare childhood disease associated with morbidity and mortality. Limited data exist on paediatric CM in Canada. Given the rare nature, single-centre studies are not sufficiently powered to address important questions. Therefore, administrative health data may serve as a resource for the study of childhood CM. The goal of this study was to validate the accuracy of International Classification of Diseases (ICD)-based algorithms to identify paediatric CM in health databases using a clinical registry as the gold standard. Methods: The clinical registry was compiled from outpatient and inpatient records at the Stollery Children's Hospital (January 1, 2013, to December 31, 2021). Patients were categorized as having CM or screened without CM. Data were linked to administrative health databases using the patient's Unique Lifetime Identifier. Algorithms based on the presence of ICD, 10th Revision, codes for CM were then evaluated, and cross-tabulations against the clinical registry were generated. Accuracy, positive predictive value, negative predictive value, sensitivity, and specificity were calculated. Results: The clinical registry had 90 patients with CM and 249 screened without CM. The algorithms ruled out CM (high negative predictive value) but had variability in the ability to diagnose CM positive predictive value. The algorithm that performed the best was based on a diagnosis of CM in a hospitalization or 2 ambulatory visits. Conclusions: A combination of inpatient and outpatient databases can be used, with acceptable accuracy, to identify paediatric patients with CM. This finding allows for the use of the identified algorithm for the comprehensive study of paediatric CM in Canada.

Contexte: La cardiomyopathie (CM) est une maladie rare de l'enfance associée à des taux élevés de morbidité et de mortalité et sur laquelle les données sont limitées en contexte canadien. En raison de la rareté de cette maladie, les études monocentriques ne peuvent atteindre la puissance statistique nécessaire pour répondre à certaines questions importantes. Les données administratives sur la santé peuvent donc constituer une ressource intéressante pour examiner la CM chez les enfants. La présente étude visait à valider l'exactitude d'algorithmes fondés sur la Classification internationale des maladies (CIM) pour repérer les cas de CM pédiatrique dans des bases de données sur la santé, en utilisant un registre clinique comme référence. Méthodologie: Un registre clinique a été élaboré à partir des dossiers de clinique interne et externe du Stollery Children's Hospital (du 1er janvier 2013 au 31 décembre 2021). Les patients ont été classés en deux catégories : atteints de CM ou dépistés sans CM. Les données ont été liées aux bases de données administratives sur la santé en utilisant le numéro d'identification unique des patients (Unique Lifetime Identifier). Des algorithmes fondés sur les codes de la CIM-10 ont été évalués et des analyses croisées avec le registre clinique ont été réalisées. L'exactitude, la valeur prédictive positive (VPP), la valeur prédictive négative (VPN), la sensibilité et la spécificité des algorithmes ont été calculées. Résultats: Le registre clinique comprenait 90 patients atteints de CM et 249 patients dépistés sans CM. Les algorithmes permettaient d'exclure correctement la CM (VPN élevée), mais leur capacité à établir le diagnostic de CM variait (VPP). L'algorithme le plus performant était fondé sur l'attribution d'un code diagnostique de CM lors d'une hospitalisation ou de deux visites ambulatoires. Conclusions: La combinaison de bases de données sur les hospitalisations et sur les soins externes peut être utilisée pour identifier les enfants atteints de CM avec une exactitude acceptable. Cette observation corrobore l'utilisation de l'algorithme ciblé pour réaliser une étude exhaustive de la CM chez l'enfant au Canada.

Correction: Multimedia Knowledge Translation Tools for Parents About Childhood Heart Failure: Environmental Scan.

[This corrects the article DOI: 10.2196/34166.].

Multimedia Knowledge Translation Tools for Parents About Childhood Heart Failure: Environmental Scan.

BACKGROUND: Childhood heart failure is a factor in many hospital admissions each year. It can impose a steep learning curve for parents who need to learn the key information to care for their child at home. In this study, we conducted an environmental scan to identify and assess web-based knowledge translation tools about childhood heart failure for parent audiences developed within North America. OBJECTIVE: The aim of this study is to inventory tools publicly available to parents about childhood heart failure from popular web-based venues, assess how each tool communicates health information, and explore how they were developed. METHODS: Our search strategy included two commonly used multimedia-based platforms: two app stores (Google Play and Apple App Store) and one search engine (Advanced Google Search). Common search terms were used, and results were uploaded to Microsoft Excel for screening between 2 reviewers. The inclusion criteria for the tools were as follows: content focused on educating parents about their child's heart failure, developed in the English language, and originating within Canada and the United States. A total of 2 reviewers screened the app store and internet search results for relevant tools. Each tool was assessed using the Suitability Assessment of Materials (SAM), a validated tool that objectively assesses the suitability of how health information is communicated to a particular audience. Key informants who were involved in tool development were identified and invited for a qualitative interview using a semistructured format to provide data about the development process. Key themes were identified in the semistructured interview process. RESULTS: Frequencies and SAM percent ratings of eligible tools were reported. No apps exist for parents relating to pediatric heart failure. Overall, 17 relevant internet tools were identified, and their suitability was assessed for the parent audience. Most tools scored well in layout and type, but they scored lower in readability and graphics. Qualitative interviews with key informants revealed three key themes: timely and introductory knowledge, credible and trustworthy knowledge, and challenges and evolution in knowledge. CONCLUSIONS: This is the first environmental scan looking for parent tools relating to childhood heart failure in Canada and the United States. Findings from this study reveal that there are no apps on this topic and there is a small number of tools for parents on the internet (n=17). Using the SAM, no tools scored in the superior range, and further work in knowledge translation strategies needs to be done in this area to improve more effective education to parents and caregivers who have a child with heart failure. These findings will inform the development of a new resource on children's heart failure that targets parents and caregiver audiences.

Use of Photovoice to Explore Pediatric Patients with Hypertrophic Cardiomyopathy and their Parents' Perceptions of a Heart-Healthy Lifestyle.

Background Heart-healthy lifestyles promote lifelong cardiovascular health. However, patients with hypertrophic cardiomyopathy are often advised to avoid strenuous exercise because of the risk of sudden cardiac death. Given these restrictions, this study explored youth and parent perceptions of a heart-healthy lifestyle and the barriers and facilitators to this lifestyle. Methods and Results Youth and parents were purposefully recruited at 2 Canadian hospitals for this photovoice project. Participants were given cameras and took pictures of everyday heart-healthy or heart-unhealthy choices. Photos were discussed during one-on-one qualitative interviews with youth and parents separately to understand the photos' meaning and significance. Inductive descriptive thematic analysis was employed. A total of 16 youth (median age, 14.4 years [range, 10.5-17.7 years]; 63% boys) and 16 parents (100% women) participated. A total of 15 youth were activity restricted. Data analysis revealed 7 categories organized into perceptions of healthy living (health is holistic and individualized) and factors influencing engagement in healthy living (self-awareness, ownership and autonomy, feeling restricted and peer pressure, support from parents, and support from the cardiologist). Participants had a complex understanding of health and discussed the importance of physical, mental, and social well-being. Youth used self-awareness and taking responsibility as facilitators of healthy living. Healthy living was shaped by peers, parent role-modeling, and cardiologist recommendations. Conclusions This study depicts the realities for youth with hypertrophic cardiomyopathy and can be used to inform the development of responsive interventions. Holistic, patient-specific interventions may be more successful, and strategies such as shared decision making may be important to promote self-awareness and autonomy.

Estimating nutritional needs in paediatric heart failure: beyond the equations.

BACKGROUND: Nutrition optimisation is imperative in paediatric patients with heart failure. Energy needs can be assessed using indirect calorimetry. METHODS: Presented are two cases of children with clinical heart failure who benefited from indirect calorimetry. RESULTS: Using indirect calorimetry, it was determined both cases were hypermetabolic. CONCLUSION: These cases demonstrate the impact of heart failure on metabolic rate and growth. Energy requirements were up to two times higher than estimations from predictive equations.

Strategies to maintain a family-centered care approach in the era of COVID-19: Experiences of a Canadian pediatric cardiology program.

The COVID-19 pandemic has resulted in strict provincial guidelines to prevent its spread. Physical distancing requirements, the postponement of elective pediatric cardiac surgeries and non-invasive cardiac interventions, and hospital visitor restrictions have significantly impacted services provided by our pediatric cardiac program. Rapid modifications to current inpatient and outpatient practices were required to maintain a family-centered care approach. Strategies our team used to maintain a family-centered care approach focused on six key areas including inpatient care, outpatient pediatric cardiology clinics, family meetings, discharge planning and teaching, the connection of inpatient pediatric patients to the outside world, and social support. The majority of our strategies are adaptable to other pediatric cardiology programs and some may prove useful after the pandemic and as restrictions lift. These strategies each have their own limitations and challenges that must be considered when adapting them to other pediatric cardiology programs and continuing their use after the pandemic has resolved.

Hypertension masquerading as Pediatric Cardiomyopathy: an exercise in cognitive biases.

Heuristics and cognitive biases constantly influence clinical decision-making and often facilitate judgements under uncertainty. They can frequently, however, lead to diagnostic errors and adverse outcomes, particularly when considering rare disease processes that have common, masquerading presentations. Herein, we present two such cases of newborn infants with hypertensive renal disorders that were initially thought to have cardiomyopathy.

Risk Factors for Cardiac and Non-cardiac Causes of Death in Males with Duchenne Muscular Dystrophy.

As survival and neuromuscular function in Duchenne muscular dystrophy (DMD) have improved with glucocorticoid (GC) therapy and ventilatory support, cardiac deaths are increasing. Little is known about risk factors for cardiac and non-cardiac causes of death in DMD. A multi-center retrospective cohort study of 408 males with DMD, followed from January 1, 2005 to December 31, 2015, was conducted to identify risk factors for death. Those dying of cardiac causes were compared to those dying of non-cardiac causes and to those alive at study end. There were 29 (7.1%) deaths at a median age of 19.5 (IQR: 16.9-24.6) years; 8 (27.6%) cardiac, and 21 non-cardiac. Those living were younger [14.9 (IQR: 11.0-19.1) years] than those dying of cardiac [18 (IQR 15.5-24) years, p = 0.03] and non-cardiac [19 (IQR: 16.5-23) years, p = 0.002] causes. GC use was lower for those dying of cardiac causes compared to those living [2/8 (25%) vs. 304/378 (80.4%), p = 0.001]. Last ejection fraction prior to death/study end was lower for those dying of cardiac causes compared to those living (37.5% ± 12.8 vs. 54.5% ± 10.8, p = 0.01) but not compared to those dying of non-cardiac causes (37.5% ± 12.8 vs. 41.2% ± 19.3, p = 0.58). In a large DMD cohort, approximately 30% of deaths were cardiac. Lack of GC use was associated with cardiac causes of death, while systolic dysfunction was associated with death from any cause. Further work is needed to ensure guideline adherence and to define optimal management of systolic dysfunction in males with DMD with hopes of extending survival.

Self-reported and Accelerometer-Measured Physical Activity in Children With Cardiomyopathy.

PURPOSE: Children with cardiomyopathy are at risk of heart failure with reduced physical activity (PA) as a cardinal manifestation. Clinical assessment of PA in children is challenging due to the limited validity of subjective reports. The aim of the study was to compare accelerometery measurement with parental report and to identify factors associated with movement behavior (PA, sedentary time, steps per day). METHOD: Fifteen patients with cardiomyopathy (mean, 9.7 years; 64% male) were asked to wear an Actigraph GT3X accelerometer in Edmonton, Canada. Demographics, clinical characteristics, parental-reported PA, and health-related quality of life data were also collected. RESULTS: Participants engaged in a median of 38 minutes of moderate-vigorous PA per day with higher levels in boys versus girls (73.9 [25.03, 78.91] vs 4.13 [2.53, 37.67] minutes, P = .03). Children participating in recreational sports showed a higher level of moderate-vigorous PA (73.92 min/d) compared with those who did not participate (73.9 vs 22.7 min/d, P = .05), and positive family outlook on the child's health was associated with less sedentary time (P = .04). CONCLUSION: Accelerometer measurement in children with cardiomyopathy is an achievable and more objective measure of PA compared with parental report alone and highlighted low levels of PA among these children.

Optimizing Nutrition in Pediatric Heart Failure: The Crisis Is Over and Now It's Time to Feed.

Pediatric heart failure is a complex disease occurring when cardiac output is unable to meet the metabolic demands of the body. With improved surgical interventions and medical therapies, survival rates have improved, and care has shifted from focusing on survival to optimizing quality of life and health outcomes. Based on current literature, this review addresses the nutrition needs of infants and children in heart failure and describes the pathophysiology and metabolic implications of this disease. The prevalence of wasting in pediatric heart failure has been reported to be as high as 86%, highlighting the importance of nutrition assessment through all stages of treatment to provide appropriate intake of energy, protein, and micronutrients. The etiology of malnutrition in pediatric heart failure is multifactorial and involves hypermetabolism, decreased intake, increased nutrient losses, inefficient utilization of nutrients, and malabsorption. Children in heart failure often present with tachypnea, tachycardia, fatigue, nausea, and vomiting and consequently may not be able to meet their nutrition requirements through oral intake alone. Nutrition support, including enteral nutrition and parenteral nutrition, should be considered an essential part of routine care. The involvement of multiple allied health professionals may be needed to create a feeding therapy plan to support patients and their families. With appropriate nutrition interventions, clinical outcomes and quality of life can be significantly improved.