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.

Continent cutaneous diversion: Unveiling the interplay of neuro-urology and oncological challenges.

OBJECTIVES: The objective of our study is to demonstrate the practical application of continent cutaneous urinary diversion (CCUD) in oncological patients, with a focus on various aspects of the procedure: surgical challenges, functional outcomes, and quality of life. MATERIALS AND METHODS: We studied the perioperative and follow-up data of patients who underwent cystectomy for cancer associated with CCUD (Mitrofanoff, Monti or Casale). We retrospectively analyzed complications within 30days and beyond 30days post-surgery. We evaluated oncological outcomes. Patients' quality of life was assessed using the Bladder Cancer Index (BCI) questionnaire. Results are given on an intention-to-treat basis. RESULTS: A total of 24 patients were included in the study (July 2001 and May 2022), with a median follow-up of 62.5months. We report three deaths due to neoplasic recurrence. Forty-six percent had an early postoperative complication, two of whom required revision surgery. Overall, the medium-term complication rate was 70% and the reoperation rate was 62%. There were 8 stomal cutaneous stenoses (33%) and 3 uretero-ileal stenoses (12.5%). Overall satisfaction was rated at 9.2/10 on average, and body image was unaltered or slightly altered in 62.5% of patients. Of the patients who responded to the BCI, 75% had complete continence. DISCUSSION: The experience gained with continent stomas in neuro-urology has allowed, in carefully selected cases, to offer patients an alternative that can improve their quality of life in a context already burdened by the shadow of cancer. CCUD can be proposed as an alternative to Bricker diversion in cases of urethral invasion or a high risk of neobladder incontinence, in selected patients.

Let's get physical: Aerobic capacity, muscle strength, and muscle endurance after pediatric heart transplantation.

BACKGROUND: Pediatric heart (HTx) and kidney transplant (KTx) recipients may have lower physical fitness than healthy children. This study sought to quantify fitness levels in transplant recipients, investigate associations to clinical factors and quality of life, and identify whether a quick, simple wall-sit test is feasible as a surrogate for overall fitness for longitudinal assessment. METHODS: Aerobic capacity (6-min walk test, 6MWT), normalized muscle strength, muscle endurance, physical activity questionnaire (PAQ), and quality of life (PedsQL™) were prospectively assessed in transplanted children and matched healthy controls. RESULTS: Twenty-two HTx were compared to 20 controls and 6 KTx. 6MWT %predicted was shorter in HTx (87.2 [69.9-118.6] %) than controls (99.9 [80.4-120] %), but similar to KTx (90.3 [78.6-115] %). Muscle strength was lower in HTx deltoids (6.15 [4.35-11.3] kg/m2) and KTx quadriceps (9.27 [8.65-19.1] kg/m2) versus controls. Similarly, muscle endurance was lower in HTx push-ups (28.6 [0-250] %predicted), KTx push-ups (8.35 [0-150] %predicted), HTx curl-ups (115 [0-450] %predicted), and KTx wall-sit time (18.5 [10.0-54.0] s) than controls. In contrast to HTx with only 9%, all KTx were receiving steroid therapy. The wall-sit test significantly correlated with other fitness parameters (normalized quadriceps strength R = .31, #push-ups R = .39, and #curl-ups R = .43) and PedsQL™ (R = .36). CONCLUSIONS: Compared to controls, pediatric HTx and KTx have similarly lower aerobic capacity, but different deficits in muscle strength, likely related to steroid therapy in KTx. The convenient wall-sit test correlates with fitness and reported quality of life, and thus could be a useful easy routine for longitudinal assessment.

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.

Cardiac Biomarkers Aid in Differentiation of Kawasaki Disease from Multisystem Inflammatory Syndrome in Children Associated with COVID-19.

Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 show clinical overlap and both lack definitive diagnostic testing, making differentiation challenging. We sought to determine how cardiac biomarkers might differentiate KD from MIS-C. The International Kawasaki Disease Registry enrolled contemporaneous KD and MIS-C pediatric patients from 42 sites from January 2020 through June 2022. The study population included 118 KD patients who met American Heart Association KD criteria and compared them to 946 MIS-C patients who met 2020 Centers for Disease Control and Prevention case definition. All included patients had at least one measurement of amino-terminal prohormone brain natriuretic peptide (NTproBNP) or cardiac troponin I (TnI), and echocardiography. Regression analyses were used to determine associations between cardiac biomarker levels, diagnosis, and cardiac involvement. Higher NTproBNP (≥ 1500 ng/L) and TnI (≥ 20 ng/L) at presentation were associated with MIS-C versus KD with specificity of 77 and 89%, respectively. Higher biomarker levels were associated with shock and intensive care unit admission; higher NTproBNP was associated with longer hospital length of stay. Lower left ventricular ejection fraction, more pronounced for MIS-C, was also associated with higher biomarker levels. Coronary artery involvement was not associated with either biomarker. Higher NTproBNP and TnI levels are suggestive of MIS-C versus KD and may be clinically useful in their differentiation. Consideration might be given to their inclusion in the routine evaluation of both conditions.

Physical Activity Promotion Among Individuals With Tetralogy of Fallot.

In the general population, the most cited barriers to physical activity (PA) are time, energy, and motivation. Consequently, despite the significant contribution of PA to health and well-being, many individuals are insufficiently active. Physical inactivity and sedentary lifestyles increase the risk of acquired cardiometabolic disease, a risk that may complicate and is compounded by the anatomic and physiologic features inherent in the patient with repaired tetralogy of Fallot (ToF). Individuals with ToF commonly present with reduced exercise capacity and PA levels. In light of historically PA restrictive management of their heart disease, known reductions in exercise capacity among individuals with ToF are combined with psychosocial barriers to their participation, potentially establishing a cycle of further detraining, inactivity, and disease progression/health decline. To this end, children and young adults with ToF are known to have reduced self-efficacy towards PA, defined as their confidence in their ability to participate. In this review, we apply self-efficacy as an overarching mediator of PA participation and explore trends and determinants of PA participation among individuals with ToF and its subsequent impact on exercise capacity, disease risk, and health-related quality of life. We outline the rationale and strategies aimed at improving PA in children and adults with ToF and highlight current knowledge gaps and future directions in the promotion of PA in the population with ToF.

Dans la population générale, les obstacles à la pratique de l'activité physique (AP) le plus fréquemment mentionnés sont le manque de temps, d'énergie et de motivation. Malgré les bienfaits importants de l'AP pour la santé et le bien-être, de nombreuses personnes ne sont pas suffisamment actives. L'inactivité physique et la sédentarité augmentent le risque de maladies cardiométaboliques acquises, et ce risque pourrait s'aggraver et s'assortir de complications chez les patients présentant une tétralogie de Fallot (TF) réparée, en raison de leurs caractéristiques anatomiques et physiologiques. Une tolérance limitée à l'effort et un faible niveau d'AP sont fréquents chez les patients ayant une TF. Compte tenu des approches thérapeutiques antérieures qui limitaient l'AP, cette réduction de la tolérance à l'effort est combinée à des obstacles psychosociaux qui favorisent l'inactivité et la progression de la maladie ou la détérioration de l'état de santé. Les enfants et les jeunes adultes ayant une TF présentent donc une réduction connue de l'auto-efficacité à cet égard, c'est-à-dire leur niveau de confiance dans leur capacité de participer à des AP. Notre article de synthèse présente l'auto-efficacité comme un médiateur global de la participation à l'AP et nous explorons les tendances et les déterminants de la participation à l'AP chez les personnes présentant une TF. Nous présentons également les répercussions sur la tolérance à l'effort, le risque de maladies et l'indice de la qualité de vie liée à la santé (QVLS). Nous décrivons les raisons qui font valoir l'importance de l'AP chez les enfants et les adultes présentant une TF et des stratégies pour y arriver, en plus de souligner les lacunes dans les connaissances actuelles et de proposer des orientations futures pour la promotion de l'AP chez ces patients.