Design and rationale of re-energize fontan: Randomized exercise intervention designed to maximize fitness in fontan patients.

In this manuscript, we describe the design and rationale of a randomized controlled trial in pediatric Fontan patients to test the hypothesis that a live-video-supervised exercise (aerobic+resistance) intervention will improve cardiac and physical capacity; muscle mass, strength, and function; and endothelial function. Survival of children with single ventricles beyond the neonatal period has increased dramatically with the staged Fontan palliation. Yet, long-term morbidity remains high. By age 40, 50% of Fontan patients will have died or undergone heart transplantation. Factors that contribute to onset and progression of heart failure in Fontan patients remain incompletely understood. However, it is established that Fontan patients have poor exercise capacity which is associated with a greater risk of morbidity and mortality. Furthermore, decreased muscle mass, abnormal muscle function, and endothelial dysfunction in this patient population is known to contribute to disease progression. In adult patients with 2 ventricles and heart failure, reduced exercise capacity, muscle mass, and muscle strength are powerful predictors of poor outcomes, and exercise interventions can not only improve exercise capacity and muscle mass, but also reverse endothelial dysfunction. Despite these known benefits of exercise, pediatric Fontan patients do not exercise routinely due to their chronic condition, perceived restrictions to exercise, and parental overprotection. Limited exercise interventions in children with congenital heart disease have demonstrated that exercise is safe and effective; however, these studies have been conducted in small, heterogeneous groups, and most had few Fontan patients. Critically, adherence is a major limitation in pediatric exercise interventions delivered on-site, with adherence rates as low as 10%, due to distance from site, transportation difficulties, and missed school or workdays. To overcome these challenges, we utilize live-video conferencing to deliver the supervised exercise sessions. Our multidisciplinary team of experts will assess the effectiveness of a live-video-supervised exercise intervention, rigorously designed to maximize adherence, and improve key and novel measures of health in pediatric Fontan patients associated with poor long-term outcomes. Our ultimate goal is the translation of this model to clinical application as an "exercise prescription" to intervene early in pediatric Fontan patients and decrease long-term morbidity and mortality.

Bereaved Caregiver Perspectives on the End of Life in Pediatric Patients With Ventricular Assist Devices.

OBJECTIVES: Ventricular assist devices (VADs) are increasingly used in pediatric heart failure as bridges to heart transplantation, although 25% will die with VADs. Family experiences in this population are not well-described. The objective is to understand bereaved families' perspectives on VAD and end-of-life decision-making. DESIGN: Semistructured interviews with bereaved caregivers of pediatric VAD patients. SETTING: Tertiary children's hospital. PATIENTS: Families of six pediatric VAD patients who died from 2014 to 2020. INTERVENTIONS: Not available. MEASUREMENTS AND MAIN RESULTS: Applying a grounded theory framework, interviews were coded by two independent readers using qualitative software. Themes were discussed in iterative multidisciplinary meetings. Participants were interviewed at a median 2.4 years after their child died. Three major themes emerged: 1) "lack of regret" for VAD implantation despite the outcome; 2) "caregiver-child accord" (via patient's verbal assent or physical cues) at implantation and end-of-life was important in family decision-making; and 3) development of a "local surrogate family" (medical team and peer families) provided powerful support. CONCLUSIONS: Bereaved families' perspectives provide insight into quality decision-making for major interventions and end-of-life care in pediatric patients with chronic illness who face decisions regarding technology dependence.

An exploratory assessment of pediatric patient and parent needs after implantable cardioverter defibrillator implant.

BACKGROUND: Placement of an implantable cardioverter defibrillator (ICD) is often accompanied by psychological adjustment issues in pediatric patients and their parents. Although anxiety, depression, and lowered quality of life have been seen in these patients, no studies have investigated patient or parent reported needs. This exploratory study describes the needs of pediatric ICD patients and parents and assesses whether patient factors of age, gender, depression, and anxiety are associated with specific needs. METHODS: ICD patients ages 8-21 years and their parents completed a needs analysis survey assessing various domains of functioning. Patients also completed self-reported measures of depression and anxiety. RESULTS: Thirty-two patients (28% female) and their parents (72% mothers) completed the survey. Patients' most frequently endorsed needs involved educational issues: understanding their cardiac event/diagnosis (34%), medications (34%), and how the ICD would change their lifestyle (31%). Parents' most frequently endorsed needs involved family issues; almost half of parents (47%) were concerned about their children's frustration with their overprotectiveness and 28% were concerned with their child feeling depressed or anxious. Patients who reported feeling overprotected (12.1 ± 3.4 vs 17.4 ± 3.5 years; P = .001) were significantly younger than those who did not. Experiencing peer issues was more frequently endorsed by females than males (33% of females vs 4% of males; P = .026). CONCLUSIONS: ICD patients and parents endorsed markedly different needs. Patients focused on understanding their ICD, whereas parents were more focused on their children's emotional needs. Novel ways of educating patients about their device and clinic-based screenings of emotional functioning may serve to meet these needs.

Posttraumatic stress disorder in pediatric patients with implantable cardioverter-defibrillators and their parents.

BACKGROUND: An implantable cardioverter-defibrillator (ICD) in the pediatric patient (and the precipitating events that led to ICD placement) can be traumatic for patients and their families and may lead to posttraumatic stress disorder (PTSD). OBJECTIVES: This study aimed to estimate the prevalence of PTSD in pediatric patients with an ICD and their parents and identify the factors associated with PTSD incidence. METHODS: Pediatric participants with an ICD aged 8-21 years and parents of children aged 0-21 years completed surveys that included demographic characteristics and PTSD measures. Pediatric participants completed additional psychosocial measures, such as anxiety and depression self-report questionnaires. RESULTS: Fifty youth (30% female) and 43 parents (70% female) completed the measures. Six of 50 youth (12%) met the screening criteria for a likely PTSD diagnosis, while 20 of 43 parents (47%) met the cutoff for PTSD on the screening measure. Children with PTSD were more likely to have had a secondary prevention ICD (83% vs 17%; P = .021), meet the clinical cutoff for depression (67% vs 16%; P = .005), and had higher shock anxiety scores (31.7 vs 17.9; P = .003) than children without PTSD. Female gender (57% vs 23%; P = .043) and patient depression (31% vs 5%; P = .042) were associated with PTSD in parents. CONCLUSION: Parents were found to be more likely to meet the criteria for PTSD than youth. In youth, PTSD was associated with medical and psychosocial factors, whereas PTSD in parents was associated with being female and child depression. Clinic-based screenings and management planning of emotional functioning are warranted to address psychological distress in patients and parents.

A Case of Abulia From Left Middle Cerebral Artery Stroke in an Adolescent Treated Successfully With Short Duration Olanzapine.

INTRODUCTION: Abulia is defined as a pathological state of amotivation, apathy, and global absence of willpower. It presents with a challenging array of overlapping symptoms, making effective identification and treatment difficult. CASE PRESENTATION: We describe the first known report of an adolescent with a ventricular assist device who developed abulia following a left middle cerebral artery (MCA) stroke who responded successfully to treatment with olanzapine. DISCUSSION: The neurobiological etiology of abulia is still unclear but is postulated to be related to deficits in the dopaminergic reward circuitry in the frontal-subcortical-mesolimbic regions. There have been reports of poststroke patients with abulia being treated by modulating this dopamine circuitry and in some cases with short-term low-dose olanzapine. CONCLUSION: Further research is needed to develop a better understanding of the pathophysiology of abulia leading to more effective treatment algorithms including more specific diagnostic tools and effective pharmacological interventions.