Physical activity in the Fontan population: provider recommendations and patient actions.

BACKGROUND: Emerging evidence suggests that routine physical activity may improve exercise capacity, long-term outcomes, and quality of life in individuals with Fontan circulation. Despite this, it is unclear how active these individuals are and what guidance they receive from medical providers regarding physical activity. The aim of this study was to survey Fontan patients on personal physical activity behaviours and their cardiologist-directed physical activity recommendations to set a baseline for future targeted efforts to improve this. METHODS: An electronic survey assessing physical activity habits and cardiologist-directed guidance was developed in concert with content experts and patients/parents and shared via a social media campaign with Fontan patients and their families. RESULTS: A total of 168 individuals completed the survey. The median age of respondents was 10 years, 51% identifying as male. Overall, 21% of respondents spend > 5 hours per week engaged in low-exertion activity and only 7% spend > 5 hours per week engaged in high-exertion activity. In all domains questioned, pre-adolescents reported higher participation rates than adolescents. Nearly half (43%) of respondents reported that they do not discuss activity recommendations with their cardiologist. CONCLUSIONS: Despite increasing evidence over the last two decades demonstrating the benefit of exercise for individuals living with Fontan circulation, only a minority of patients report engaging in significant amounts of physical activity or discussing activity goals with their cardiologist. Specific, individualized, and actionable education needs to be provided to patients, families, and providers to promote and support regular physical activity in this patient population.

Validity of Wrist-Worn Activity Tracker Heart Rate Detection in Fontan Patients During Exercise.

PURPOSE: Physical activity and a healthy lifestyle play an essential role in optimizing long-term health in patients with Fontan physiology. Wrist-worn activity trackers may be useful in medically directed exercise programs for patients with Fontan physiology. The objective of this study was to measure the validity of Garmin and Fitbit activity tracker heart rate detection in patients with Fontan circulation when compared to electrocardiogram (ECG) during cardiopulmonary exercise testing (CPET). METHODS: 47 Fontan patients undergoing CPET for clinical indications were included and wore activity trackers during CPET. Heart rate via the activity tracker was collected at baseline, maximal exercise, and recovery. Patient heart rates, peak VO2, and peak respiratory exchange ratio (RER) were collected using standard CPET protocols and equipment. Heart rate at each time point was compared between the activity trackers and CPET ECG. RESULTS: Median age of participants was 17.1 years, 15.1 years since Fontan completion. Mean percent of predicted peak VO2 was 56.8%, z-score -3.2 with 61.7% of participants completing a maximal CPET (RER ≥ 1.09). Baseline oxygen saturation mean was 92.9%, 90.0% at maximal exercise. Activity trackers demonstrated mean absolute percentage error < 10% at most time points, comparable with other studies. Demographics, Fontan-associated comorbidities, and echocardiogram findings did not impact the accuracy. CONCLUSIONS: Consumer-oriented wrist-worn activity trackers show promising accuracy for heart rate monitoring in medically directed exercise programs for adolescents and young adults with Fontan physiology. Further validation across different exercise modalities is needed.

Developing the WE BEAT Well-Being Education Programme to foster resilience and build connection in paediatric heart disease.

BACKGROUND: The study of psychological well-being and related resilient outcomes is of increasing focus in cardiovascular research. Despite the critical importance of psychological well-being and related resilient outcomes in promoting optimal cardiac health, there have been very few psychological interventions directed towards children with heart disease. This paper describes the development and theoretical framework of the WE BEAT Wellbeing Education Program, a group-based psychoeducation and coping skills training intervention designed to improve psychological well-being and resilience in adolescents with paediatric heart disease. METHODS: Program development was informed by patient and family needs and input gathered via large, international survey methods as well as qualitative investigation, a theoretical framework, and related resilience intervention research. RESULTS: An overview of the WE BEAT intervention components and structure of the programme is provided. CONCLUSIONS: The WE BEAT Wellbeing Education Program was developed as one of the first resiliency-focused interventions in paediatric heart disease with an overall objective to foster positive psychological well-being and resilient outcomes through a health promotion and prevention lens in an accessible format while providing access to safe, peer-to-peer community building. Feasibility pilot results are forthcoming. Future directions include mobile app-based delivery and larger-scale efficacy and implementation trials.

The Effect of Udenafil on Heart Rate and Blood Pressure in Adolescents With the Fontan Circulation.

The Fontan Udenafil Exercise Longitudinal (FUEL) trial showed that treatment with udenafil was associated with improved exercise performance at the ventilatory anaerobic threshold in children with Fontan physiology. However, it is not known how the initiation of phosphodiesterase 5 inhibitor therapy affects heart rate and blood pressure in this population. These data may help inform patient selection and monitoring after the initiation of udenafil therapy. The purpose of this study is to evaluate the effects of udenafil on vital signs in the cohort of patients enrolled in the FUEL trial. This international, multicenter, randomized, double-blind, placebo-controlled trial of udenafil included adolescents with single ventricle congenital heart disease who had undergone Fontan palliation. Changes in vital signs (heart rate [HR], systolic [SBP] and diastolic blood pressure [DBP]) were compared both to subject baseline and between the treatment and the placebo groups. Additional exploratory analyses were performed to evaluate changes in vital signs for prespecified subpopulations believed to be most sensitive to udenafil initiation. Baseline characteristics were similar between the treatment and placebo cohorts (n = 200 for each). The groups demonstrated a decrease in HR, SBP, and DBP 2 hours after drug/placebo administration, except SBP in the placebo group. There was an increase in SBP from baseline to after 6-min walk test in the treatment and placebo groups, and the treatment group showed an increase in HR (87.4 ± 15.0 to 93.1 ± 19.4 beats/min, p <0.01) after exercise. When comparing changes from baseline to the 26-week study visit, small decreases in both SBP (-1.9 ± 12.3 mm Hg, p = 0.03) and DBP (-3.0 ± 9.6 mm Hg, p <0.01) were seen in the treatment group. There were no clinically significant differences between treatment and placebo group in change in HR or blood pressure in the youngest age quartile, lightest weight quartile, or those on afterload-reducing agents. In conclusion, initiation of treatment with udenafil in patients with Fontan circulation was not associated with clinically significant changes in vital signs, implying that for patients similar to those enrolled in the FUEL trial, udenafil can be started without the requirement for additional monitoring after initial administration.

ehealth technology in cardiac exercise therapeutics for pediatric patients with congenital and acquired heart conditions: a summary of evidence and future directions.

Many children and adolescents with congenital and acquired heart disease (CHD) are physically inactive and participate in an insufficient amount of moderate-to-vigorous intensity exercise. Although physical activity (PA) and exercise interventions are effective at improving short- and long-term physiological and psychosocial outcomes in youth with CHD, several barriers including resource limitations, financial costs, and knowledge inhibit widespread implementation and dissemination of these beneficial programs. New and developing eHealth, mHealth, and remote monitoring technologies offer a potentially transformative and cost-effective solution to increase access to PA and exercise programs for youth with CHD, yet little has been written on this topic. In this review, a cardiac exercise therapeutics (CET) model is presented as a systematic approach to PA and exercise, with assessment and testing guiding three sequential PA and exercise intervention approaches of progressive intensity and resource requirements: (1) PA and exercise promotion within a clinical setting; (2) unsupervised exercise prescription; and (3) medically supervised fitness training intervention (i.e., cardiac rehabilitation). Using the CET model, the goal of this review is to summarize the current evidence describing the application of novel technologies within CET in populations of children and adolescents with CHD and introduce potential future applications of these technologies with an emphasis on improving equity and access to patients in low-resource settings and underserved communities.

EKG Abnormalities in a Youth Athlete Following COVID-19: It's Not Always Myocarditis!

COVID-19 associated myocarditis following mild infections is rare while incidental findings may be more common. A young athlete fully recovered from a mild COVID-19 infection presented with inferolateral T-wave inversions and left ventricular hypertrophy on imaging. Exercise testing aided in correctly diagnosing the patient with masked systolic hypertension.

Angiotensin-Converting Enzyme Inhibitor Prescription for Patients With Single Ventricle Physiology Enrolled in the NPC-QIC Registry.

Background The routine use of angiotensin-converting enzyme inhibitors (ACEI) during palliation of hypoplastic left heart syndrome is controversial. We sought to describe ACEI prescription in the interstage between stage 1 palliation (stage I Norwood procedure) discharge and stage 2 palliation (stage II superior cavopulmonary anastomosis procedure) admission using the NPC-QIC (National Pediatric Cardiology Quality Improvement Collaborative) registry. Methods and Results Analysis of all patients (n=2180) enrolled in NPC-QIC from 2008 to 2016 included preoperative anatomy, risk factors, and echocardiographic data. ACEI were prescribed at stage I Norwood procedure discharge in 38% of patients. ACEI prescription declined from 2011 to 2016 compared with pre-2010 (36.8% versus 45%; P=0.005) with significant variation across centers (range 7-100%; P<0.001) and decreased prescribing rates associated with increased center volume (P=0.004). There was no difference in interstage mortality (P=0.662), change in atrioventricular valve regurgitation (P=0.101), or change in ventricular dysfunction (P=0.134) between groups. In multivariable analysis of all patients, atrioventricular septal defect (odds ratio [OR], 1.84; 95% CI, 1.28-2.65) or double outlet right ventricle (OR, 1.47; CI, 1.02-2.11), and preoperative mechanical ventilation (OR, 1.37; 95% CI, 1.12-1.68) were associated with increased ACEI prescription. In multivariable analysis of patients with complete echocardiographic data (n=812), ACEI prescription was more common with at least moderate atrioventricular valve regurgitation (OR, 1.88; 95% CI, 1.22-2.31). Conclusions ACEI prescription remains common in the interstage despite limited evidence of benefit. ACEI prescription is associated with preoperative mechanical ventilation, double outlet right ventricle, and atrioventricular valve regurgitation with marked inter-center variation. ACEI prescription is not associated with reduction in mortality, ventricular dysfunction, or atrioventricular valve regurgitation during the interstage.

Reducing Interdisciplinary Communication Failures Through Secure Text Messaging: A Quality Improvement Project.

INTRODUCTION: Interdisciplinary communication failures contribute to medical mistakes and adverse events. At our institution, provider communication previously occurred through unidirectional pager systems. We utilized quality improvement methodology to (1) implement a secure text messaging system for providers on a pediatric ward and (2) evaluate its impact on communication failures. We aimed to reduce potential communication failures between providers by > 25% within 1 month. METHODS: Implementation of secure text messaging occurred via Plan-Do-Study-Act cycles focused on education, feedback, and electronic health record interventions. We collected pager data before implementation and both pager and secure text messaging data after intervention. Potential communication failures were identified a priori through manual review of the messaging data to capture lack of closed-loop communication. A run chart was used to track daily potential communication failures and total communication volumes. RESULTS: Before implementation of secure text messaging, the median daily potential communication failure rate was 5.5%. Usage of secure text messaging increased after implementation, representing 3.5 of 7.2 communications per patient-day. Paging communications decreased from 4.2 to 3.7 per patient-day. Potential communication failures decreased to a median daily rate of 2.2%, representing a 59% reduction in communication failures. CONCLUSION: Implementation of secure text messaging using quality improvement methods resulted in a significant reduction in potential communication failures between residents and nurses. Future interventions will be aimed at maintaining and augmenting providers' use of secure text messaging to ensure the potential for communication failure remains low.

Longitudinal Health Care Cost in Hypoplastic Left Heart Syndrome Palliation.

Management of hypoplastic left heart syndrome (HLHS) is resource intensive. Heath care systems are pressured to provide value to patients by improving outcomes while decreasing costs. A single-center retrospective cohort of infants with HLHS who underwent Norwood procedure or hybrid Norwood from 2004 to 2014 and survived to first outpatient follow up were studied. The primary outcome was total cost through 12 months with a sub-analysis of patients with 60 months of data. Costs were calculated using internal cost accounting system and reported by cost center. Of the 152 HLHS patients identified, 69 met inclusion criteria. Stage I hospitalization (n = 69), with a median length of stay 34 days [interquartile range (IQR) 24-58 days], resulted in a median cost of $203,817 (IQR $136,236-272,453). Of survivors at 12 months (n = 55), the median cost was $369,393 (IQR $216,289-594,038) generated in part by a median of 67 (40-126 days) hospitalized days during that year. A subgroup analysis of patients who reached 60 months of age (n = 29) demonstrated a median total cost of $391,812 (IQR $293,801-577,443) and a median of 74 lifetime hospitalized days (IQR 58-116 days). High cost centers included intensive care (41%), non-ICU hospital (17%), operative services (11%), catheterization lab (9%), and pharmacy (9%). Using multiple regression analysis, significant drivers of cost included reoperation, length of hospitalization, low birthweight, and use of ECMO. Costs related to HLHS management are driven both by care-related complications such as surgical re-intervention and patient factors such as low birth weight.

Specificity of ARGONAUTE7-miR390 interaction and dual functionality in TAS3 trans-acting siRNA formation.

Trans-acting siRNA form through a refined RNAi mechanism in plants. miRNA-guided cleavage triggers entry of precursor transcripts into an RNA-DEPENDENT RNA POLYMERASE6 pathway, and sets the register for phased tasiRNA formation by DICER-LIKE4. Here, we show that miR390-ARGONAUTE7 complexes function in distinct cleavage or noncleavage modes at two target sites in TAS3a transcripts. The AGO7 cleavage, but not the noncleavage, function could be provided by AGO1, the dominant miRNA-associated AGO, but only when AGO1 was guided to a modified target site through an alternate miRNA. AGO7 was highly selective for interaction with miR390, and miR390 in turn was excluded from association with AGO1 due entirely to an incompatible 5' adenosine. Analysis of AGO1, AGO2, and AGO7 revealed a potent 5' nucleotide discrimination function for some, although not all, ARGONAUTEs. miR390 and AGO7, therefore, evolved as a highly specific miRNA guide/effector protein pair to function at two distinct tasiRNA biogenesis steps.