Respiratory performance in Duchenne muscular dystrophy: Clinical manifestations and lessons from animal models.

Duchenne muscular dystrophy (DMD) is a fatal genetic neuromuscular disease. Lack of dystrophin in skeletal muscles leads to intrinsic weakness, injury, subsequent degeneration and fibrosis, decreasing contractile function. Dystropathology eventually presents in all inspiratory and expiratory muscles of breathing, severely curtailing their critical function. In people with DMD, premature death is caused by respiratory or cardiac failure. There is an urgent need to develop therapies that improve quality of life and extend life expectancy in DMD. Surprisingly, there is a dearth of information on respiratory control in animal models of DMD, and respiratory outcome measures are often limited or absent in clinical trials. Characterization of respiratory performance in murine and canine models has revealed extensive remodelling of the diaphragm, the major muscle of inspiration. However, significant compensation by extradiaphragmatic muscles of breathing is evident in early disease, contributing to preservation of peak respiratory system performance. Loss of compensation afforded by accessory muscles in advanced disease is ultimately associated with compromised respiratory performance. A new and potentially more translatable murine model of DMD, the D2.mdx mouse, has recently been developed. Respiratory performance in D2.mdx mice is yet to be characterized fully. However, based on histopathological features, D2.mdx mice might serve as useful preclinical models, facilitating the testing of new therapeutics that rescue respiratory function. This review summarizes the pathophysiological mechanisms associated with DMD both in humans and in animal models, with a focus on breathing. We consider the translational value of each model to human DMD and highlight the urgent need for comprehensive characterization of breathing in representative preclinical models to better inform human trials.

Impact of disability-based discrimination in healthcare on parents of children with medical complexity.

AIM: To qualitatively assess the impact of disability-based discrimination in healthcare on the parents of children with medical complexity (CMC). METHOD: In this qualitative study, we conducted in-depth, semi-structured interviews with the parents of CMC. Data collection and analysis occurred iteratively; constant comparison methods were used to identify themes describing the impact of disability-based discrimination in pediatric healthcare on the parents of CMC. RESULTS: Thirty participants from 15 US states were interviewed. Four themes were developed regarding the impact of disability-based discrimination in healthcare on parents. The themes were: (1) discrimination leads to a loss of trust in healthcare providers; (2) discrimination increases the burden of caregiving; (3) discrimination impacts parental well-being; and (4) racism and poverty-based discrimination amplifies disability-based discrimination. INTERPRETATION: The experience of discrimination toward their child results in loss of trust and therapeutic relationship between provider and parent, causes increased burden to the family, and contributes to decreased parental well-being. These experiences are magnified in minoritized families and in families perceived to have a lower socioeconomic status based on insurance type.

Parents' quality of life and health after treatment decision for a fetus with severe congenital heart defect.

PURPOSE: This exploratory study examines differences in parents' quality of life by treatment decision and the child's survival outcome in the context of life-threatening congenital heart disease (CHD). DESIGN AND METHODS: Parents of a fetus or neonate diagnosed with severe CHD enrolled in the observational control group of a clinical trial (NCT04437069) and completed quality of life (i.e., contact with clinicians, social support, partner relationship, state of mind), mental and physical health survey measures. Comparisons were made between parents who chose comfort-directed care or surgery and between those whose child did and did not survive. RESULTS: Parents who chose surgery and their child did not survive reported the most contact with their clinicians. Parents who chose comfort-directed care reported lower social support than parents who chose surgery and their child did not survive as well as poorer state of mind compared to parents who chose surgery. CONCLUSIONS: Some aspects of parents' quality of life differed based on their treatment decision. Parents who choose comfort-directed care are vulnerable to some negative outcomes. PRACTICE IMPLICATIONS: Decision support tools and bereavement resources to assist parents with making and coping with a complex treatment decision is important for clinical care.

Parents' decision-making for their foetus or neonate with a severe congenital heart defect.

BACKGROUND: Parents who receive a diagnosis of a severe, life-threatening CHD for their foetus or neonate face a complex and stressful decision between termination, palliative care, or surgery. Understanding how parents make this initial treatment decision is critical for developing interventions to improve counselling for these families. METHODS: We conducted focus groups in four academic medical centres across the United States of America with a purposive sample of parents who chose termination, palliative care, or surgery for their foetus or neonate diagnosed with severe CHD. RESULTS: Ten focus groups were conducted with 56 parents (Mage = 34 years; 80% female; 89% White). Results were constructed around three domains: decision-making approaches; values and beliefs; and decision-making challenges. Parents discussed varying approaches to making the decision, ranging from relying on their "gut feeling" to desiring statistics and probabilities. Religious and spiritual beliefs often guided the decision to not terminate the pregnancy. Quality of life was an important consideration, including how each option would impact the child (e.g., pain or discomfort, cognitive and physical abilities) and their family (e.g., care for other children, marriage, and career). Parents reported inconsistent communication of options by clinicians and challenges related to time constraints for making a decision and difficulty in processing information when distressed. CONCLUSION: This study offers important insights that can be used to design interventions to improve decision support and family-centred care in clinical practice.

Provider insights on shared decision-making with families affected by CHD.

BACKGROUND AND OBJECTIVES: Little data exist on provider perspectives about counselling and shared decision-making for complex CHD, ways to support and improve the process, and barriers to effective communication. The goal of this qualitative study was to determine providers' perspectives regarding factors that are integral to shared decision-making with parents faced with complex CHD in their fetus or newborn; and barriers and facilitators to engaging in effective shared decision-making. METHODS: We conducted semi-structured interviews with providers from different areas of practice who care for fetuses and/or children with CHD. Providers were recruited from four geographically diverse centres. Interviews were recorded, transcribed, and analysed for key themes using an open coding process with a grounded theory approach. RESULTS: Interviews were conducted with 31 providers; paediatric cardiologists (n = 7) were the largest group represented, followed by nurses (n = 6) and palliative care providers (n = 5). Key barriers to communication with parents that providers identified included variability among providers themselves, factors that influenced parental comprehension or understanding, discrepant expectations, circumstantial barriers, and trust/relationship with providers. When discussing informational needs of parents, providers focused on comprehensive short- and long-term outcomes, quality of life, and breadth and depth that aligned with parental goals and needs. In discussing resources to support shared decision-making, providers emphasised the need for comprehensive, up-to-date information that was accessible to parents of varying situations and backgrounds. CONCLUSIONS: Provider perspectives on decision-making with families with CHD highlighted key communication issues, informational priorities, and components of decision support that can enhance shared decision-making.

Don't Risk It. Older Adults Perceive Fewer Future Opportunities and Avoid Social Risk Taking.

Drawing from life-span psychology, we conducted two studies to test perceptions of time left in the future as an underlying mechanism for age differences in self-reported social risk taking. Study 1 included 120 younger (25-35 years) and 119 older (60-91 years) community-dwelling adults. Study 2 included 439 participants (18-85 years) mostly recruited from Amazon Mechanical Turk. In both studies, older age was associated with rating a lower likelihood of social risk taking (e.g., speaking about an unpopular issue) and perceiving the future as holding fewer future opportunities and being more limited. Perceptions of fewer future opportunities with aging statistically mediated age-related declines in social risk taking. Findings highlight motivational factors as key for understanding age differences in social risk taking. Implications of age differences in social risk taking on factors related to well-being, such as social support and strain, are discussed.

Knowing you are there makes the difference: perceived social support, preferences for using support, and health.

Using cluster analysis, we investigated whether perceived social support and individual differences in preferences to use support combined to form distinct profiles. Self-report data were collected from U.S. adults (N = 454; aged 40-90, Mage = 55.37, SD = 9.73). Four profiles were identified: disengaged, interpersonally connected, isolated independent, and connected independent. Profiles characterized by high perceived support were associated with better overall health, even among those who preferred not to use support; men and those not married or cohabiting were less likely to be in these profiles. Implications for understanding associations between social support and health and the identification of at-risk groups are discussed.

Lack of IL-1 Receptor Signaling Reduces Spontaneous Airway Eosinophilia in Juvenile Mice with Muco-Obstructive Lung Disease.

Previous studies demonstrated spontaneous type 2 airway inflammation with eosinophilia in juvenile Scnn1b (sodium channel, non-voltage-gated 1, ß-subunit)-transgenic (Scnn1b-Tg) mice with muco-obstructive lung disease. IL-1 receptor (IL-1R) signaling has been implicated in allergen-driven airway disease; however, its role in eosinophilic inflammation in muco-obstructive lung disease remains unknown. In this study, we examined the role of IL-1R signaling in the development of airway eosinophilia and type 2 inflammation in juvenile Scnn1b-Tg mice. We determined effects of genetic deletion of Il1r1 (IL-1 receptor type I) on eosinophil counts, transcript levels of key type 2 cytokines, markers of eosinophil activation and apoptosis, and tissue morphology in lungs of Scnn1b-Tg mice at different time points during neonatal development. Furthermore, we measured endothelial surface expression of intercellular adhesion molecule 1 (ICAM-1), an integrin involved in eosinophil transendothelial migration, and determined effects of eosinophil depletion using an anti-IL-5 antibody on lung morphology. Lack of IL-1R reduced airway eosinophilia and structural lung damage, but it did not reduce concentrations of type 2 cytokines and associated eosinophil activation in Scnn1b-Tg mice. Structural lung damage in Scnn1b-Tg mice was also reduced by eosinophil depletion. Lack of IL-1R was associated with reduced expression of ICAM-1 on lung endothelial cells and reduced eosinophil counts in lungs from Scnn1b-Tg mice. We conclude that IL-1R signaling is implicated in airway eosinophilia independent of type 2 cytokines in juvenile Scnn1b-Tg mice. Our data suggest that IL-1R signaling may be relevant in the pathogenesis of eosinophilic airway inflammation in muco-obstructive lung diseases, which may be mediated in part by ICAM-1-dependent transmigration of eosinophils into the lungs.

Targeting of cathepsin S reduces cystic fibrosis-like lung disease.

Cathepsin S (CatS) is upregulated in the lungs of patients with cystic fibrosis (CF). However, its role in CF lung disease pathogenesis remains unclear.In this study, ß-epithelial Na+ channel-overexpressing transgenic (ßENaC-Tg) mice, a model of CF-like lung disease, were crossed with CatS null (CatS-/-) mice or treated with the CatS inhibitor VBY-999.Levels of active CatS were elevated in the lungs of ßENaC-Tg mice compared with wild-type (WT) littermates. CatS-/-ßENaC-Tg mice exhibited decreased pulmonary inflammation, mucus obstruction and structural lung damage compared with ßENaC-Tg mice. Pharmacological inhibition of CatS resulted in a significant decrease in pulmonary inflammation, lung damage and mucus plugging in the lungs of ßENaC-Tg mice. In addition, instillation of CatS into the lungs of WT mice resulted in inflammation, lung remodelling and upregulation of mucin expression. Inhibition of the CatS target, protease-activated receptor 2 (PAR2), in ßENaC-Tg mice resulted in a reduction in airway inflammation and mucin expression, indicating a role for this receptor in CatS-induced lung pathology.Our data indicate an important role for CatS in the pathogenesis of CF-like lung disease mediated in part by PAR2 and highlight CatS as a therapeutic target.

Variations in Decision-Making Profiles by Age and Gender: A Cluster-Analytic Approach.

Using cluster-analysis, we investigated whether rational, intuitive, spontaneous, dependent, and avoidant styles of decision making (Scott & Bruce, 1995) combined to form distinct decision-making profiles that differed by age and gender. Self-report survey data were collected from 1,075 members of RAND's American Life Panel (56.2% female, 18-93 years, Mage = 53.49). Three decision-making profiles were identified: affective/experiential, independent/self-controlled, and an interpersonally-oriented dependent profile. Older people were less likely to be in the affective/experiential profile and more likely to be in the independent/self-controlled profile. Women were less likely to be in the affective/experiential profile and more likely to be in the interpersonally-oriented dependent profile. Interpersonally-oriented profiles are discussed as an overlooked but important dimension of how people make important decisions.

Are people with diabetes getting the support they need? Deficits between support desired and received from family and friends relates to poorer health.

OBJECTIVE: To determine the mismatch of desired support versus support received and to evaluate the impact of these mismatches on health outcomes of people with diabetes. METHODS: This cross-sectional study is a secondary data analysis of medical record and survey data of participants with Type 1 and Type 2 diabetes from a diabetes care and education program. Biophysical metrics included HbA1c, body mass index, systolic blood pressure, diastolic blood pressure, triglycerides, and high- and low-density lipoproteins. Psychosocial and self-care survey outcomes included diabetes distress, diabetes self-care, and diabetes self-efficacy. Support mismatch was a difference score (support desired-support received). Descriptive statistics were computed for demographics, clinical characteristics, and primary outcomes. Multiple linear regressions were computed. RESULTS: The percentage of participants experiencing support mismatch (surplus/deficits) across six domains was: 15%/27% (foot care), 22%/24% (take medicine), 24%/23% (test blood sugar), 21%/29% (physical activity), and 18%/34% (follow meal plan). Greater support deficits were associated with higher triglyceride levels, increased diabetes distress, and lower diabetes self-efficacy. CONCLUSIONS: Findings indicate that greater support deficits can be a risk factor for some poorer physical and psychosocial health outcomes. PRACTICE IMPLICATIONS: Interventions to facilitate functional supportive behaviors are an avenue for future research and clinical practice.

Perceived Disability-Based Discrimination in Health Care for Children With Medical Complexity.

BACKGROUND AND OBJECTIVES: Disability-based discrimination in health care can lead to low quality of care, limited access to care, and negative health consequences. Yet, little is known regarding the experiences of disability-based discrimination in health care for children with medical complexity and disability. An understanding of disability-based discrimination in pediatrics is needed to drive change and improve care. METHODS: We conducted in-depth, semistructured interviews with caregivers of children with medical complexity and disability. Participants were purposefully recruited through national advocacy and research networks. Interviews were conducted via video conferencing, recorded, and transcribed. Data collection and analysis occurred iteratively. An inductive thematic analysis approach with constant comparison methods was used to identify themes that form a conceptual framework of disability-based discrimination in health care. RESULTS: Thirty participants from diverse backgrounds were interviewed. Six themes emerged, forming a conceptual framework of disability-based discrimination in health care. Three themes described drivers of discrimination: lack of clinician knowledge, clinician apathy, and clinician assumptions. Three themes described manifestations of discrimination: limited accessibility to care, substandard care, and dehumanization. CONCLUSIONS: Children with medical complexity may face disability-based discrimination in health care. Themes describing the drivers and manifestations of discrimination offer a conceptual framework of disability-based discrimination. Understanding the drivers and acknowledging perceived manifestations can provide insight into improving patient care for children with disabilities.

Parents' Psychological and Decision-Making Outcomes following Prenatal Diagnosis with Complex Congenital Heart Defect: An Exploratory Study.

Background. Parents with a fetus diagnosed with a complex congenital heart defect (CHD) are at high risk of negative psychological outcomes. Purpose. To explore whether parents' psychological and decision-making outcomes differed based on their treatment decision and fetus/neonate survival status. Methods. We prospectively enrolled parents with a fetus diagnosed with a complex, life-threatening CHD from September 2018 to December 2020. We tested whether parents' psychological and decision-making outcomes 3 months posttreatment differed by treatment choice and survival status. Results. Our sample included 23 parents (average Age[years]: 27 ± 4, range = 21-37). Most were women (n = 18), non-Hispanic White (n = 20), and married (n = 21). Most parents chose surgery (n = 16), with 11 children surviving to the time of the survey; remaining parents (n = 7) chose comfort-directed care. Parents who chose comfort-directed care reported higher distress (x¯ = 1.51, s = 0.75 v. x¯ = 0.74, s = 0.55; Mdifference = 0.77, 95% confidence interval [CI], 0.05-1.48) and perinatal grief (x¯ = 91.86, s = 22.96 v. x¯ = 63.38, s = 20.15; Mdifference = 27.18, 95% CI, 6.20-48.16) than parents who chose surgery, regardless of survival status. Parents who chose comfort-directed care reported higher depression (x¯ = 1.64, s = 0.95 v. x¯ = 0.65, s = 0.49; Mdifference = 0.99, 95% CI, 0.10-1.88) than parents whose child survived following surgery. Parents choosing comfort-directed care reported higher regret (x¯ = 26.43, s = 8.02 v. x¯ = 5.00, s = 7.07; Mdifference = 21.43, 95% CI, 11.59-31.27) and decisional conflict (x¯ = 20.98, s = 10.00 v. x¯ = 3.44, s = 4.74; Mdifference = 17.54, 95% CI; 7.75-27.34) than parents whose child had not survived following surgery. Parents whose child survived following surgery reported lower grief (Mdifference = -19.71; 95% CI, -39.41 to -0.01) than parents whose child had not. Conclusions. The results highlight the potential for interventions and care tailored to parents' treatment decisions and outcomes to support parental coping and well-being. Highlights: Question: Do the psychological and decision-making outcomes of parents differ based on their treatment decision and survival outcome following prenatal diagnosis with complex CHD?Findings: In this exploratory study, parents who decided to pursue comfort-directed care after a prenatal diagnosis reported higher levels of psychological distress and grief as well as higher decisional conflict and regret than parents who decided to pursue surgery.Meaning: The findings from this exploratory study highlight potential differences in parents' psychological and decision-making outcomes following a diagnosis of complex CHD for their fetus, which appear to relate to the treatment approach and the treatment outcome and may require tailoring of psychological and decision support.

Preparing Patients with Early Stage Prostate Cancer to Participate in Clinical Appointments Using a Shared Decision Making Training Video.

BACKGROUND: Rates of shared decision making (SDM) are relatively low in early stage prostate cancer decisions, as patients' values are not well integrated into a preference-sensitive treatment decision. The study objectives were to develop a SDM training video, measure usability and satisfaction, and determine the effect of the intervention on preparing patients to participate in clinical appointments. METHODS: A randomized controlled trial was conducted to compare a plain-language decision aid (DA) to the DA plus a patient SDM training video. Patients with early stage prostate cancer completed survey measures at baseline and after reviewing the intervention materials. Survey items assessed patients' knowledge, beliefs related to SDM, and perceived readiness/intention to participate in their upcoming clinical appointment. RESULTS: Of those randomized to the DA + SDM video group, most participants (91%) watched the video and 93% would recommend the video to others. Participants in the DA + SDM video group, compared to the DA-only group, reported an increased desire to participate in the decision (mean = 3.65 v. 3.39, P < 0.001), less decision urgency (mean = 2.82 v. 3.39, P < 0.001), and improved self-efficacy for communicating with physicians (mean = 4.69 v. 4.50, P = 0.05). These participants also reported increased intentions to seek a referral from a radiation oncologist (73% v. 51%, P = 0.004), to take notes (mean = 3.23 v. 2.86, P = 0.004), and to record their upcoming appointments (mean = 1.79 v. 1.43, P = 0.008). CONCLUSIONS: A novel SDM training video was accepted by patients and changed several measures associated with SDM. This may be a scalable, cost-effective way to prepare patients with early stage prostate cancer to participate in their clinical appointments.[Box: see text].

Two Specialists, Two Recommendations: Discordance Between Urologists' & Radiation Oncologists' Prostate Cancer Treatment Recommendations.

OBJECTIVE: To examine the treatment recommendation patterns among urologists and radiation oncologists, the level of concordance or discordance between physician recommendations, and the association between physician recommendations and the treatment that patients received. METHOD: The study was a secondary analysis of data from a randomized clinical trial conducted November 2010 to April 2014 (NCT02053389). Eligible participants were patients from the trial who saw both specialists. The primary outcome was physician recommendations that were scored using an adapted version of the validated PhyReCS coding system. Secondary outcomes included concordance between physician recommendations and the treatment patients received. RESULTS: Participants were 108 patients (Mean age 61.9 years; range 43-82; 87% non-Hispanic White). Urologists were more likely to recommend surgery (79% of recommendations) and radiation oncologists were more likely to recommend radiation (68% of recommendations). Recommendations from the urologists and radiation oncologists were concordant for only 33 patients (30.6%). Most patients received a treatment that both physicians recommended (59%); however, 35% received a treatment that only one of their physicians recommended. When discordant, urologists more often recommended surgery and radiation oncologists recommended radiation and surgery as equally appropriate options. CONCLUSION: Urologists and radiation oncologists are more likely to differ than agree in their treatment recommendations for the same patients with clinically localized prostate cancer and more likely to favor treatment aligned with their specialty. Additional studies are needed to better understand how patients make decisions after meeting with two different specialists to inform the development of best practices within oncology clinics.

An explainable artificial intelligence approach for predicting cardiovascular outcomes using electronic health records.

Understanding the conditionally-dependent clinical variables that drive cardiovascular health outcomes is a major challenge for precision medicine. Here, we deploy a recently developed massively scalable comorbidity discovery method called Poisson Binomial based Comorbidity discovery (PBC), to analyze Electronic Health Records (EHRs) from the University of Utah and Primary Children's Hospital (over 1.6 million patients and 77 million visits) for comorbid diagnoses, procedures, and medications. Using explainable Artificial Intelligence (AI) methodologies, we then tease apart the intertwined, conditionally-dependent impacts of comorbid conditions and demography upon cardiovascular health, focusing on the key areas of heart transplant, sinoatrial node dysfunction and various forms of congenital heart disease. The resulting multimorbidity networks make possible wide-ranging explorations of the comorbid and demographic landscapes surrounding these cardiovascular outcomes, and can be distributed as web-based tools for further community-based outcomes research. The ability to transform enormous collections of EHRs into compact, portable tools devoid of Protected Health Information solves many of the legal, technological, and data-scientific challenges associated with large-scale EHR analyses.

Experiences of a Health System's Faculty, Staff, and Trainees' Career Development, Work Culture, and Childcare Needs During the COVID-19 Pandemic.

Importance: In March 2020, US public buildings (including schools) were shut down because of the COVID-19 pandemic, and 42% of US workers resumed their employment duties from home. Some shutdowns remain in place, yet the extent of the needs of US working parents is largely unknown. Objective: To identify and address the career development, work culture, and childcare needs of faculty, staff, and trainees at an academic medical center during a pandemic. Design, Setting, and Participants: For this survey study, between August 5 and August 20, 2020, a Qualtrics survey was emailed to all faculty, staff, and trainees at University of Utah Health, an academic health care system that includes multiple hospitals, community clinics, and specialty centers. Participants included 27 700 University of Utah Health faculty, staff, and trainees who received a survey invitation. Data analysis was performed from August to November 2020. Main Outcomes and Measures: Primary outcomes included experiences of COVID-19 and their associations with career development, work culture, and childcare needs. Results: A total of 5030 participants completed the entire survey (mean [SD] age, 40 [12] years); 3738 (75%) were women; 4306 (86%) were White or European American; 561 (11%) were Latino or Latina (of any race), Black or African American, American Indian, Alaska Native, and Native Hawaiian or Pacific Islander; and 301 (6%) were Asian or Asian American. Of the participants, 2545 (51%) reported having clinical responsibilities, 2412 (48%) had at least 1 child aged 18 years or younger, 3316 (66%) were staff, 791 (16%) were faculty, and 640 (13%) were trainees. Nearly one-half of parents reported that parenting (1148 participants [49%]) and managing virtual education for children (1171 participants [50%]) were stressors. Across all participants, 1061 (21%) considered leaving the workforce, and 1505 (30%) considered reducing hours. Four hundred forty-nine faculty (55%) and 397 trainees (60%) perceived decreased productivity, and 2334 participants (47%) were worried about COVID-19 impacting their career development, with 421 trainees (64%) being highly concerned. Conclusions and Relevance: In this survey of 5030 faculty, staff, and trainees of a US health system, many participants with caregiving responsibilities, particularly women, faculty, trainees, and (in a subset of cases) those from racial/ethnic groups that underrepresented in medicine, considered leaving the workforce or reducing hours and were worried about their career development related to the pandemic. It is imperative that medical centers support their employees and trainees during this challenging time.

Study protocol for a randomised clinical trial of a decision aid and values clarification method for parents of a fetus or neonate diagnosed with a life-threatening congenital heart defect.

INTRODUCTION: Parents who receive the diagnosis of a life-threatening, complex heart defect in their fetus or neonate face a difficult choice between pursuing termination (for fetal diagnoses), palliative care or complex surgical interventions. Shared decision making (SDM) is recommended in clinical contexts where there is clinical equipoise. SDM can be facilitated by decision aids. The International Patient Decision Aids Standards collaboration recommends the inclusion of values clarification methods (VCMs), yet little evidence exists concerning the incremental impact of VCMs on patient or surrogate decision making. This protocol describes a randomised clinical trial to evaluate the effect of a decision aid (with and without a VCM) on parental mental health and decision making within a clinical encounter. METHODS AND ANALYSIS: Parents who have a fetus or neonate diagnosed with one of six complex congenital heart defects at a single tertiary centre will be recruited. Data collection for the prospective observational control group was conducted September 2018 to December 2020 (N=35) and data collection for two intervention groups is ongoing (began October 2020). At least 100 participants will be randomised 1:1 to two intervention groups (decision aid only vs decision aid with VCM). For the intervention groups, data will be collected at four time points: (1) at diagnosis, (2) postreceipt of decision aid, (3) postdecision and (4) 3 months postdecision. Data collection for the control group was the same, except they did not receive a survey at time 2. Linear mixed effects models will assess differences between study arms in distress (primary outcome), grief and decision quality (secondary outcomes) at 3-month post-treatment decision. ETHICS AND DISSEMINATION: This study was approved by the University of Utah Institutional Review Board. Study findings have and will continue to be presented at national conferences and within scientific research journals. TRIAL REGISTRATION NUMBER: NCT04437069 (Pre-results).

Shared Decision Making in Cardiac Electrophysiology Procedures and Arrhythmia Management.

Shared decision making (SDM) has been advocated to improve patient care, patient decision acceptance, patient-provider communication, patient motivation, adherence, and patient reported outcomes. Documentation of SDM is endorsed in several society guidelines and is a condition of reimbursement for selected cardiovascular and cardiac arrhythmia procedures. However, many clinicians argue that SDM already occurs with clinical encounter discussions or the process of obtaining informed consent and note the additional imposed workload of using and documenting decision aids without validated tools or evidence that they improve clinical outcomes. In reality, SDM is a process and can be done without decision tools, although the process may be variable. Also, SDM advocates counter that the low-risk process of SDM need not be held to the high bar of demonstrating clinical benefit and that increasing the quality of decision making should be sufficient. Our review leverages a multidisciplinary group of experts in cardiology, cardiac electrophysiology, epidemiology, and SDM, as well as a patient advocate. Our goal is to examine and assess SDM methodology, tools, and available evidence on outcomes in patients with heart rhythm disorders to help determine the value of SDM, assess its possible impact on electrophysiological procedures and cardiac arrhythmia management, better inform regulatory requirements, and identify gaps in knowledge and future needs.

A systematic review of intensive outpatient care programs for high-need, high-cost patients.

Five percent of the patient population accounts for 50% of U.S. healthcare expenditures. High-need, high-cost patients are medically complex for numerous reasons, often including behavioral health needs. Intensive outpatient care programs (IOCPs) are emerging, innovative clinics which provide patient-centered care leveraging multidisciplinary teams. The overarching goals of IOCPs are to reduce emergency department visits and hospitalizations (and related costs), and improve care continuity and patient outcomes. The purpose of this review was to examine the effectiveness of IOCPs on multiple outcomes to inform clinical care. A systematic search of the literature was conducted to identify articles. Six studies were included that varied in rigor of research design, analysis, and measurement of outcomes. Most studies reported results on healthcare utilization (n = 4) and costs (n = 3), with fewer reporting results on patient-reported and health-related outcomes (n = 2). Overall, there were decreasing trends in emergency department visits and hospitalizations. However, results on healthcare utilization varied based on time of follow-up, with shorter follow-up times yielding more significant results. Two of the three studies that evaluated costs found significant reductions associated with IOCPs, and the third was cost-neutral. Two studies reported improvements in patient-reported outcomes (e.g., satisfaction, depression, and anxiety). Overall, these programs reported positive impacts on healthcare utilization and costs; however, few studies evaluated patient characteristics and behaviors (e.g., engagement in care) which may serve as key mechanisms of program effectiveness. Future research should examine patient characteristics, behaviors, and clinic engagement metrics to inform clinical practice.