Reliability and validity of the revised Rushton Moral Resilience Scale for healthcare workers.

AIM: To refine the Rushton Moral Resilience Scale (RMRS) by creating a more concise scale, improving the reliability, particularly of the personal integrity subscale and providing further evidence of validity. BACKGROUND: Healthcare workers are exposed to moral adversity in practice. When unable to preserve/restore their integrity, moral suffering ensues. Moral resilience is a resource that may mitigate negative consequences. To better understand mechanisms for doing so, a valid and reliable measurement tool is necessary. DESIGN: Cross-sectional survey. METHODS: Participants (N = 1297) had completed ≥1 items on the RMRS as part of the baseline survey of a larger longitudinal study. Item analysis, confirmatory factor analyses, reliability analyses (Cronbach's alpha), and correlations were used to establish reliability and validity of the revised RMRS. RESULTS: Item and confirmatory factor analysis were used to refine the RMRS from 21 to 16 items. The four-factor structure (responses to moral adversity, personal integrity, relational integrity and moral efficacy) demonstrated adequate fit in follow-up confirmatory analyses in the initial and hold-out sub-samples. All subscales and the total scale had adequate reliabilities (α ≥ 0.70). A higher-order factor analysis supports the computation of either subscale scores or a total scale score. Correlations of scores with stress, anxiety, depression and moral distress provide evidence of the scale's validity. Reliability of the personal integrity subscale improved. CONCLUSION AND IMPLICATIONS: The RMRS-16 demonstrates adequate reliability and validity, particularly the personal integrity subscale. Moral resilience is an important lever for reducing consequences when confronted with ethical challenges in practice. Improved reliability of the four subscales and having a shorter overall scale allow for targeted application and will facilitate further research and intervention development. PATIENT/PUBLIC CONTRIBUTION: Data came from a larger study of Canadian healthcare workers from multiple healthcare organizations who completed a survey about their experiences during COVID-19.

Organizational Impact on Healthcare Workers' Moral Injury During COVID-19: A Mixed-Methods Analysis.

OBJECTIVE: The aim of this study was to explore relationships between organizational factors and moral injury among healthcare workers and the impact of perceptions of their leaders and organizations during COVID-19. BACKGROUND: COVID-19 placed healthcare workers at risk for moral injury, which often involves feeling betrayed by people with authority and can impact workplace culture. METHODS: Secondary data from a Web-based survey of mid-Atlantic healthcare workers were analyzed using mixed methods. Data were synthesized using the Reina Trust & Betrayal Model. RESULTS: Fifty-five percent (n = 328/595) of respondents wrote comments. Forty-one percent (n = 134/328) of commenters had moral injury scores of 36 or higher. Three themes emerged: organizational infrastructure, support from leaders, and palliative care involvement. Respondents outlined organizational remedies, which were organized into 5 domains. CONCLUSIONS: Findings suggest healthcare workers feel trust was breached by their organizations' leaders during COVID-19. Further study is needed to understand intersections between organizational factors and moral injury to enhance trust within healthcare organizations.

Epilepsy and related challenges in children with COL4A1 and COL4A2 mutations: A Gould syndrome patient registry.

Recently, patient advocacy groups started using the name Gould syndrome to describe clinical features of COL4A1 and COL4A2 mutations. Gould syndrome is increasingly identified in genetic screening panels, and because it is a rare disease, there is a disproportionate burden on families to understand the disease and chart the course for clinical care. Among the chief concerns for caregivers of children with Gould syndrome are the challenges faced because of epilepsy, including severe manifestations such as infantile spasms. To document the concerns of the patient population, the Gould Syndrome Foundation established the Gould Syndrome Global Registry (GSGR). METHODS: The Gould Syndrome Foundation developed questions for the GSGR with iterative input from patients and caregivers. An institutional review board issued an exemption determination before data collection began. Participants were recruited through social media and clinician referrals. All participants consented electronically, and the data were collected and managed using REDCap electronic data capture tools. De-identified data representing responses received between October 2019 and February 2021 were exported and analyzed with IBM SPSS 27 using descriptive statistics (mean, standard deviation, frequency, range, and percent). RESULTS: Seventy families from twelve countries provided data for the registry, representing 100 affected people (40 adults and 60 children). This analysis represents a subanalysis of the 35 out of 60 children <=18 years of age who reported a history of seizures. Nearly half of these participants were diagnosed with infantile spasms. Participants with epilepsy frequently reported developmental delays (88.6%), stroke (60.0%), cerebral palsy (65.7%), and constipation (57.1%). Ten (28.6%) children use a feeding tube. Despite the fact that more than half of respondents reported stroke, only 34.3% reported ever receiving education on stroke recognition. CONCLUSION: Here we describe the development and deployment of the first global registry for individuals and family members with Gould syndrome, caused by mutations in COL4A1 and COL4A2. It is important for pediatric neurologists to have access to resources to provide families upon diagnosis. Specifically, all families with Gould Syndrome must have access to infantile spasms awareness and stroke education materials. The Gould Syndrome Foundation is planning several improvements to this patient registry which will encourage collaboration and innovation for the benefit of people living with Gould syndrome.

Dental health in persons with disability.

Systemic and structural barriers limit dental health for individuals with special healthcare needs (SHCN), who have poorer dental hygiene, higher rates of dental disorders, and less access to oral care. We aimed to understand these barriers directly from the patient and caregiver population as well as review the literature on oral health of individuals with SHCN. We reviewed the literature on individuals and caregivers of those with SHCN to identify barriers to dental healthcare faced by these individuals. We focused on clinical and educational interventions to support clinicians treating this population. For the literature review, PubMed, Google, and Google Scholar were searched. We also relied upon the knowledge gained during the course of routine clinical care and patient advocacy activities. Published manuscripts were searched for the following Medical Subject Heading (MeSH) term: "Dental Care for Disabled" and the following subheading: pharmacology, adverse effects, ethics, methods, standards, and therapy. Relatively few dentists have formal training on caring for those with SHCN. Barriers faced by these individuals include accessibility, comorbidities, communication challenges, and barriers to home oral hygiene. Strong care coordination and communication between dentists, caregivers, and other providers is essential for positive outcomes. Our current dental healthcare system has failed to meet the needs of those with SHCN. The comfort and dignity of the patient are of paramount importance.

Barriers to transition from pediatric to adult care for patients with Dravet syndrome: A focus group study of caregivers.

Caregivers of individuals with intellectual and developmental disabilities and epilepsy such as Dravet syndrome (DS) must navigate a complex web of state and community services through the transition from child-centered to adult-oriented healthcare. This study examined barriers to successful transition from the caregivers' perspective. Primary caregivers of teenagers or adults with DS who had contemplated or completed transition to adult care were eligible. A three-week, asynchronous, web-based focus group was conducted on Facebook. Data were analyzed in an iterative process based on a Grounded Theory approach. Participants reviewed findings for accuracy. Transition success was defined by how well it ensured adequate care for the child when caregivers became unable to provide it. Existing transition programs were described as "not for our kids." All caregivers reported that transition programs began too late. Challenges to identifying suitable providers were formidable, with 71% of adult patients still being seen by pediatric neurologists. Many adult physicians lacked a general knowledge of DS, yet caregivers perceived that adult physicians were unwilling to listen to caregivers, and few were comfortable accommodating patients with intellectual disabilities and challenging behaviors. Community programs often excluded patients with DS, and rural healthcare disparities created additional barriers. Analysis produced recommendations for improving the transition process including the creation of a certified Transition Navigator position in the clinical setting. The limitations of this focus group analysis include possible selection bias, but our study identified key issues and pathways to improve the transition process for patients with DS and their caregivers.

Exploring National Nursing Readiness for a Radiological or Nuclear Incident: A Cross-Sectional Study.

INTRODUCTION: As the largest component of the United States health care workforce, nurses will play a critical role in radiological or nuclear disaster medical response. Despite this, the United States' schools of nursing are not currently providing radiation content (75% teach zero or <1 hour), and much of the current nursing workforce may not have received adequate response education and training. Nurses working in emergency departments and those who work at hospitals within the Radiation Injury Treatment Network will be relied on heavily, but little is known about whether these nurses possess the knowledge and skills needed to care for and protect patients after a radiation emergency. Current federal and state radiological/nuclear preparedness plans may be built on false assumptions of readiness, which would have serious implications for national preparedness and the National Health Security Strategy. The purpose of this study was to assess nurses' knowledge and skill in emergency radiological or nuclear response and determine their willingness to use mobile technology for education and training in response to a large-scale radiation event. METHODS: Descriptive cross-sectional survey of registered nurse members of the Emergency Nurses Association and/or those employed at Radiation Injury Treatment Network centers. RESULTS: Knowledge scores were low for all respondents. Prior attendance at a Radiation Emergency Medical Management course, use of online resources, and having a preparedness plan were associated with higher scores. Experience with a radiation emergency was associated with the highest score. Nurses are willing to use mobile technology during a radiological or nuclear disaster response. DISCUSSION: Key nurses may not possess adequate knowledge or clinical competence to participate in radiation response activities. The results of this assessment identified educational gaps and areas to strengthen nursing education and clinical skills.

The refractory epilepsy screening tool for Lennox-Gastaut syndrome (REST-LGS).

BACKGROUND: The complex clinical presentation and progression of Lennox-Gastaut syndrome (LGS) can complicate the accurate diagnosis of this severe, lifelong, childhood-onset epilepsy, often resulting in suboptimal treatment. The Refractory Epilepsy Screening Tool for LGS (REST-LGS) was developed to improve the identification of patients with LGS. METHODS: Using the Modified Delphi Consensus, a group of experts developed and tested the REST-LGS Case Report Form (CRF) comprising 8 criteria (4 major, 4 minor) considered potentially indicative of LGS. Diagnosis-blinded specialist and nonspecialist raters at 2 epilepsy centers applied the CRF to deidentified patient records, including 1:1 records of patients with drug-resistant epilepsy or confirmed LGS. Interrater reliability was measured by Cohen's κ. Diagnosis was then unblinded to reveal common criteria for LGS or drug-resistant epilepsy. Cronbach's α was used to measure internal consistency between raters for all criteria combined. RESULTS: Of 200 patients, 81% to 85% met 1 to 3 major criteria. At both sites, moderate (κ, 0.41-0.60) to good (κ, 0.61-0.80) agreement on most criteria was reached between expert and nonexpert raters. Unblinding revealed that most patients with LGS met 3 major and 2 to 3 minor criteria, while patients with drug-resistant epilepsy met ≤1 major and only 1 to 2 minor criteria. Cronbach's α of raters at both sites was 0.64. CONCLUSIONS: The combined number of major/minor criteria on the CRF may be particularly indicative of LGS. Therefore, the REST-LGS may be a valuable clinical tool in identifying patients requiring further diagnostic evaluation for LGS.

What do you think caused your ALS? An analysis of the CDC national amyotrophic lateral sclerosis patient registry qualitative risk factor data using artificial intelligence and qualitative methodology.

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is an incurable, progressive neurodegenerative disease with a significant health burden and poorly understood etiology. This analysis assessed the narrative responses from 3,061 participants in the Centers for Disease Control and Prevention's National ALS Registry who answered the question, "What do you think caused your ALS?" METHODS: Data analysis used qualitative methods and artificial intelligence (AI) using natural language processing (NLP), specifically, Bidirectional Encoder Representations from Transformers (BERT) to explore responses regarding participants' perceptions of the cause of their disease. RESULTS: Both qualitative and AI analysis methods revealed several, often aligned themes, which pointed to perceived causes including genetic, environmental, and military exposures. However, the qualitative analysis revealed detailed themes and subthemes, providing a more comprehensive understanding of participants' perceptions. Although there were areas of alignment between AI and qualitative analysis, AI's broader categories did not capture the nuances discovered using the more traditional, qualitative approach. The qualitative analysis also revealed that the potential causes of ALS were described within narratives that sometimes indicate self-blame and other maladaptive coping mechanisms. CONCLUSIONS: This analysis highlights the diverse range of factors that individuals with ALS consider as perceived causes for their disease. Understanding these perceptions can help clinicians to better support people living with ALS (PLWALS). The analysis highlights the benefits of using traditional qualitative methods to supplement or improve upon AI-based approaches. This rapidly evolving area of data science has the potential to remove barriers to accessing the rich narratives of people with lived experience.

Real-world use of the updated refractory epilepsy screening tool for Lennox-Gastaut syndrome.

OBJECTIVE: To evaluate the Refractory Epilepsy Screening Tool for Lennox-Gastaut Syndrome (REST-LGS) for real-world identification of LGS in adults and to develop a scoring system for the tool. METHODS: A retrospective chart review of adults with drug resistant epilepsy (DRE) and intellectual development disorder (IDD) was conducted by 2 primary care providers blinded to diagnosis. The REST-LGS was designed via the Modified Delphi Consensus and was previously validated. This tool consists of 8 criteria (4 major, 4 minor) considered indicative of LGS. To account for missing data in the earlier validation study and to evaluate applicability in a real-world setting, the REST-LGS was refined to include a scoring system in which major criteria were more heavily weighted than minor criteria, producing categories of "likely" (>11 points), "possible" (8-11 points), and "unlikely" (<8 points) LGS. Statistical analyses were descriptive. RESULTS: Of the 100 patients included in the analysis, data for slow spike-waves in electroencephalography and seizure onset age - both major REST-LGS criteria - were missing for 46% and 42% of patients, respectively. The majority of patients met 4 of the 8 REST-LGS criteria (cognitive impairment since childhood, 71%; persistent seizures despite a trial of ≥2 antiseizure medications, 65%; seizure onset before the age of 12 years, 57%; ≥2 seizure types, 56%). All 4 major criteria were met in 22 patients (22%). The percentages of patients considered "likely," "possible," or "unlikely" to have LGS were 26%, 30%, and 44%, respectively. Of the 74 patients without a previous LGS diagnosis, 42 (57%) were identified as "possible" or "likely" to have LGS using REST-LGS. SIGNIFICANCE: In this analysis, the validated REST-LGS was evaluated in a real-world setting. The majority of previously undiagnosed patients were identified via REST-LGS as "possible" or "likely" to have LGS. Extensive missing data highlights challenges of LGS diagnosis in adults. PLAIN LANGUAGE SUMMARY: There is a need to identify adult patients with Lennox-Gastaut syndrome (LGS) so they can receive appropriate treatment. The Refractory Epilepsy Screening Tool for LGS (REST-LGS) questionnaire was designed by experts to identify whether patients with seizures that are not controlled by medications may have LGS. In this study, 2 physicians completed the REST-LGS using charts for 100 patients who experience seizures not controlled by medications. Of the patients who were previously diagnosed as not having LGS, the majority were "likely" or "possible" to have LGS based on the REST-LGS; therefore, the REST-LGS can identify patients for further evaluation.

Regional and global biventricular function in pulmonary arterial hypertension: a cardiac MR imaging study.

PURPOSE: To determine whether chronic pulmonary arterial pressure (PAP) elevation affects regional biventricular function and whether regional myocardial function may be reduced in pulmonary arterial hypertension (PAH) patients with preserved global right ventricular (RV) function. MATERIALS AND METHODS: After informed consent, 35 PAH patients were evaluated with right heart catheterization and cardiac magnetic resonance (MR) imaging and compared with 13 healthy control subjects. Biventricular segmental, section, and mean ventricular peak systolic longitudinal strain (E(LL)), as well as left ventricular (LV) circumferential and RV tangential strains were compared between PAH patients and control subjects and correlated with global function and catheterization of the right heart indexes. Spearman ρ correlation with Bonferroni correction was used. Multiple linear regression analysis was performed to determine predictors for regional myocardial function. RESULTS: In the RV of PAH patients, longitudinal contractility was reduced at the basal, mid, and apical levels, and tangential contractility was reduced at the midventricular level. Mean RV E(LL) positively correlated with mean PAP (r = 0.62, P < .0014) and pulmonary vascular resistance index (PVRI) (r = 0.77, P < .0014). Mean PAP was a predictor of mean RV E(LL) (ß = .19, P = .005) in a multiple linear regression analysis. In the LV, reduced LV longitudinal and circumferential contractility were noted at the base. LV anteroseptal E(LL) positively correlated with increased mean PAP (r = 0.5, P = .03) and septal eccentricity index (r = 0.5, P = .01). In a subgroup of PAH patients with normal global RV function, significantly reduced RV longitudinal contractility was noted at basal and mid anterior septal insertions, as well as the mid anterior RV wall (P < .05 for all). CONCLUSION: In PAH patients, reduced biventricular regional function is associated with increased RV afterload (mean PAP and PVRI). Cardiac MR imaging helps identify regional RV dysfunction in PAH patients with normal global RV function. SUPPLEMENTAL MATERIAL: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.12111599/-/DC1.

The Mindful Ethical Practice and Resilience Academy: Sustainability of Impact.

BACKGROUND: Nurses face many ethical challenges, placing them at risk for moral distress and burnout and challenging their ability to provide safe, high-quality patient care. Little is known about the sustainability of interventions to address this problem. OBJECTIVE: To determine the long-term impact on acute care nurses of a 6-session experiential educational program called the Mindful Ethical Practice and Resilience Academy (MEPRA). METHODS: MEPRA includes facilitated discussion, role play, guided mindfulness and reflective practices, case studies, and high-fidelity simulation training to improve nurses' skills in mindfulness, resilience, and competence in confronting ethical challenges. A prospective, longitudinal study was conducted on the impact of the MEPRA curriculum at 2 hospitals in a large academic medical system. The study involved surveys of 245 nurses at baseline, immediately after the intervention, and 3 and 6 months after the intervention. RESULTS: The results of the intervention were generally sustained for months afterward. The most robust improvements were in ethical confidence, moral competence, resilience, work engagement, mindfulness, emotional exhaustion, depression, and anger. Some outcomes were not improved immediately after the intervention but were significantly improved at 3 months, including anxiety and empathy. Depersonalization and turnover intentions were initially reduced, but these improvements were not sustained at 6 months. CONCLUSIONS: Many MEPRA results were sustained at 3 and 6 months after conclusion of the initial foundational program. Some outcomes such as depersonalization and turnover intentions may benefit from boosters of the intervention or efforts to supplement the training by making organizational changes to the work environment.

A qualitative evaluation of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R) by the patient community: a web-based cross-sectional survey.

OBJECTIVE: The revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) is the most commonly used outcome measure in ALS studies. The aim of this study was to identify potential limitations of the ALSFRS-R from the perspective of people living with ALS and their caregivers. METHODS: A web-based survey was developed by investigators, people living with ALS, and their caregivers, and shared across social media. For each item, participants were asked, "Can you think of a situation where you might not be able to answer this item accurately or that your answer might not reflect your abilities?" Responses were divided into two categories: criticisms that could be addressed in a manual or issues with the items/responses that would require measure modification. RESULTS: 57 participants (72% participants with ALS, 28% caregivers) responded to at least one item question, of which 71.9% expressed concern about at least one item. The most frequently identified items were speech, walking, and cutting food. Common criticisms were: language used is of a medical literacy level too high; item is situational; difficult to distinguish the difference between response choices; and the structure and/or underlying assumptions of the item makes it difficult to answer. CONCLUSIONS: Several items of the ALSFRS-R were considered to inaccurately reflect the abilities of patients with ALS. The ALSFRS-R may need a revision to address these issues, preferably in co-development with people living with ALS and their caregivers, and/or alternate outcome measures should be considered for patients with ALS.

Leigh syndrome global patient registry: uniting patients and researchers worldwide.

BACKGROUND: Leigh Syndrome (LS) is a rare genetic neurometabolic disorder, that leads to the degeneration of the central nervous system and subsequently, early death. LS can be caused by over 80 mutations in mitochondrial or nuclear DNA. Patient registries are important for many reasons, such as studying the natural history of the disease, improving the quality of care, and understanding the healthcare burden. For rare diseases, patient registries are significantly important as patient numbers are small, and funding is limited. Cure Mito Foundation started a global patient registry for LS in September 2021 to identify and learn about the LS patient population, facilitate clinical trial recruitment, and unite international patients and researchers. Priorities were to allow researchers and industry partners to access data at no cost through a clear and transparent process, active patient engagement, and sharing of results back to the community. RESULTS: Patient registry platform, survey design, data analysis process, and patient recruitment strategies are described. Reported results include demographics, diagnostic information, symptom history, loss of milestones, disease management, healthcare utilization, quality of life, and caregiver burden for 116 participants. Results show a high disease burden, but a relatively short time to diagnosis. Despite the challenges faced by families impacted by Leigh syndrome, participants, in general, are described as having a good quality of life and caregivers are overall resilient, while also reporting a significant amount of stress. CONCLUSION: This registry provides a straightforward, no-cost mechanism for data sharing and contacting patients for clinical trials or research participation, which is important given the recruitment challenges for clinical trials for rare diseases. This is the first publication to present results from a global patient registry for Leigh Syndrome, with details on a variety of patient-specific and caregiver outcomes reported for the first time. Additionally, this registry is the first for any mitochondrial disease with nearly 70% of participants residing outside of the United States. Future efforts include continued publication of results and further collaboration with patients, industry partners, and researchers.

Pulmonary arterial hypertension: MR imaging-derived first-pass bolus kinetic parameters are biomarkers for pulmonary hemodynamics, cardiac function, and ventricular remodeling.

PURPOSE: To prospectively compare contrast material-enhanced (CE) magnetic resonance (MR) imaging-derived right-to-left ventricle pulmonary transit time (PTT), left ventricular (LV) full width at half maximum (FWHM), and LV time to peak (TTP) between patients with pulmonary arterial hypertension (PAH) and healthy volunteers and to correlate these measurements with survival markers in patients with PAH. MATERIALS AND METHODS: This HIPAA-compliant study received institutional review board approval. Written informed consent was obtained from all participants. Forty-three patients (32 with PAH [29 women; median age, 55.4 years], 11 with scleroderma but not PAH [seven women; median age, 58.9 years]) underwent right-sided heart catheterization and 3-T CE cardiac MR imaging. Eighteen age- and sex-matched healthy control subjects (12 women; median age, 51.7 years) underwent only CE MR imaging. A short-axis saturation-recovery gradient-echo section was acquired in the basal third of both ventricles, and right-to-left-ventricle PTT, LV FWHM, and LV TTP were calculated. Statistical analysis included Kruskal-Wallis test, Wilcoxon rank sum test, Spearman correlation coefficient, multiple linear regression analysis, and Lin correlation coefficient analysis. RESULTS: Patients had significantly longer PTT (median, 8.2 seconds; 25th-75th percentile, 6.9-9.9 seconds), FWHM (median, 8.2 seconds; 25th-75th percentile, 5.7-11.4 seconds), and TTP (median, 4.8 seconds; 25th-75th percentile, 3.9-6.5 seconds) than did control subjects (median, 6.4 seconds; 25th-75th percentile, 5.7-7.1 seconds; median, 5.2 seconds; 25th-75th percentile, 4.1-6.1 seconds; median, 3.2 seconds; 25th-75th percentile, 2.8-3.8 seconds, respectively; P < .01 for each) and subjects with scleroderma but not PAH (median, 6.5 seconds; 25th-75th percentile, 5.6-7.0 seconds; median, 5.0 seconds; 25th-75th percentile, 4.0-7.3 seconds; median, 3.6 seconds; 25th-75th percentile, 2.7-4.0 seconds, respectively; P < .02 for each). PTT, LV FWHM, and LV TTP correlated with pulmonary vascular resistance index (P < .01), right ventricular stroke volume index (P ≤ .01), and pulmonary artery capacitance (P ≤ .02). In multiple linear regression models, PTT, FWHM, and TTP were associated with mean pulmonary arterial pressure and cardiac index. CONCLUSION: CE MR-derived PTT, LV FWHM, and LV TTP are noninvasive compound markers of pulmonary hemodynamics and cardiac function in patients with PAH. Their predictive value for patient outcome warrants further investigation.

Cardiac MRI evaluation of hypertrophic cardiomyopathy: left ventricular outflow tract/aortic valve diameter ratio predicts severity of LVOT obstruction.

PURPOSE: To evaluate if left ventricular outflow tract/aortic valve (LVOT/AO) diameter ratio measured by cardiac magnetic resonance (CMR) imaging is an accurate marker for LVOT obstruction in patients with hypertrophic cardiomyopathy (HCM) compared to Doppler echocardiography. MATERIALS AND METHODS: In all, 92 patients with HCM were divided into three groups based on their resting echocardiographic LVOT pressure gradient (PG): <30 mmHg at rest (nonobstructive, n = 31), <30 mmHg at rest, >30 mmHg after provocation (latent, n = 29), and >30 mmHg at rest (obstructive, n = 32). The end-systolic dimension of the LVOT on 3-chamber steady-state free precession (SSFP) CMR was divided by the end diastolic aortic valve diameter to calculate the LVOT/AO diameter ratio. RESULTS: There were significant differences in the LVOT/AO diameter ratio among the three subgroups (nonobstructive 0.60 ± 0.13, latent 0.41 ± 0.16, obstructive 0.24 ± 0.09, P < 0.001). There was a strong linear inverse correlation between the LVOT/AO diameter ratio and the log of the LVOT pressure gradient (r = -0.84, P < 0.001). For detection of a resting gradient >30 mmHg, the LVOT/AO diameter ratio the area under the receiver operating characteristic (ROC) curve was 0.91 (95% confidence interval [CI] 0.85-0.97). For detection of a resting and/or provoked gradient >30 mmHg, the LVOT/AO diameter ratio area under the ROC curve was 0.90 (95% CI 0.84-0.96). CONCLUSION: The LVOT/AO diameter ratio is an accurate, reproducible, noninvasive, and easy to use CMR marker to assess LVOT pressure gradients in patients with HCM.

Moral Injury and Moral Resilience in Health Care Workers during COVID-19 Pandemic.

Background: The 2019 coronavirus (COVID-19) pandemic placed unprecedented strains on the U.S. health care system, putting health care workers (HCWs) at increased risk for experiencing moral injury (MI). Moral resilience (MR), the ability to preserve or restore integrity, has been proposed as a resource to mitigate the detrimental effects of MI among HCWs. Objectives: The objectives of this study were to investigate the prevalence of MI among HCWs, to identify the relationship among factors that predict MI, and to determine whether MR can act as buffer against it. Design: Web-based exploratory survey. Setting/Subjects: HCWs from a research network in the U.S. mid-Atlantic region. Measurements: Survey items included: our outcome, Moral Injury Symptoms Scale-Health Professional (MISS-HP), and predictors including demographics, items derived from the Rushton Moral Resilience Scale (RMRS), and ethical concerns index (ECI). Results: Sixty-five percent of 595 respondents provided COVID-19 care. The overall prevalence of clinically significant MI in HCWs was 32.4%; nurses reporting the highest occurrence. Higher scores on each of the ECI items were significantly positively associated with higher MI symptoms (p < 0.05). MI among HCWs was significantly related to the following: MR score, ECI score, religious affiliation, and having ≥20 years in their profession. MR was a moderator of the effect of years of experience on MI. Conclusions: HCWs are experiencing MI during the pandemic. MR offers a promising individual resource to buffer the detrimental impact of MI. Further research is needed to understand how to cultivate MR, reduce ECI, and understand other systems level factors to prevent MI symptoms in U.S. HCWs.

Right and left ventricular myocardial perfusion reserves correlate with right ventricular function and pulmonary hemodynamics in patients with pulmonary arterial hypertension.

PURPOSE: To evaluate the relationships of right ventricular (RV) and left ventricular (LV) myocardial perfusion reserves with ventricular function and pulmonary hemodynamics in patients with pulmonary arterial hypertension (PAH) by using adenosine stress perfusion cardiac magnetic resonance (MR) imaging. MATERIALS AND METHODS: This HIPAA-compliant study was institutional review board approved. Twenty-five patients known or suspected to have PAH underwent right heart catheterization and adenosine stress MR imaging on the same day. Sixteen matched healthy control subjects underwent cardiac MR imaging only. RV and LV perfusion values at rest and at adenosine-induced stress were calculated by using the Fermi function model. The MR imaging-derived RV and LV functional data were calculated by using dedicated software. Statistical testing included Kruskal-Wallis tests for continuous data, Spearman rank correlation tests, and multiple linear regression analyses. RESULTS: Seventeen of the 25 patients had PAH: 11 with scleroderma-associated PAH, and six with idiopathic PAH. The remaining eight patients had scleroderma without PAH. The myocardial perfusion reserve indexes (MPRIs) in the PAH group (median RV MPRI, 1.7 [25th-75th percentile range, 1.3-2.0]; median LV MPRI, 1.8 [25th-75th percentile range, 1.6-2.1]) were significantly lower than those in the scleroderma non-PAH (median RV MPRI, 2.5 [25th-75th percentile range, 1.8-3.9] [P = .03]; median LV MPRI, 4.1 [25th-75th percentile range, 2.6-4.8] [P = .0003]) and control (median RV MPRI, 2.9 [25th-75th percentile range, 2.6-3.6] [P < .01]; median LV MPRI, 3.6 [25th-75th percentile range, 2.7-4.1] [P < .01]) groups. There were significant correlations between biventricular MPRI and both mean pulmonary arterial pressure (mPAP) (RV MPRI: ρ = -0.59, Bonferroni P = .036; LV MPRI: ρ = -0.79, Bonferroni P < .002) and RV stroke work index (RV MPRI: ρ = -0.63, Bonferroni P = .01; LV MPRI: ρ = -0.75, Bonferroni P < .002). In linear regression analysis, mPAP and RV ejection fraction were independent predictors of RV MPRI. mPAP was an independent predictor of LV MPRI. CONCLUSION: Biventricular vasoreactivity is significantly reduced with PAH and inversely correlated with RV workload and ejection fraction, suggesting that reduced myocardial perfusion reserve may contribute to RV dysfunction in patients with PAH.

Myocardial delayed enhancement in pulmonary hypertension: pulmonary hemodynamics, right ventricular function, and remodeling.

OBJECTIVE: The purpose of this study was to assess predictors of MRI-identified septal delayed enhancement mass at the right ventricular (RV) insertion sites in relation to RV remodeling, altered regional mechanics, and pulmonary hemodynamics in patients with suspected pulmonary hypertension (PH). SUBJECTS AND METHODS: Thirty-eight patients with suspected PH were evaluated with right heart catheterization and cardiac MRI. Ten age- and sex-matched healthy volunteers acted as controls for MRI comparison. Septal delayed enhancement mass was quantified at the RV insertions. Systolic septal eccentricity index, global RV function, and remodeling indexes were quantified with cine images. Peak systolic circumferential and longitudinal strain at the sites corresponding to delayed enhancement were measured with conventional tagging and fast strain-encoded MRI acquisition, respectively. RESULTS: PH was diagnosed in 32 patients. Delayed enhancement was found in 31 of 32 patients with PH and in one of six patients in whom PH was suspected but proved absent (p = 0.001). No delayed enhancement was found in controls. Delayed enhancement mass correlated with pulmonary hemodynamics, reduced RV function, increased RV remodeling indexes, and reduced eccentricity index. Multiple linear regression analysis showed RV mass index was an independent predictor of total delayed enhancement mass (p = 0.017). Regional analysis showed delayed enhancement mass was associated with reduced longitudinal strain at the basal anterior septal insertion (r = 0.6, p < 0.01). Regression analysis showed that basal longitudinal strain remained an independent predictor of delayed enhancement mass at the basal anterior septal insertion (p = 0.02). CONCLUSION: In PH, total delayed enhancement burden at the RV septal insertions is predicted by RV remodeling in response to increased afterload. Local fibrosis mass at the anterior septal insertion is associated with reduced regional longitudinal contractility at the base.

Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension.

RATIONALE: Pulmonary arterial hypertension (PAH) related to systemic sclerosis (SSc) has a poorer prognosis compared with other forms of PAH for reasons that remain unexplained. OBJECTIVES: To identify risk factors of mortality in a well-characterized cohort of patients with PAH related to systemic sclerosis (SSc-PAH). METHODS: Seventy-six consecutive patients with SSc (64 women and 12 men; mean age 61 +/- 11 yr) were diagnosed with PAH by heart catheterization in a single center, starting in January 2000, and followed over time. Kaplan-Meier estimates were calculated and mortality risk factors were analyzed. MEASUREMENTS AND MAIN RESULTS: Forty (53%) patients were in World Health Organization functional class III or IV. Mean pulmonary artery pressure was 41 +/- 11 mm Hg, pulmonary vascular resistance (PVR) was 8.6 +/- 5.6 Wood units, and cardiac index was 2.4 +/- 0.7 L/min/m(2). Median follow-up time was 36 months, with 42 deaths observed. Survival estimates were 85%, 72%, 67%, 50%, and 36% at 1, 2, 3, 4, and 5 years, respectively. Multivariate analysis identified PVR (hazard ratio [HR], 1.10; 95% confidence interval [CI], 1.03-1.18; P < 0.01), stroke volume index (HR, 0.94; 95% CI, 0.89-0.99; P = 0.02), and pulmonary arterial capacitance (HR, 0.43; 95% CI, 0.20-0.91; P = 0.03) as strong predictors of survival. An estimated glomerular filtration rate less than 60 ml/min/1.73 m(2) portended a threefold risk of mortality. CONCLUSIONS: Our results suggest that specific components of right ventricular dysfunction and renal impairment contribute to increased mortality in SSc-PAH. Understanding the mechanisms of right ventricular dysfunction in response to increased afterload should lead to improved targeted therapy in these patients.

New Adopters of Telemedicine During the Coronavirus-19 Pandemic in Respondents to an Online Community Survey: The Case for Access to Remote Management Tools for Individuals with Chronic Obstructive Pulmonary Disease.

OBJECTIVES: To investigate telemedicine adoption, emergency department avoidance, and related characteristics of patients with chronic obstructive pulmonary disease (COPD) with and without exacerbations since the coronavirus 2019 (COVID-19) pandemic began. METHODS: We conducted the second of a series of online surveys via SurveyMonkey.com of people with COPD between May 1, 2020 and May 31, 2020. Frequency, percentage, and Fisher's exact test (2-sided) were calculated using SPSS version 26. RESULTS: More than half of respondents (157, 64%), indicated that they started using telemedicine in 2020. A total of 47% of respondents reported having had at least 1 exacerbation since January 1, 2020. Respondents who had at least 1 exacerbation in 2020 were more likely to start using telemedicine in 2020 than respondents who did not report any exacerbation in 2020 (75.7% versus 54.3%, p < 0.001). Respondents reporting a 2020 exacerbation indicated having a significantly higher avoidance of emergency health care since the pandemic began (27.8%) as compared to those who did not have an exacerbation in 2020 (10.1%), p < 0.001. CONCLUSIONS: In response to social distancing and other COVID-19 precautions, people with COPD are avoiding traditional, in-person health care environments and turning to telemedicine to prevent and manage exacerbations. Further investigation is needed to identify best practices in and barriers to telemedicine in this population.