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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Diet Pattern and Respiratory Morbidity in the Atherosclerosis Risk in Communities Study.

RATIONALE: Dietary intake is a potential risk factor for respiratory morbidity in adult populations. Few studies capture the effect of dietary patterns, representative of the combination of nutrients consumed, on self-reported respiratory morbidity in combination with objective measures of lung function. OBJECTIVES: To evaluate patterns of dietary intake in relation to respiratory morbidity and objective measures of lung function in a U.S. METHODS: The ARIC (Atherosclerosis Risk in Communities) study investigators enrolled 15,792 participants from four U.S. communities between 1987 and 1989 and collected data using a validated food frequency questionnaire to assess diet. Principal component analysis was applied, and patterns representative of "Western" and "Prudent" diets emerged. We investigated cross-sectional associations between dietary patterns and pulmonary assessments that included asthma and chronic obstructive pulmonary disease (COPD) diagnosis, respiratory symptoms, and lung function. Multivariable Poisson regression models included quintiles of dietary patterns and potential confounders. Interaction of dietary patterns with obesity, sex, and smoking status was assessed in relation to all outcomes. RESULTS: Higher scores in the "Western" dietary pattern (quintile 5 vs. quintile 1) were associated with higher prevalence of COPD (prevalence ratio [PR], 1.62; 95% confidence ratio [CI], 1.33-1.97), wheeze (PR, 1.37; 95% CI, 1.11-1.69), cough (PR, 1.32; 95% CI, 1.32-1.59), and phlegm (PR, 1.27; 95% CI, 1.05-1.54) and lower percent predicted forced expiratory volume in 1 second (FEV1), percent predicted forced vital capacity (FVC), and FEV1/FVC ratio. Higher scores in the "Prudent" dietary pattern (quintile 5 vs. quintile 1) were associated with lower prevalence of COPD (PR, 0.82; 95% CI, 0.70-0.95) and cough (PR, 0.77; 95% CI, 0.67-0.89) and higher percent predicted FEV1 and FEV1/FVC ratio. The prevalence of asthma was not related to dietary intake. CONCLUSIONS: A "Western" dietary pattern was associated with respiratory symptoms, lower lung function, and COPD in ARIC participants.

Bidimensional measurements of right ventricular function for prediction of survival in patients with pulmonary hypertension: comparison of reproducibility and time of analysis with volumetric cardiac magnetic resonance imaging analysis.

We tested the hypothesis that bidimensional measurements of right ventricular (RV) function obtained by cardiac magnetic resonance imaging (CMR) in patients with pulmonary arterial hypertension (PAH) are faster than volumetric measures and highly reproducible, with comparable ability to predict patient survival. CMR-derived tricuspid annular plane systolic excursion (TAPSE), RV fractional shortening (RVFS), RV fractional area change (RVFAC), standard functional and volumetric measures, and ventricular mass index (VMI) were compared with right heart catheterization data. CMR analysis time was recorded. Receiver operating characteristic curves, Kaplan-Meier, Cox proportional hazard (CPH), and Bland-Altman test were used for analysis. Forty-nine subjects with PAH and 18 control subjects were included. TAPSE, RVFS, RVFAC, RV ejection fraction, and VMI correlated significantly with pulmonary vascular resistance and mean pulmonary artery pressure (all P < 0.05). Patients were followed up for a mean (± standard deviation) of 2.5 ± 1.6 years. Kaplan-Meier curves showed that death was strongly associated with TAPSE <18 mm, RVFS <16.7%, and RVFAC <18.8%. In CPH models with TAPSE as dichotomized at 18 mm, TAPSE was significantly associated with risk of death in both unadjusted and adjusted models (hazard ratio, 4.8; 95% confidence interval, 2.0-11.3; P = 0.005 for TAPSE <18 mm). There was high intra- and interobserver agreement. Bidimensional measurements were faster (1.5 ± 0.3 min) than volumetric measures (25 ± 6 min). In conclusion, TAPSE, RVFS, and RVFAC measures are efficient measures of RV function by CMR that demonstrate significant correlation with invasive measures of PAH severity. In patients with PAH, TAPSE, RVFS, and RVFAC have high intra- and interobserver reproducibility and are more rapidly obtained than volumetric measures. TAPSE <18 mm by CMR was strongly and independently associated with survival in PAH.

Severity of systemic sclerosis-associated pulmonary arterial hypertension in African Americans.

African Americans (AA) with systemic sclerosis (SSc) have a worse prognosis compared to Americans of European descent (EA). We conducted the current study to test the hypothesis that AA patients with SSc have more severe disease and poorer outcomes compared to EA patients when afflicted with pulmonary arterial hypertension (PAH). We studied 160 consecutive SSc patients with PAH diagnosed by right heart catheterization, comparing demographics, hemodynamics, and outcomes between AA and EA patients. The cohort included 29 AA and 131 EA patients with similar baseline characteristics except for increased prevalence of diffuse SSc in AA. AA patients had worse functional class (FC) (80% FC III-IV vs 53%; p = 0.02), higher brain natriuretic peptide (NT-pro-BNP) (5729 ± 9730 pg/mL vs 1892 ± 2417 pg/mL; p = 0.02), more depressed right ventricular function, a trend toward lower 6-minute walk distance (263 ± 111  m vs 333 ± 110  m; p = 0.07), and worse hemodynamics (cardiac index 1.95 ± 0.58 L/min/m vs 2.62 ± 0.80 L/min/m; pulmonary vascular resistance 10.3 ± 6.2 WU vs 7.6 ± 5.0 WU; p  < 0.05) compared with EA patients. Kaplan-Meier survival estimates for AA and EA patients, respectively, were 62% vs 73% at 2 years and 26% vs 44% at 5 years (p  > 0.05). In conclusion, AA patients with SSc-PAH are more likely to have diffuse SSc and to present with significantly more severe PAH compared with EA patients. AA patients also appear to have poorer survival, though larger studies are needed to investigate this association definitively.

Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension.

BACKGROUND: Systemic sclerosis­associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis­related pulmonary hypertension with interstitial lung disease imposes a greater pulmonary vascular load than IPAH and leads to worse RV contractile function. METHODS AND RESULTS: We analyzed pulmonary artery pressures and mean flow in 282 patients with pulmonary hypertension (166 SScPAH, 49 systemic sclerosis­related pulmonary hypertension with interstitial lung disease, and 67 IPAH). An inverse relation between pulmonary resistance and compliance was similar for all 3 groups, with a near constant resistance×compliance product. RV pressure­volume loops were measured in a subset, IPAH (n=5) and SScPAH (n=7), as well as SSc without PH (n=7) to derive contractile indexes (end-systolic elastance [Ees] and preload recruitable stroke work [Msw]), measures of RV load (arterial elastance [Ea]), and RV pulmonary artery coupling (Ees/Ea). RV afterload was similar in SScPAH and IPAH (pulmonary vascular resistance=7.0±4.5 versus 7.9±4.3 Wood units; Ea=0.9±0.4 versus 1.2±0.5 mm Hg/mL; pulmonary arterial compliance=2.4±1.5 versus 1.7±1.1 mL/mm Hg; P>0.3 for each). Although SScPAH did not have greater vascular stiffening compared with IPAH, RV contractility was more depressed (Ees=0.8±0.3 versus 2.3±1.1, P<0.01; Msw=21±11 versus 45±16, P=0.01), with differential RV-PA uncoupling (Ees/Ea=1.0±0.5 versus 2.1±1.0; P=0.03). This ratio was higher in SSc without PH (Ees/Ea=2.3±1.2; P=0.02 versus SScPAH). CONCLUSIONS: RV dysfunction is worse in SScPAH compared with IPAH at similar afterload, and may be because of intrinsic systolic function rather than enhanced pulmonary vascular resistive and pulsatile loading.