An update of palliative care in lung transplantation with a focus on symptoms, quality of life and functional outcomes.

PURPOSE OF REVIEW: Palliative care (PC) in lung transplantation is increasingly acknowledged for its important role in addressing symptoms, enhancing functionality, and facilitating advance care planning for patients, families, and caregivers. The present review provides an update in PC management in lung transplantation. RECENT FINDINGS: Research confirms the effectiveness of PC for patients with advanced lung disease who are undergoing transplantation, showing improvements in symptoms and reduced healthcare utilization. Assessment tools and patient-reported outcome measures for PC are commonly used in lung transplant candidates, revealing discrepancies between symptom severity and objective measures such as exercise capacity. The use of opioids to manage dyspnea and cough in the pretransplant period is deemed safe and does not heighten risks posttransplantation. However, the integration of PC support in managing symptoms and chronic allograft dysfunction in the posttransplant period has not been as well described. SUMMARY: Palliative care support should be provided in the pretransplant and select peri-operative and posttransplant periods to help support patient quality of life, symptoms, communication and daily function.

Intermittent neck flexion induces greater sternocleidomastoid deoxygenation than inspiratory threshold loading.

PURPOSE: To compare deoxygenation of the sternocleidomastoid, scalenes, and diaphragm/intercostals (Dia/IC) during submaximal intermittent neck flexion (INF) versus submaximal inspiratory threshold loading (ITL) in healthy adults. METHODS: Fourteen participants performed a randomized, cross-over, repeated measures design. After evaluation of maximal inspiratory pressures (MIP) and maximum voluntary contraction (MVC) for isometric neck flexion, participants were randomly assigned to submaximal ITL or INF until task failure. At least 2 days later, they performed the submaximal exercises in the opposite order. ITL or INF targeted 50 ± 5% of the MIP or MVC, respectively, until task failure. Near-infrared spectroscopy (NIRS) was applied to evaluate changes of deoxy-hemoglobin (ΔHHb), oxy-hemoglobin (ΔO2Hb), total hemoglobin (ΔtHb), and tissue saturation of oxygen (StO2) of the sternocleidomastoid, scalenes, and Dia/IC. Breathlessness and perceived exertion were evaluated using Borg scales. RESULTS: Initially during INF, sternocleidomastoid HHb slope was greatest compared to the scalenes and Dia/IC. At isotime (6.5-7 min), ΔtHb (a marker of blood volume) and ΔO2Hb of the sternocleidomastoid were higher during INF than ITL. Sternocleidomastoid HHb, O2Hb, and tHb during INF also increased at quartile and task failure timepoints. In contrast, scalene ΔO2Hb was higher during ITL than INF at isotime. Further, Dia/IC O2Hb and tHb increased during ITL at the third quartile and at task failure. Borg scores were lower at task failure during INF compared to ITL. CONCLUSION: Intermittent INF induces significant metabolic activity of the sternocleidomastoid and a lower perception of effort, which may provide an alternative inspiratory muscle training approach for mechanically ventilated patients.

Clinical implications of frailty assessed in hospitalized patients with acute-exacerbation of interstitial lung disease.

BACKGROUND: Approximately 50% of patients with interstitial lung disease (ILD) experience frailty, which remains unexplored in acute exacerbations of ILD (AE-ILD). A better understanding may help with prognostication and resource planning. We evaluated the association of frailty with clinical characteristics, physical function, hospital outcomes, and post-AE-ILD recovery. METHODS: Retrospective cohort study of AE-ILD patients (01/2015-10/2019) with frailty (proportion ≥0.25) on a 30-item cumulative-deficits index. Frail and non-frail patients were compared for pre- and post-hospitalization clinical characteristics, adjusted for age, sex, and ILD diagnosis. One-year mortality, considering transplantation as a competing risk, was analysed adjusting for age, frailty, and Charlson Comorbidity Index (CCI). RESULTS: 89 AE-ILD patients were admitted (median: 67 years, 63% idiopathic pulmonary fibrosis). 31 were frail, which was associated with older age, greater CCI, lower 6-min walk distance, and decreased independence pre-hospitalization. Frail patients had more major complications (32% vs 10%, p = .01) and required more multidisciplinary support during hospitalization. Frailty was not associated with 1-year mortality (HR: 0.97, 95%CI: [0.45-2.10]) factoring transplantation as a competing risk. CONCLUSIONS: Frailty was associated with reduced exercise capacity, increased comorbidities and hospital complications. Identifying frailty may highlight those requiring additional multidisciplinary support, but further study is needed to explore whether frailty is modifiable with AE-ILD.

The association between nutritional risk index and ICU outcomes across hematologic malignancy patients with acute respiratory failure.

Patients with hematological malignancies (HM) are at risk of acute respiratory failure (ARF). Malnutrition, a common association with HM, has the potential to influence ICU outcomes. Geriatric nutritional risk index (G-NRI) is a score derived from albumin and weight, which reflects risk of protein-energy malnutrition. We evaluated the association between G-NRI at ICU admission and ICU mortality in HM patients with ARF. We conducted a single center retrospective study of ventilated HM patients between 2014 and 2018. We calculated G-NRI for all patients using their ICU admission albumin and weight. Our primary outcome was ICU mortality. Secondary outcomes included duration of mechanical ventilation and ICU length of stay. Two hundred eighty patients were admitted to the ICU requiring ventilation. Median age was 62 years (IQR 51-68), 42% (n = 118) were females, and median SOFA score was 11 (IQR 9-14). The most common type of HM was acute leukemia (54%) and 40% underwent hematopoietic cell transplant. Median G-NRI was 87 (IQR 79-99). ICU mortality was 51% (n = 143) with a median duration of ventilation of 4 days (IQR 2-7). Mortality across those at severe malnutrition (NRI < 83.5) was 59% (65/111) compared to 46% (76/164) across those with moderate-no risk (p = 0.047). On multivariable analysis, severe NRI (OR 2.34, 95% CI 1.04-5.27, p = 0.04) was significantly associated with ICU mortality. In this single center, exploratory study, severe G-NRI was prognostic of ICU mortality in HM patients admitted with respiratory failure.

Characterization and reliability of internet resources on pulmonary rehabilitation for individuals with chronic lung disease.

BACKGROUND: Individuals with lung disease commonly use the internet as a source of health information on pulmonary rehabilitation (PR). The objective of this study was to characterize internet resources on PR, and to assess the content, readability, and quality of patient-directed PR resources. METHODS: The first 200 websites for the search term 'pulmonary rehabilitation resources and exercise' were analyzed on Google, Yahoo, and Bing. Website content was assessed based on 30 key components of PR from the 2013 and 2021 international consensus statements. Website quality was determined using DISCERN, JAMA benchmarks, and Global Quality Scale (GQS). RESULTS: 66 unique PR websites were identified with the two most common categories being scientific resources (39%) and foundation/advocacy organizations (33%). The average reading level of websites was 11 ± 3. PR content varied significantly across websites (mean range 13.4-21.5). Median DISCERN total score and GQS score were 4 (IQR 3-4) and 3.5 (IQR 2-4), respectively, representing moderate-good quality. Foundation/advocacy websites had higher DISCERN and GQS scores compared to other websites. CONCLUSION: Foundation/advocacy websites had the highest quality and reliability metrics; however, the higher-than-recommended reading levels may compromise patient comprehension and utilization. This study provides critical insight on the current state of online PR health-related information.

Impact of cognitive capacity on physical performance in chronic obstructive pulmonary disease patients: A scoping review.

Background: Chronic obstructive pulmonary disease (COPD) is often accompanied by impaired cognitive and physical function. However, the role of cognitive function on motor control and purposeful movement is not well studied. The aim of the review was to determine the impact of cognition on physical performance in COPD. Methods: Scoping review methods were performed including searches of the databases: MEDLINE, EMBASE, Cochrane Systematic Reviews, Cochrane (CENTRAL), APA PsycINFO, and CINAHL. Two reviewers independently assessed articles for inclusion, data abstraction, and quality assessment. Results: Of 11,252 identified articles, 44 met the inclusion criteria. The review included 5743 individuals with COPD (68% male) with the forced expiratory volume in one second range of 24-69% predicted. Cognitive scores correlated with strength, balance, and hand dexterity, while 6-min walk distance (n = 9) was usually similar among COPD patients with and without cognitive impairment. In 2 reports, regression analyses showed that delayed recall and the trail making test were associated with balance and handgrip strength, respectively. Dual task studies (n = 5) reported impaired balance or gait in COPD patients compared to healthy adults. Cognitive or physical Interventions (n = 20) showed variable improvements in cognition and exercise capacity. Conclusions: Cognition in COPD appears to be more related to balance, hand, and dual task function, than exercise capacity.

Differential prognostic significance of sarcopenia in metastatic esophageal squamous and adenocarcinoma.

BACKGROUND: Sarcopenia indicates poor prognosis in various malignancies. We evaluated the association of sarcopenia with overall (OS) and progression-free survival (PFS) in metastatic esophageal cancer (MEC) patients, a population often presenting with poor nutritional status. METHODS: In newly diagnosed MEC patients managed at the Princess Margaret (PM) Cancer Centre (diagnosed 2006-2015), total muscle area, visceral adiposity (VA), and subcutaneous adiposity (SA) were quantified on abdominal computed tomography at L3. Sarcopenia was determined using published cutoffs, based on sex and height. RESULTS: Of 202 MEC patients, most were male (166/82%), < 65 years (116/57%), and had adenocarcinoma histology (141/70%); 110/54% had recurrent MEC after initial curative-intent treatment; 92/46% presented with de novo MEC. At stage IV diagnosis, 20/10% were underweight, 97/48% were normal-weight and 84/42% were overweight/obese; 103/51% were sarcopenic. Sarcopenia was associated with worse median OS (4.6 vs. 7.9 months; log-rank p = 0.03) and 1-year survival, even after adjusting for other body composition variables (e.g., BMI, VA, and SA): adjusted-HR 1.51 [95% CI 1.1-2.2, p = 0.02]. In post hoc analysis, sarcopenia was highly prognostic in adenocarcinomas (p = 0.003), but not squamous cell carcinomas (SCC). In patients receiving palliative systemic treatment (104/51%), sarcopenia was associated with shorter PFS (p = 0.004) in adenocarcinoma patients (75/72%). CONCLUSIONS: In metastatic esophageal adenocarcinomas, sarcopenia is associated with worse PFS and OS. In metastatic esophageal SCC, there was a non-significant trend for worse PFS but no association with OS. In order to offset the poor prognosis associated with sarcopenia particularly in metastatic esophageal adenocarcinoma patients, future research should focus on possible countermeasures.

The positive impact of a telemedicine education program on healthcare workers during the COVID-19 pandemic in Ontario, Canada.

Context The impact of the COVID-19 pandemic on health care service and delivery has been profound. In addition to shifting and expanding clinical responsibilities, rapidly evolving information and guidelines during the COVID-19 pandemic has made it difficult for healthcare workers (HCWs) to synthesize and translate COVID-19 information into practice. Objective The objectives of this study are 1) to examine the impact of a telemedicine education program on HCW's self-efficacy and satisfaction and 2) to explore HCWs' experience in the program and context of practice during the COVID-19 pandemic. Study Design We conducted a prospective mixed methods parallel-design study among ECHO COVID participants using pre-post questionnaires and focus group discussions (FGDs). We examined questionnaire results for changes in HCWs' self-efficacy and satisfaction. We analyzed FGD data to explore HCWs' experience in ECHO and the context of their practice during the COVID-19 pandemic. Setting Project Extension for Community Healthcare Outcomes (ECHO) model is a virtual, telemedicine education model that provides longitudinal support and addresses the emerging needs of HCWs. "ECHO Ontario: Managing COVID-19 Patients in the Community" (ECHO) was launched in July 2020. The goal of the program was to disseminate best practices regarding COVID-19 as they emerged and to increase HCW confidence and knowledge of COVID-19. Population Studied The study population were practicing HCWs in the province of Ontario, Canada. 1) HCW self-efficacy 2) HCW satisfaction Results Of 299 HCWs registered in ECHO COVID, 114 (38.1%) participated in this analysis. Median self-efficacy scores increased from 5 (IQR 4 - 6) to 6 (IQR 6 - 6) (p < 0.0001), independent of profession, years in practice, age group, or type of practice environment. Participants were highly satisfied with the ECHO sessions with a median score of 4 (IQR 4 - 5). FGD data indicated that HCWs gained knowledge through ECHO, established the context of their practice, and revealed facilitators for ECHO program success, including the transition to virtual care, the practicability of knowledge provided, and a "perspective from the trenches." Conclusions This study demonstrated that a telemedicine education program aimed to support HCWs in managing patients with COVID-19 had a positive impact on self-efficacy and satisfaction, and that this impact was specifically mediated through the ECHO program.

Glucocorticoid-induced myopathy in people with asthma: a systematic review.

OBJECTIVES: To review the current literature on the evidence and the underlying characteristics of glucocorticoids (type, dosage, and duration) associated with myopathy in asthma. DATA SOURCES: Four electronic databases were searched to October 19, 2020. STUDY SELECTION: Inclusion criteria: adults or adolescents with asthma, taking systemic glucocorticoids, and measures of muscle impairments. RESULTS: Nine studies met the eligibility criteria. The methodologic quality of most studies was fair or good. Two studies reported significantly lower inspiratory muscle function in outpatients taking daily oral glucocorticoids (≥10 mg), but one study reported no such difference. No differences was found in limb muscle strength in one study. Only 11-36% patients with acute exacerbation taking glucocorticoids intravenously suffered from limb muscle weakness during/after critical care admissions. Two studies reported significant associations between dosage of oral glucocorticoid use and inspiratory and limb muscle function, whereas seven studies did not find any significant correlations among the characteristics of systemic glucocorticoids and myopathy. Two studies comparing people with non-glucocorticoid dependent asthma taking inhaled glucocorticoid and healthy people did not find any significant differences in their inspiratory muscle strength and endurance. CONCLUSIONS: There were limited studies and inconsistent results on glucocorticoid-induced myopathy in people with asthma, and its association with the characteristics of glucocorticoids use. We recommended future studies should use a commonly accepted operational definition of myopathy, utilize a cohort study design, measure the cumulative dosage of glucocorticoids, and integrate possible confounding factors in the analysis.

Decreased automaticity contributes to dual task decrements in older compared to younger adults.

PURPOSE: To contrast older and younger adults' prefrontal cortex (PFC) neural activity (through changes in oxygenated hemoglobin) during single and dual tasks, and to compare decrements in task performance. METHODS: Changes in oxygenated hemoglobin of dorsolateral PFC were monitored using functional near-infrared spectroscopy during single tasks of spelling backwards (cognitive task) and 30 m preferred paced walk; and a dual task combining both. Gait velocity was measured by a pressure sensitive mat. RESULTS: Twenty sex-matched younger (27.6 ± 3.5 years) and 17 older adults (71.2 ± 4.9 years) were recruited. The left PFC oxygenated hemoglobin decreased from start (1st quintile) to the end (5th quintile) of the walking task in younger adults ( - 0.03 ± 0.03 to - 0.72 ± 0.20 µM; p < .05) unlike the non-significant change in older adults (0.03 ± 0.06 to - 0.41 ± 0.32 µM, p > .05). Overall, oxygenation increased bilaterally during dual versus single walk task in older adults (Left PFC: 0.22 ± 0.16 vs. - 0.23 ± 0.21 µM, respectively; Right PFC: 0.17 ± 0.18 vs. - 0.33 ± 0.22 µM, respectively), but only in right PFC in younger adults ( - 0.02 ± 0.15 vs. - 0.47 ± 0.13 µM). Older adults exhibited lower velocity during the dual task compared to younger adults (1.03 ± 0.16 vs. 1.20 ± 0.17 m/s, respectively). Older age was associated with dual task cost on velocity during walking after adjusting for confounding variables. CONCLUSIONS: Age-related cognitive decline in older adults may increase neural activity for cognitive tasks and diminish walking automaticity that may lead to decrements during dual tasking; the greater PFC increases in the oxygenated hemoglobin and lower velocity may be due to increased cognitive load and limited attentional resources.

Computed tomography-based body composition measures in COPD and their association with clinical outcomes: A systematic review.

BACKGROUND: Computed tomography (CT) is commonly utilized in chronic obstructive pulmonary disease (COPD) for lung cancer screening and emphysema characterization. Computed tomography-morphometric analysis of body composition (muscle mass and adiposity) has gained increased recognition as a marker of disease severity and prognosis. This systematic review aimed to describe the CT-methodology used to assess body composition and identify the association of body composition measures and disease severity, health-related quality of life (HRQL), cardiometabolic risk factors, respiratory exacerbations, and survival in patients with COPD. METHODS: Six databases were searched (inception-September 2021) for studies evaluating adult COPD patients using thoracic or abdominal CT-muscle or adiposity body composition measures. The systematic review was conducted in accordance with the PRISMA guidelines. RESULTS: Twenty eight articles were included with 15,431 COPD patients, across all GOLD stages with 77% males, age range (mean/median 59-78 years), and BMI range 19.8-29.3 kg/m2. There was heterogeneity in assessment of muscle mass and adiposity using thoracic (n = 22) and abdominal (n = 8) CT-scans, capturing different muscle groups, anatomic locations, and adiposity compartments (visceral, subcutaneous, and epicardial). Low muscle mass and increased adiposity were associated with increased COPD severity measures (lung function, exercise capacity, dyspnea) and lower HRQL, but were not consistent across studies. Increased visceral adiposity (n = 6) was associated with cardiovascular disease or risk factors (hypertension, hyperlipidemia, and diabetes). Low muscle CSA was prognostic of respiratory exacerbations or mortality in three of six studies, whereas the relationship with increased intermuscular adiposity and greater mortality was only observed in one of three studies. CONCLUSION: There was significant variability in CT-body composition measures. In several studies, low muscle mass was associated with increased disease severity and lower HRQL, whereas adiposity with cardiovascular disease/risk factors. Given the heterogeneity in body composition measures and clinical outcomes, the prognostic utility of CT-body composition in COPD requires further study.

Frailty and aging-associated syndromes in lung transplant candidates and recipients.

Many lung transplant candidates and recipients are older and frailer compared to previous eras. Older patients are at increased risk for pre- and posttransplant mortality, but this risk is not explained by numerical age alone. This manuscript represents the product of the American Society of Transplantation (AST) conference on frailty. Experts in the field reviewed the latest published research on assessment of elderly and frail lung transplant candidates. Physical frailty, often defined as slowness, weakness, low physical activity, shrinking, and exhaustion, and frailty evaluation is an important tool for evaluation of age-associated dysfunction. Another approach is assessment by cumulative deficits, and both types of frailty are common in lung transplant candidates. Frailty is associated with death or delisting before transplant, and may be associated with posttransplant mortality. Sarcopenia, cognitive dysfunction, depression, and nutrition are other important components for patient evaluation. Aging-associated inflammation, telomere dysfunction, and adaptive immune system senescence may also contribute to frailty. Developing tools for frailty assessment and interventions holds promise for improving patient outcomes before and after lung transplantation.

Feasibility and Outcomes of a Standardized Management Protocol for Acute Exacerbation of Interstitial Lung Disease.

BACKGROUND AND OBJECTIVE: Despite the high mortality of acute exacerbations of interstitial lung disease (AE-ILD), there is minimal evidence to guide management decisions. We aimed to assess the feasibility and outcomes of a standardized management protocol for AE-ILD. METHODS: We performed a retrospective cohort study of patients with AE-ILD admitted to hospital between January 2015 and August 2019. Patients were managed with a standardized protocol including chest computed tomography (CT) at diagnosis, pulse corticosteroid treatment, and a follow-up CT 7 days after corticosteroid pulse. The association between idiopathic pulmonary fibrosis (IPF) versus non-IPF diagnosis and transplant-free survival within 1-year of AE-ILD was assessed using adjusted Cox proportional hazards regression survival analysis. Associations with CT chest improvement 7 days after corticosteroid pulse were secondarily assessed. RESULTS: 89 patients with AE-ILD were identified. 1-year transplant-free and overall survival were 20.2 and 51.7%, respectively. Protocol adherence to pulse corticosteroids was high (95.5%). A diagnosis of IPF was associated with higher risk of death or transplant at 1-year versus a non-IPF diagnosis [hazard ratio (HR) 2.23, 95% CI 1.19-4.17, p = 0.012]. There were no significant associations with 7-day CT improvement; however, CT improvement was associated with higher transplant-free survival (p = 0.02) and a lower risk of in-hospital mortality (χ2 = 7.06, p = 0.01) on unadjusted analysis. CONCLUSIONS: IPF is associated with a higher risk of death or transplant at 1-year as compared to a non-IPF diagnosis in patients with AE-ILD managed using a standardized protocol. Improvement on CT chest 7 days after corticosteroid pulse is associated with better survival.

Muscle and cerebral oxygenation during cycling in chronic obstructive pulmonary disease: A scoping review.

To synthesize evidence for prefrontal cortex (PFC), quadriceps, and respiratory muscle oxygenation using near-infrared spectroscopy (NIRS) during cycling in individuals with chronic obstructive pulmonary disease (COPD). A scoping review was performed searching databases (inception-August 2020): Ovid MEDLINE, EMBASE, Cochrane Systematic Reviews, Cochrane Central Register of Controlled Clinical Trials, CINAHL, SPORTDiscus and Pedro. The search focused on COPD, cycling, and NIRS outcomes. 29 studies (541 COPD participants) were included. Compared to healthy individuals (8 studies), COPD patients at lower cycling workloads had more rapid increases in vastus lateralis (VL) deoxygenated hemoglobin (HHb); lower increases in VL total hemoglobin (tHb) and blood flow; and lower muscle tissue saturation (StO2). Heliox and bronchodilators were associated with smaller and slower increases in VL HHb. Heliox increased VL and intercostal blood flow compared to room air and supplemental oxygen in COPD patients (1 study). PFC oxygenated hemoglobin (O2Hb) increased in COPD individuals during cycling in 5 of 8 studies. Individuals with COPD and heart failure demonstrated worse VL and PFC NIRS outcomes compared to patients with only COPD-higher or more rapid increase in VL HHb and no change or decrease in PFC O2Hb. Individuals with COPD present with a mismatch between muscle oxygen delivery and utilization, characterized by more rapid increase in VL HHb, lower muscle O2Hb and lower muscle StO2. PFC O2Hb increases or tends to increase in individuals with COPD during exercise, but this relationship warrants further investigation. NIRS can be used to identify key deoxygenation thresholds during exercise to inform PFC and muscle oxygenation.

A review of respiratory manifestations and their management in Ehlers-Danlos syndromes and hypermobility spectrum disorders.

BACKGROUND: Ehlers-Danlos Syndromes (EDS) and Hypermobility Spectrum Disorders (HSD) are a heterogeneous group of heritable genetic connective tissue disorders with multiple characteristics including joint hypermobility, tissue fragility, and multiple organ dysfunction. Respiratory manifestations have been described in EDS patients, but have not been systematically characterized. A narrative review was undertaken to describe the respiratory presentations and management strategies of individuals with EDS and HSD. METHODS: A broad literature search of Medline, Embase, Cochrane Database of Systematic Reviews, and Cochrane CENTRAL was undertaken from inception to November 2020 of all study types, evaluating EDS/ HSD and pulmonary conditions. This narrative review was limited to adult patients and publications in English. RESULTS: Respiratory manifestations have generally been described in hypermobile EDS (hEDS), classical and vascular EDS subtypes. Depending on EDS subtype, they may include but are not limited to dyspnea, dysphonia, asthma, sleep apnea, and reduced respiratory muscle function, with hemothorax and pneumothorax often observed with vascular EDS. Respiratory manifestations in HSD have been less frequently characterized in the literature, but exertional dyspnea is the more common symptom described. Respiratory symptoms in EDS can have an adverse impact on quality of life. The respiratory management of EDS patients has followed standard approaches with thoracotomy tubes and pleurodesis for pleural manifestations, vocal cord strengthening exercises, continuous positive pressure support for sleep apnea, and exercise training. Reduced respiratory muscle function in hEDS patients responds to inspiratory muscle training. CONCLUSION: Respiratory symptoms and manifestations are described in EDS and HSD, and have generally been managed using conservative non-surgical strategies. Research into the prevalence, incidence and specific respiratory management strategies in EDS and HSD is needed to mitigate some of the associated morbidity.

Utilization of the 2017 diagnostic criteria for hEDS by the Toronto GoodHope Ehlers-Danlos syndrome clinic: A retrospective review.

The new 2017 diagnostic criteria for hypermobile Ehlers-Danlos Syndrome (hEDS) provide a framework for diagnosing hEDS but are more stringent than the previous Villefranche criteria. Our clinical experience at the GoodHope EDS clinic was that the 2017 criteria left many highly symptomatic patients without a diagnosis of hEDS. We conducted a retrospective cohort study to confirm our clinic experience and assess the accuracy of the 2017 diagnostic criteria for hEDS in patients who had a previous hEDS diagnosis based on the Villefranche criteria. Our study found that 15% (n = 20 of 131) of patients with a prior diagnosis of hEDS met the 2017 diagnostic criteria, and many of the traits used to distinguish hEDS were not significantly more frequent in patients who met 2017 criteria versus those who did not. In both groups objective systemic manifestations were found less frequently than subjective systemic manifestations. Beighton score (BS) as assessed by primary care practitioner was found to be higher than assessment by EDS practitioner in 81% (n = 74 of 91) of cases. Generalized joint hypermobility was confirmed in only 46% (n = 51 of 111) of patients who had a previous diagnosis of hEDS. Higher BS did not correlate with increased number of systemic manifestations in our cohort. Common comorbidities of hEDS were found with similar frequency in those who met 2017 criteria and those who did not. Based on our cohort, the 2017 hEDS diagnostic criteria require refinement to improve its diagnostic accuracy.

Pre-transplant short physical performance battery: Response to pre-habilitation and relationship to pre- and early post-lung-transplant outcomes.

PURPOSE: To evaluate whether the short physical performance battery (SPPB) pre-lung transplant (LTx) was responsive to pre-habilitation and predicted pre- and early post-transplant outcomes. METHODS: A retrospective study of LTx candidates accepted for transplant between 2016 and 2017. SPPB was categorized as frail/pre-frail (≤9/12) and non-frail (≥10/12). RESULTS: 150 patients had LTx assessment SPPB data (53% male, 61 [52-67] years, 59% had interstitial lung disease (ILD), 26% frail/pre-frail). 131 (87%) underwent transplant by December 31, 2018. Adjusting for age, sex, diagnosis and Canadian transplant listing urgency, and frailty/pre-frailty at LTx assessment was associated with a lower 6MWD pre-transplant [-89 meters 95%CI (-125 to -53), P < .0001]. 62 patients underwent six weeks of pre-habilitation. SPPB increased (11 [10-12) vs. 12 [11-12], P = .01) reflected in the chair stand component (11.4 ± 4.4 vs. 9.8 ± 2.8 seconds, P = .007), with larger improvements in the frail/pre-frail group. A frail/pre-frail SPPB closest to the time of transplant was associated with a lower 6MWD [-77 m 95%CI (-128 to -25), P = .004] but not with hospital length of stay or gait aid use three months post-transplant. CONCLUSIONS: Frailty/pre-frailty was associated with a decreased 6MWD pre- and post-transplant. The SPPB increased following pre-habilitation, which may reflect increased lower extremity strength.

Clinical outcomes associated with computed tomography-based body composition measures in lung transplantation: a systematic review.

Computed tomography (CT) is gaining increased recognition in the assessment of body composition in lung transplant (LTx) candidates as a prognostic marker of post-transplant outcomes. This systematic review was conducted to describe the methodology of CT measures of body composition used in LTx patients and its association with post-transplant outcomes. Six databases were searched (inception-April 2020) for studies of adult LTx patients with thoracic or abdominal CT measures [muscle cross-sectional area (CSA) and/or adiposity]. Thirteen articles were included with 1911 LTx candidates, 58% males, mean age range (48-61 years) and body mass index of 21.0-26.1 kg/m2 . Several methods were utilized using thoracic or abdominal CT scans to assess skeletal muscle (n = 11) and adiposity (n = 4) at various anatomic locations (carina, thoracic, and lumbar vertebrae), differing muscle groups, and adipose tissue compartments. Low muscle mass was associated with adverse outcomes in 6/11 studies, including longer mechanical ventilation days (n = 2), intensive care (n = 2) and hospital stay (n = 2), and mortality (n = 4). Greater subcutaneous and mediastinal fat were associated with increased risk of primary graft dysfunction (n = 2), but implications of adiposity on survival were variable across four studies. Further standardization of CT body composition assessments is needed to assess the prognostic utility of these measures on LTx outcomes.

An update on frailty in lung transplantation.

PURPOSE OF REVIEW: Frailty is prevalent in lung transplant candidates, and recent studies have demonstrated associations with increased mortality before and after transplantation. This review highlights important findings on the trajectory of frailty throughout the lung transplant process and provides valuable insight into frailty and some of its modifiable elements. RECENT FINDINGS: There have been several frailty indices used in lung transplantation, specifically the Frailty Phenotype, Short Physical Performance Battery (SPPB), and Cumulative Deficits. The two most commonly used measures - Frailty Phenotype and SPPB - reflect physical frailty and have been associated with increased morbidity and mortality pre and post-transplantation. However, there is emerging evidence that physical elements of frailty are reversible with rehabilitation before and after transplantation with improvement in frailty by 6 months after transplantation. The associations of frailty with physical activity levels, exercise capacity, and inflammation are discussed. SUMMARY: Frailty is prevalent before transplant, but physical frailty is modifiable with rehabilitation and transplantation. Thus, physical frailty should not be an absolute contraindication to lung transplantation, but efforts should focus on elements of frailty that are potentially modifiable.

Impaired cardiac autonomic response in lung transplant patients: A retrospective cohort study.

BACKGROUND: Cardiac autonomic dysfunction (AD) is associated with adverse prognosis in lung disease; however, its implications in lung transplantation have not been previously described. This study evaluated the prevalence of AD in lung transplant (LTx) candidates, its determinants, prognostic implications, and prevalence post-transplant. METHODS: Retrospective one-year study of 103 LTx candidates with AD assessed using heart rate (HR) from the six-minute walk test (6MWT). Impaired chronotropic response index (CRI) was defined as <80% age-adjusted HR during 6MWT. Abnormal HR recovery (HRR) was categorized as reduction in HR ≤ 12 beats/minute after the first minute of 6MWT cessation. RESULTS: Abnormal CRI and HRR were observed in 94% and 76% of patients, respectively. A lower CRI was associated with obstructive lung disease and lower estimated aerobic capacity. CRI was independently associated with 6MW distance [12 m per 10% CRI, P = 0.03], but not with post-transplant cardiovascular events, hospital stay, or one-year mortality. At 3 months post-transplant, no improvement was observed in CRI (99%, n = 66/67, P = 0.25) or HRR (88%, n = 36/41, P = 0.12). CONCLUSION: Cardiac AD was prevalent in LTx candidates and recipients, with chronotropic incompetence a modest contributor to exercise intolerance pretransplant. Further study may help determine whether direct autonomic measures such as HR variability may be more prognostic of LTx outcomes.