Valle, Kathryn T. del

Purpose Survivors of critical illness often acquire significant physical, cognitive, and psychological burdens following their intensive care unit (ICU) stay, termed “post-intensive care syndrome” (PICS). Screening for PICS is generally not performed outside of research settings largely due to lack of a simple assessment tool and time constraints. Methods This was a prospective cohort study at a single academic medical center in the USA. We included adults (18 years or older) who spent greater than 48 h in any ICU within the medical center. Following transfer out of the ICU and at 3 months, psychological, cognitive, and physical functioning were assessed using the Hospital Anxiety and Depression Scale, Impact of Event Scale-Revised, Montreal Cognitive Assessment-blind, and Barthel Index. We compared these validated questionnaires to a brief, 15-item PICS screening tool (5 questions/domain) that was developed by our multidisciplinary study team and administered to study participants after ICU discharge to general care. The primary objectives of this study were to assess the ability of validated screening tools administered at ICU discharge to predict PICS at 3 months and develop a novel screening tool for identifying patients at risk of PICS. Major Findings 191 participants completed the initial questionnaire, and 109 (56%) completed the 3-month follow-up. PICS tool scores were compared to 3-month follow-up data from validated questionnaires to evaluate predictive performance. Overall, the screening tool weakly-to-moderately correlated with continuous outcomes of the validated measures at 3-month follow-up. When evaluating sensitivity and specificity for individual domains, the screening tool had an area under curve of >0.7 for all three domains, indicating moderate-to-good sensitivity and specificity. Conclusions We developed a brief PICS screening tool that is feasible to use without prior training that modestly predicted the development of PICS following ICU stay.

Abstract Introduction Extramedullary hematopoiesis (EMH) is the production of blood cells outside of bone marrow. It is associated with myeloproliferative neoplasms such as myelofibrosis and chronic myeloid leukemia. Most common sites for EMH are liver and spleen, but other sites may be involved. Pulmonary EMH is extremely rare. It is associated with symptoms of dyspnea, cough or chest pain. Chest imaging may demonstrate pulmonary nodules or masses, alveolar septal thickening, ground glass opacities, and/or patchy consolidations. We present a case of a woman with primary myelofibrosis who developed pulmonary hypertension (PH) in the setting of pulmonary EMH. Case description 73-year-old woman with history of primary myelofibrosis (diagnosed via bone marrow biopsy, grade 3 fibrosis, being treated with hydroxyurea) presented with progressive exertional dyspnea for 1.5 years. Exam was notable for elevated jugular venous pressure and mild peripheral edema. Labs revealed anemia with hemoglobin 8.2 g/dL, thrombocytosis with platelets 549K/dL and no leukocytosis. Brain natriuretic peptide was elevated at 1361 ng/L. Chest CT demonstrated few peripheral wedge-shaped opacities in the bilateral lower lobes were noted. Pulmonary function tests revealed normal spirometry and lung volumes but moderately reduced diffusion capacity. Echocardiogram showed normal left ventricular (LV) size and function with ejection fraction 60%, moderately enlarged right ventricle (RV) and RV systolic pressure 63 mmHg. Right heart catheterization showed RA pressure:14 mmHg, PA: 49/26/36 mmHg, PCWP 15mmHg, thermodilution CO 5.5 L/min, CI 3.1L/min/m2, Fick CO 4.6 L/min, CI 2.6 L/min/m2, pulmonary vascular resistance 3.8-4.6 Woods Units. Given her history of myelofibrosis, Tc-99m bone marrow scan was performed which showed diffuse radiotracer uptake in both lungs suggestive of pulmonary EMH. She subsequently underwent radiation therapy with 100cGy to bilateral lungs. On a follow-up visit two months later, she reported symptomatic improvement. Repeat NM bone scan showed decreased tracer uptake in the lungs. Discussion PH associated with myeloproliferative neoplasia is classified as group 5 in the latest clinical classification. It can be due to thrombocytosis, hypercoagulability, left ventricular failure, portal hypertension and/or hematopoietic infiltration of the pulmonary parenchyma. Tc-99m sulfur colloid scanning can be used to detect hematopoietic tissue in the lung. Treatment is usually radiation-therapy to the lungs to control the hematopoiesis. In patients with myeloproliferative neoplasms presenting with dyspnea, consider screening for PH with echocardiogram.

Abstract Rationale Patients with pulmonary hypertension (PH) often require significant assistance from caregivers. Caregivers frequently help patients with numerous PH-related tasks, such as medication administration and insurance coordination, in addition to emotional and other forms of support. However, little is known about optimal strategies for engaging and empowering caregivers in PH. We sought to improve understanding of PH patients’ and caregivers’ perspectives on the role of caregiving to identify ways to better support them. Methods We performed a cross-sectional survey of PH patients and/or their adult caregivers participating in the Pulmonary Hypertension Association conference in August 2024. Questions focused on roles and perspectives towards caregiving. Study participation was voluntary without monetary or other compensation. Results Sixteen patients and 18 caregivers completed the survey, with 9 patient and caregiver pairs. Patient characteristics and responses are detailed in Table 1. The majority of patients (87.5%) reported that caregivers play a “very important” role in their lives. (Table 1) Most surveyed caregivers were patients’ significant others (10/18, 55.5%) or parents (7/18, 38.9%), with average age 59 years, and were employed full-(10/18, 55.5%) or part-time (3/18, 16.7%). Regarding their role(s), all caregivers (18/18) reported providing emotional support, 7/18 (38.9%) physical support (e.g. mobility, hygiene assistance), 7/18 (38.9%) help with medications, and 13/18 (72.2%) help with transportation to appointments. 4/18 (22.2%) caregivers reported they spend more than 20 hours/week on caregiving-related tasks while most reported they spend 5-20 hours/week. Caregivers detailed challenges regarding emotional support (e.g. “Spouse seems to be very short with me most of the time. I think she hates this disease and takes it out on others”), among others. However, they also reported caregiving to be rewarding (e.g. “Seeing my husband improve, and getting to be on his side to advocate for him and other patients.”) Caregivers had many suggestions for what they would like to see in a program focused on supporting them, including “Online education such as resources and experts such as new treatments, insurance information…How to advocate better. Tips for self care” and “Connection to therapist who understand chronic diseases and the challenges of long term caregivers.” Conclusions Caregivers play an integral and multifaceted role in the lives of patients with PH. Caregivers would appreciate additional education, resources and support.

Abstract RATIONALE: Many ICU patients experience physical, psychological, and cognitive impairments following resolution of critical illness, collectively referred to as post-intensive care syndrome (PICS). PICS has become widely recognized within the critical care field, but how to screen for PICS does not currently have consensus. Development of a validated, concise PICS screening tool would allow for rapid identification of this vulnerable and understudied population. Therefore, this study's goal was to develop and evaluate a screening tool to identify patients at risk for PICS. METHODS: We performed a prospective cohort study in a quaternary academic hospital. We included adults ≥ 18 years old with ≥48 hours spent in an ICU. Exclusion criteria were admission for suicide attempt, severe cognitive impairment/dementia, life expectancy <3 months, and non-English speaking. We assessed mental, cognitive, and physical function following ICU discharge and at 3 months using Hospital Anxiety and Depression Scale, Impact of Event Scale-Revised, Montreal Cognitive Assessment-blind, and Barthel Index. Our team, including critical care physicians, psychiatrists, psychologists, physical and occupational therapists, and nurses, developed a brief 15-item PICS screening tool (5 questions/domain). This tool was administered after patients were discharged from ICU stay to general care to assess its effectiveness in predicting impairments in all 3 domains at discharge and again at three months after hospital discharge, in comparison to previously mentioned validated questionnaires. Youden index statistical methods were used to determine potential “cutoff” values (scores) for the tool to maximize sensitivity and specificity. RESULTS: A total of 194 patients from 6 ICUs were recruited and completed the initial questionnaire, and 109 (56%) patients completed 3-month follow-up. Median age was 65 years (IQR 57-72) and 49% (95/194) were female. Screening tool is depicted in Figure 1. Overall, the screening tool weakly-to-moderately correlated with continuous outcomes of the validated measures upon ICU discharge and 3-month follow-up. When evaluating sensitivity and specificity for individual domains, the tool had an area under the receiver operating curve of >0.7 for the following domains: cognitive, anxiety, and physical impairments, indicating moderate-to-good sensitivity and specificity. CONCLUSIONS: This PICS screening tool was correlated with validated measures of PICS upon ICU discharge and at 3-month follow-up for all three domains. The tool's strength lies in its concise nature, making it easier for those caring for this patient population to screen for long-term effects of an ICU stay. Our proposed next step is validation of the PICS screening tool in an external cohort.

Abstract Pulmonary vascular disease is a frequent complication of chronic liver disease and portal hypertension, affecting up to 30% of patients. There are two distinct pulmonary vascular complications of liver disease: hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). HPS affects 25% of patients with chronic liver disease and is characterized by intrapulmonary vasodilatation and abnormal arterial oxygenation. HPS negatively impacts quality of life and is associated with a 2‐fold increased risk of death compared to controls with liver disease without HPS. Angiogenesis, endothelin‐1 mediated endothelial dysfunction, monocyte influx, and alveolar type 2 cell dysfunction seem to play important roles in disease pathogenesis but there are currently no effective medical therapies. Fortunately, HPS resolves following liver transplant (LT) with improvements in hypoxemia. POPH is a subtype of pulmonary arterial hypertension (PAH) characterized by an elevated mean pulmonary arterial pressure and pulmonary vascular resistance in the setting of normal left‐sided filling pressures. POPH affects 5% to 6% of patients with chronic liver disease. Although the pathogenesis has not been fully elucidated, endothelial dysfunction, inflammation, and estrogen signaling have been identified as key pathways involved in disease pathogenesis. POPH is typically treated with PAH targeted therapy and may also improve with liver transplantation in selected patients. This article highlights what is currently known regarding the diagnosis, management, pathobiology, and outcomes of HPS and POPH. Ongoing research is needed to improve understanding of the pathophysiology and outcomes of these distinct and often misunderstood pulmonary vascular complications of liver disease. © 2021 American Physiological Society. Compr Physiol 12:3281‐3302, 2022.

Background. The current model for end-stage liver disease (MELD) exception policies for portopulmonary hypertension (POPH) require serial right heart catheterizations (RHCs) every 3 mo to maintain exception points. RHC is necessary for the initial diagnosis of POPH, but the utility of serial catheterizations has not been studied. In patients with POPH MELD exceptions, we sought to compare noninvasive and invasive hemodynamics and determine the sensitivity of echocardiography for the detection of hemodynamically severe POPH that would preclude liver transplant. Methods. We performed a single-center retrospective cohort study of patients with POPH MELD exceptions who underwent liver transplant from December 2008 to January 2024. Results were validated at an external center. Echocardiograms and RHCs performed within 1 mo were compared. Pearson correlation coefficient and Bland-Altman plots assessed the association between echocardiogram and RHC variables. We examined varied echocardiographic parameters to optimize sensitivity for the detection of hemodynamically severe POPH. Results. Twenty-two individuals underwent 60 follow-up RHCs with paired echocardiograms. Right ventricular systolic pressure (RVSP) and cardiac index estimated with echocardiogram were not strongly correlated with RHC measurements at follow-up (RVSP and RHC pulmonary artery systolic pressure: R = 0.30, P = 0.02; cardiac index: R = 0.17, P = 0.21). However, echocardiograms with RVSP ≥48 mm Hg had 100% sensitivity for detecting hemodynamically severe POPH, with 100% negative predictive value. In external validation of 13 paired echocardiograms and RHCs, our algorithm had 64% specificity and 100% negative predictive value. Conclusions. Although echocardiogram and RHC hemodynamic estimates were not strongly correlated, these results could potentially negate the current requirement for repeat RHC every 3 mo to maintain POPH MELD exception.