Citations

Objectives: Pulmonary function tests (PFTs) are essential for diagnosis, management, and follow-up of a wide range of respiratory conditions as they provide quantitative insights into various aspects of lung function. Precision in ordering and scheduling PFTs, like any test, is crucial as errors can lead to misdiagnoses, delayed treatments, unnecessary retesting, inflated healthcare costs, and potentially compromised patient care. Hence, the aim of this quality improvement initiative was to reduce the PFT ordering error rate to below 5% by redesigning the ordering workflow and implementing a standardized order set tailored to specific clinical scenarios. Methods: The quality improvement initiative was implemented from August 2023 to March 2024. Data was collected from the electronic medical record (EMR) using predefined operational definitions to identify incorrect PFT orders and scheduling errors. A redesigned orderset was created to align with specific clinical presentations, including symptoms, suspected diagnosis, confirmed diagnosis, and presurgical evaluation. Scheduling was also restructured to match the expected testing time for each category. Education sessions were conducted with ordering providers, and ongoing feedback loops were established. Results: Among 575 preintervention PFT orders, 130 (22.6%) contained errors. Following implementation, review of 607 postintervention orders showed a reduction in error rates to 4.1%, representing an ∼80% improvement ( P <0.01). There was no increase in unnecessary testing, supporting the effectiveness of the intervention without unintended negative consequences. Conclusions: Implementation of a knowledge-based CDSS with standardized order sets significantly reduced PFT ordering errors and variability, improving guideline adherence and care quality. Continued refinement and integration with evolving technologies are essential for broader applicability.

ABSTRACT Pulmonary hypertension (PH) comprises a heterogeneous group of disorders characterized by elevated pressure in the pulmonary arteries. Despite advances in awareness, utilization of non‐invasive screening with echocardiography, increasing number of targeted therapies, and evolving clinical guidelines emphasizing referral to PH specialty centers, barriers to timely diagnosis and treatment persist. Key challenges span patient‐related and system‐level domains that hinder the effective diagnosis, management, and treatment of PH, particularly pulmonary arterial hypertension (PAH). In this article, the obstacles to PH care are examined and actionable recommendations are proposed. By integrating patient‐related and system‐level perspectives, this review provides a framework to bridge persistent gaps in PH care delivery in the United States.

Abstract Introduction The World Health Organization (WHO) has categorized pulmonary hypertension (PH) in five groups. Obstructive sleep apnea (OSA) is an independent cause of group 3 PH. The prevalence of OSA in PH patients is approximately 20% and untreated OSA may contribute to pulmonary vascular remodeling, exacerbating PH. Regular use of positive airway pressure (PAP) can improve pulmonary artery pressures. We hypothesized that untreated OSA is associated with mortality in all five groups of PH, and that mortality is reduced with PAP therapy. Methods We performed a retrospective chart review of the PH patient registry at Mayo Clinic Jacksonville, comprised of 696 patients classified per WHO classification of PH. Mortality in each WHO group of PH was abstracted as well as the presence of OSA as a comorbidity. Compliance with positive airway pressure therapy (PAP) was assessed across all patients with OSA. Results Patients in the registry were followed for a mean of 101.2 months. The mortality across all groups was 36.8%, ranging from 22.6% in group 4 to 64.3% in group 5. OSA was present in 46.9% patients, and it ranged from 33% in group 5 to 58% in group 2. Mortality did not vary significantly in the patients with OSA versus those without OSA (p=0.56). Of patients with OSA, 45.9% was deemed compliant with PAP therapy. Overall, the compliant patients had reduced mortality compared to the noncompliant ones (p=0.045), however this significance was due to pronounced effect in group 1 patients only. Conclusion Almost half of PH patients from our registry had OSA. Compliance with PAP therapy was associated with lesser mortality, primarily due to the effect seen in Group 1 PH patients. Support (if any)

ABSTRACT Pulmonary hypertension due to interstitial lung disease (PH‐ILD) is associated with high morbidity and mortality. Real‐world patients initiating inhaled treprostinil are not well‐characterized. This retrospective cohort study aimed to evaluate healthcare resource utilization in patients with PH‐ILD who initiated treatment with inhaled treprostinil vs patients who remained untreated. Adult patients diagnosed with PH‐ILD were indexed on initiation of inhaled treprostinil and patients who remained untreated were indexed on first observed PH diagnosis (31 March 2021–30 September 2024). Patients were excluded if they were ever treated with any pulmonary arterial hypertension therapy. Inhaled treprostinil patients were matched to up to four untreated patients. All‐cause per‐patient per‐month (PPPM) hospitalizations and ICU‐related hospitalizations were the primary outcomes of interest. 294 patients treated with inhaled treprostinil and 736 untreated patients were identified. Mean all‐cause PPPM hospitalizations remained unchanged in the pre‐index vs post‐index periods in the inhaled treprostinil cohort (0.11 vs 0.12; p = 0.42) but significantly increased in untreated patients (0.12 vs 0.23; p < 0.01). ICU utilization also remained unchanged in the pre‐index vs post‐index periods for the inhaled treprostinil cohort (0.06 vs 0.07; p = 0.11) compared to the untreated cohort (0.06 vs 0.13; p < 0.01). Untreated patients had significantly higher post‐index PPPM hospitalizations ( p < 0.01) and ICU utilization ( p < 0.01) compared to inhaled treprostinil patients. Patients who initiated inhaled treprostinil had a 30% decreased risk of hospitalization compared to untreated patients (relative risk: 0.70; 95% CI: 0.59–0.83; p < 0.01). Among real‐world patients with PH‐ILD, treatment with inhaled treprostinil is associated with fewer all‐cause hospitalizations and ICU‐related hospitalizations compared to untreated patients with PH‐ILD.

ABSTRACT Pulmonary hypertension (PH) is a common complication in interstitial lung disease (ILD), but the additional burden it imposes on patients and healthcare systems is not well characterized. This retrospective analysis of claims data from the US Merative MarketScan database assessed hospitalization rates and costs over 2 years in patients with connective tissue disease‐related ILD (CTD‐ILD) and non‐CTD‐ILD with or without PH (between January 2017 and December 2019). Index was the date of first ILD claim; baseline was the 12‐month preindex period. In total, 16,129 patients with non‐CTD‐ILD (1502 [9%] with PH) and 4545 patients with CTD‐ILD (663 [15%] with PH) were identified. A higher proportion of patients with non‐CTD‐ILD with PH, compared with patients without PH, had all‐cause and heart failure (HF)‐related hospitalizations during baseline (all‐cause, 37.0% vs. 22.3%; HF‐related, 14.2% vs. 3.1%), 0–12 months (62.4% vs. 43.0%; 29.0% vs. 7.0%), and 13–24 months (44.7% vs. 18.3%; 21.5% vs. 3.2%) follow‐up ( p < 0.0001 for all). A significantly higher proportion of patients with CTD‐ILD with PH, compared with patients without PH, had all‐cause and HF‐related hospitalizations during baseline (all‐cause: 30.0% vs. 20.6%; HF‐related: 9.1% vs. 1.9%), 0–12 months (41.9% vs. 29.1%; 15.5% vs. 3.9%), and 13–24 months (37.9% vs. 18.9%; 13.9% vs. 2.5%) follow‐up ( p < 0.0001 for all). In both cohorts, total all‐cause and HF‐related costs were significantly higher in patients with PH at baseline, 0–12 months, and 13–24 months follow‐up ( p < 0.05 for all). PH substantially increased hospitalization risks and costs in both types of ILD, underscoring the importance of improving outcomes in patients with ILD‐PH.

Abstract Rationale Pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality. The extent of medication nonadherence in PAH is uncertain and may be linked to adverse outcomes. There has been a lack of multicenter, registry-based studies assessing medication nonadherence and patient-centered outcomes in PAH. Objectives To determine the prevalence of self-reported nonadherence in Pulmonary Hypertension Association Registry (PHAR) participants with PAH or chronic thromboembolic pulmonary hypertension and the relationship of nonadherence with several patient-centered outcomes (mortality, hospitalization rates, emergency department visits, and health-related quality of life). Methods Self-reported PAH medication nonadherence was captured at PHAR enrollment and during follow-up visits. Predictors of nonadherence were modeled using generalized estimating equations assuming a binary distribution. Outcomes associated with nonadherence were modeled using generalized estimating equations with a Poisson distribution. Results A total of 1,543 patients were included, of whom 1,092 (70.8%) were female and 1,340 (86.8%) had PAH. The overall rate of any self-reported nonadherence was 6.1% (95% confidence interval [CI], 5.3–6.9). Predictors of nonadherence included self-reported male sex at birth (odds ratio [OR], 1.4; 95% CI, 1.0–1.9; P = 0.02), poverty (OR, 1.6; 95% CI, 1.2–2.3; P = 0.01), not being married or partnered (OR, 1.5; 95% CI, 1.1–1.9; P = 0.01), having Medicaid or no health insurance (OR, 2.1; 95% CI, 1.5–2.9; P &lt; 0.001), and having completed high school but not having a college degree (OR, 1.7; 95% CI, 1.1–2.9; P &lt; 0.001). PHAR participants who reported any nonadherence had 50.0% more emergency department visits (P &lt; 0.001), 13.3% more hospital admissions (P = 0.03), and 61.9% more days hospitalized (P = 0.01). No relationship was observed between nonadherence and the type or number of PAH therapies or all-cause mortality. Participants reporting nonadherence had worse mean Short Form-12 scores (P &lt; 0.001) and worse emPHasis-10 scores (P = 0.02). Conclusions The rate of self-reported nonadherence in PHAR registrants was low but was associated with male sex and several social determinants of health. Although the complexity or type of PAH regimen did not appear to influence nonadherence, nonadherence was associated with numerous adverse patient-centered outcomes, including greater healthcare use and worse health-related quality of life. Because of limitations in the structure of the gathered data, relationships between exposure and outcomes were not temporally definitive; these observations warrant additional prospective studies.

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.

Introduction: Portopulmonary hypertension (POPH) and associated pulmonary vascular resistance (PVR) elevation is seen in the context of underlying portal hypertension and liver disease. In the systemic circulation, liver cirrhosis can cause inappropriate splanchnic and systemic vasodilation with decreased systemic vascular resistance (SVR). Midodrine is used to maintain blood pressure in the setting of low SVR. Pulmonary vasodilators have systemic vasodilatory effects; the implications of combined midodrine and pulmonary vasodilator use in POPH are unanswered questions. Hypothesis: We hypothesized that SVR would decrease over time in patients with POPH started on pulmonary vasodilators, and that use of midodrine (as a surrogate for depressed SVR), may be associated with increased risk of mortality in patients with POPH starting pulmonary vasodilator therapy. Methods: Patients from the 3 Mayo Clinic sites (Rochester, MN; Jacksonville, FL; Scottsdale, AZ) diagnosed with POPH from 1988-2020 were included in the database. Patients met hemodynamic criteria for POPH via right heart catheterization (RHC). Hemodynamics were obtained from echocardiography and RHC at initial and subsequent visits. The hazard ratio for risk of death was estimated using Cox proportional hazards method. SVR was calculated using mean arterial pressure and cardiac output. Results: Analysis included 147 POPH patients, 34 (23%) on midodrine and 113 (77%) not requiring midodrine. There were 116 patients with longitudinal SVR data. The mean initial SVR was 1224 Dyne sec cm-5 and decreased 251 Dyne sec cm-5 [95% CI -337 to -165 251 Dyne sec cm-5, p<0.0001] with no difference between midodrine groups (p=0.57). Patients in the midodrine POPH group had an increased risk of mortality compared to those without midodrine use (HR 1.51, 95% CI: 1.02-2.21, p=0.0371). Conclusions: Patients with POPH starting pulmonary vasodilator have a reduction in SVR related to vasodilator therapy. Midodrine use with pulmonary vasodilator therapy is associated with increased mortality in the POPH population. Further study of the safety of combined midodrine and pulmonary vasodilator use in POPH is needed along with exploration of more selective pulmonary vasodilators.

Abstract Objectives We aimed to improve the operational efficiency of clinical staff, including physicians and allied health professionals, in the previsit review of patients by implementing a disease-focused dashboard within the electronic health record system. The dashboard was tailored to the unique requirements of the clinic and patient population. Methods A prospective quality improvement study was conducted at an accredited pulmonary hypertension (PH) clinic within a large academic center, staffed by two full time physicians and two allied health professionals. Physicians' review time before and after implementation of the PH dashboard was measured using activity log data derived from an EHR database. The review time for clinic staff was measured through direct observation, with review method—either conventional or newly implemented dashboard—randomly assigned. Results Over the study period, the median number of patients reviewed by physicians per day increased slightly from 5.50 (interquartile range [IQR]: 1.35) before to 5.95 (IQR: 0.85) after the implementation of the PH dashboard (p = 0.535). The median review time for the physicians decreased with the use of the dashboard, from 7.0 minutes (IQR: 1.55) to 4.95 minutes (IQR: 1.35; p < 0.001). Based on the observed timing of 70 patient encounters among allied clinical staff, no significant difference was found for experienced members (4.65 minutes [IQR: 2.02] vs. 4.43 minutes [IQR: 0.69], p = 0.752), while inexperienced staff saw a significant reduction in review time after familiarization with the dashboard (5.06 minutes [IQR: 1.51] vs. 4.12 minutes [IQR: 1.99], p = 0.034). Subjective feedback highlighted the need for further optimization of the dashboard to align with the workflow of allied health staff to achieve similar efficiency benefits. Conclusion A disease-focused dashboard significantly reduced physician previsit review time while that for clinic staff remained unchanged. Validation studies are necessary with our patient populations to explore further qualitative impacts on patient care efficiency and long-term benefits on workflow.

Objectives Mayo Clinic’s hospital-at-home program, Advanced Care at Home (ACH), launched in 2020. While hospital-at-home literature reported safe and effective care for the general patient population and those with COVID, comparative outcomes between these two groups were unknown. The aim of this retrospective analysis was to compare the outcomes of COVID and non-COVID patients enrolled in ACH and evaluate if COVID patients can be safely treated in this setting. Methods Demographics, clinical characteristics, and safety outcomes were retrospectively analyzed to compare COVID and non-COVID patients discharged from ACH between November 2020 and May 2022. Patient characteristics analyzed included severity of illness (SOI) and risk of mortality (ROM), calculated using All Patient Refined Diagnosis Related Groups (APR-DRG). Hospitalization-specific variables included length of stay (LOS), escalation of care, and 30-day readmission rates. Results Of 1051 patients, 173 (16%) had COVID, and 878 (84%) were non-COVID patients. The average age in the COVID cohort was 66.6 (15.3) years, compared with 72.2 (14.0) in the non-COVID cohort. Extreme SOI was higher in the COVID group (43.3% versus 17.4%), as was extreme ROM (46.2% versus 16.2%), but LOS was shorter (5.7 versus 7 days). Escalation of care (7.5% in COVID cohort versus 8.4%) and 30-day readmission outcomes (9.2% for COVID patients versus 12.9%) were not statistically different between the groups. Conclusions Despite higher SOI and ROM, COVID patients had shorter LOS with outcomes that were not statistically different from non-COVID patients. COVID patients can be safely and efficiently cared for in ACH.