Citations

Abstract Rationale Chronic thromboembolic disease (CTED), which is the result of persistent pulmonary vascular obstruction despite 3 months of anticoagulation, can present with significant functional limitations and exercise intolerance despite normal resting hemodynamics. These patients may experience functional impairment due to exercise-induced pulmonary hypertension, impaired stroke volume augmentation with exercise, ventilatory inefficiency, and/or preload insufficiency. Such exercise-induced pathophysiological abnormalities can significantly impair quality of life and contribute to poor long-term outcomes. Although balloon pulmonary angioplasty (BPA) has shown benefit in patients with chronic thromboembolic pulmonary hypertension (CTEPH), its role in CTED patients with normal resting hemodynamics remains uncertain. This systematic review and meta-analysis evaluated the efficacy of BPA in this population. Methods A systematic search of PubMed, Cochrane, Embase, and Scopus was performed from inception through March 1, 2025. A total of 763 studies were screened, of which 42 full-text articles were assessed: six met the inclusion criteria (four retrospective, two prospective), comprising 98 patients. Results Across the included studies, baseline mean values were 20.81 +/- 3.62 mmHg for mean pulmonary arterial pressure (mPAP), 3.49 +/- 1.42 Wood units for pulmonary vascular resistance (PVR), and 405.72 +/- 108.40 m for the 6-minute walk distance (6MWD). Balloon Pulmonary Angioplasty (BPA) was consistently associated with significant improvements in hemodynamic and functional outcomes; BPA reduced mPAP by 3.40 mmHg and PVR by 0.90 WU, while increasing 6MWD by 31.90 m. These changes were also statistically robust across studies, with pooled standardized mean changes (SMC) of -0.88 for mPAP (95% CI, -1.10 to -0.67; P < .05), -0.68 for PVR (95% CI, -0.87 to -0.49; P < .05), and 0.29 for 6MWD (95% CI, 0.13 to 0.45; P < .05). Study-level effect sizes for 6MWD ranged from 0.19 to 0.54 (Figure 1A). Functional improvements were further demonstrated by shifts in NYHA class distribution (Figure 1B). Conclusion BPA was associated with meaningful reductions in mPAP and PVR and significant improvements in functional capacity among patients with CTED. These results indicate that BPA could be an effective treatment for certain patients with CTED, though more research is necessary. This abstract is funded by: None

Abstract Rationale Chronic thromboembolic disease (CTED) is characterized by persistent pulmonary vascular obstruction due to unresolved thromboemboli despite at least three months of anticoagulation. Although resting hemodynamics often remain normal, affected patients frequently develop functional limitation due to exercise-induced pulmonary hypertension, ventilatory inefficiency, limited stroke volume augmentation, or preload insufficiency. Such exercise-induced pathophysiological dysregulation can significantly impair quality of life and contribute to poor long-term outcomes. Pulmonary endarterectomy (PEA) is an established treatment for Chronic Thromboembolic Pulmonary Hypertension (CTEPH), offering significant improvements in hemodynamics and survival. Its role in CTED, however, remains unclear. While CTED has traditionally been managed with anticoagulation and supportive therapy, emerging evidence suggests PEA may provide symptomatic and functional benefit in selected CTED patients. Given the ongoing uncertainty regarding the role of PEA in CTED, we performed a systematic review and meta-analysis to define its safety profile and clinical efficacy in this patient subset. Methods A systematic search of PubMed, Cochrane, Embase, Scopus, Web of Science, ClinicalTrials.gov, and Google Scholar was conducted from database inception to March 1, 2025. Of 2,244 records screened, 34 full-text articles were assessed, and seven met the inclusion criteria. Results Across the included studies, the pooled baseline mean values were 20.48 ± 3.03 mm Hg for mean pulmonary arterial pressure (mPAP), 2.83 ± 1.11 Wood units for pulmonary vascular resistance (PVR), and 397 ± 100.08 m for the 6-minute walk distance (6MWD). Pulmonary Endarterectomy (PEA) was consistently associated with significant improvements in hemodynamic and functional outcomes; PEA reduced mPAP by 3.58 mmHg (pooled Standardized Mean Change (SMC)=-1.03; 95% CI, -1.23 to -0.83; P < .05), PVR by 0.86 Woods units (pooled SMC=-0.86; 95% CI, -1.05 to -0.67; P < .05), while, conversely, increased 6MWD by 37.49 m (pooled SMC, 0.49; 95% CI, 0.32 to 0.67; P < .05). Study-level effect sizes for 6MWD ranged from 0.21 to 1.47 (Figure 1A). This clinical improvement is further reflected in the enhancement of the NYHA functional class, as illustrated in Figure 1B. Conclusion PEA was associated with significant reductions in mPAP and PVR, as well as a considerable improvement in functional capacity in patients with CTED, with robust results across multiple tests for bias, influence, and sensitivity analyses. These findings support PEA as a viable therapeutic option for selected patients with CTED, although further research is warranted. This abstract is funded by: None

ABSTRACT Hepatopulmonary syndrome (HPS) affects up to one‐third of patients with advanced chronic liver disease (ACLD) and is an independent risk factor for increased mortality. While HPS often improves following liver transplantation, patients with severe or very severe HPS—classified by partial pressure of arterial oxygen (PaO 2 ) <60 mmHg—face higher post‐transplant complications and mortality. However, evidence suggests that even patients with severe HPS may benefit from liver transplantation when performed at high‐volume centers. To assess post‐transplant outcomes in this population, we conducted a retrospective chart review of 20 patients diagnosed with severe or very severe HPS who underwent liver transplantation at Mayo Clinic Hospital in Arizona between 2014 and 2024. The most common post‐transplant complication was persistent hypoxemia, managed on a case‐by‐case basis with various treatments, though no single approach proved consistently superior. All patients who received transplants demonstrated improvement; 84% of patients ultimately discontinued supplemental oxygen, with a mean time of 250 days (range: 0–1113 days). Time was the key factor in recovery. Notably, no deaths in our cohort were directly attributed to HPS. These findings support liver transplantation as a viable treatment option for HPS, even in severe cases, and underscore the importance of experienced transplant centers in optimizing patient outcomes.

ABSTRACT Capillary malformation-arteriovenous type 2 syndrome (CM-AVM2) is a rare, genetic vascular disorder associated with abnormal capillary malformations and arteriovenous malformations. We present a case of CM-AVM2 with refractory diarrhea and failure to thrive due to aberrant, telangiectatic capillaries, and subepithelial collagen deposition found throughout the GI tract on bidirectional endoscopy. The patient was recalcitrant to standard therapies, so bevacizumab was trialed, but the patient expired. This is the first case of CM-AVM2 with diffuse GI mucosal involvement resulting in malabsorption. There is no treatment for this pathology, but treatment with bevacizumab or a mammalian target of rapamycin inhibitor may be efficacious.

10556 Background: The coexistence of interstitial lung diseases (ILD) and lung cancer (LC) presents a clinical challenge. ILD, including idiopathic pulmonary fibrosis (IPF) and non-IPF entities, is associated with an elevated risk of LC. However, the clinical features, treatment patterns, and survival outcomes of ILD-LC, especially IPF-LC, remain underexplored. We aimed to comprehensively evaluate survival outcomes and risk factors for mortality of ILD-LC patients based on a real-world long-term clinical cohort. Methods: A total of 1,341 eligible patients for this study were drawn from the Epidemiology and Genetics of Lung Cancer research program database (Mayo Clinic) including 96 IPF-LC, 111 Non-IPF ILD-LC, 99 ILD-only, and 1,035 propensity-matched LC-only patients (Controls). Data were collected on demographics, comorbidities, tumor features, treatment, and patient outcomes. Survival analyses used Kaplan-Meier methods; risk factors were assessed via Cox Proportional Hazards models. All comparisons reported below were statistically significant with p-values≤0.001 unless specified. Results: Among the 20,470 primary LC patients who were diagnosed from 1997 through 2016 and followed to 2021, 207 (1.0%) had ILD-LC, of which 96 (46.4%) had IPF. Comparing to Controls, ILD-LC patients were 4 years older at the time of LC diagnosis, 63.8% (vs. 52.8%) male, had a higher rate of former smokers (60.4% vs. 47.3%), a lower proportion of adenocarcinoma (37.7% vs. 57.5%), and a higher proportion of squamous cell (28.0% vs. 20.3%) and small cell carcinoma (13.5% vs. 8.4%). Although no significant differences in either LC stage or grade, ILD-LC patients had a significantly higher mortality rate (90.3% vs. 78.8%), particularly among IPF-LC patients (97.9% vs. 83.8%). ILD-LC patients were less likely to undergo surgery or receiving pharmacological treatments (73.9% vs. 93.5%). The median 5-year overall survival rate (OS, months) were 49.5 for Controls, 35.2 for Non-IPF ILD-LC patients, and 15.9 for IPF-LC patients. Analysis of six most common comorbidities revealed that pulmonary hypertension was associated with worse OS in ILD-LC patients compared to those without it (11.9% vs. 20.1%, P < 0.05); however unexpectedly, hypertension in ILD-LC patients was associated with better OS (21.3% vs. 14.4%) and emphysema in IPF-LC patients with better OS (7.9% vs. 3.4%, P < 0.05) when compared to those without the two comorbidities, respectively. Conclusions: This study highlights the poorer survival outcomes in ILD-LC patients, particularly IPF-LC patients, emphasizing the need for further investigation into optimized therapies and comorbidity management. Our findings also call for attention to two unexpected associations of hypertension and emphysema, warranting in-depth research on the possible biological and physiological mechanisms.

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.

11118 Background: Lung cancer survivors experience more physical and mental challenges, heavier symptom burden, and poorer quality of life than survivors of other cancers. Many symptoms persist or worsen for survivors beyond 5 years of cancer diagnosis, referred as long-term lung cancer survivors (LTLCS). Social determinants of health (SDoH) factors negatively impact patient reported outcomes (PROs) among cancer survivors, but their role in outcomes is under reported in lung cancer survivors and unknown in LTLCS. We investigated the impact of SDoH on PROs in a 20-year prospective lung cancer cohort. Methods: Our cohort includes 19,251 lung cancer patients (cases) diagnosed from 1997 through 2016 and followed up to 27 years, of whom 4799 (25%) became LTLCS. SDoH information was obtained by a lung cancer study PRO questionnaire or an institution SDoH questionnaire. Analyzed in this report are 2982 cases and 480 controls who had a low-dose chest CT scan and were ruled out for cancer, all answered at least one PRO questionnaire (first response); and 1366 LTLCS answered 5 years after diagnosis (long-term response). Along with fatigue and dyspnea, 5 SDoH variables, i.e., financial concerns, legal concerns, family and friends support, spiritual well-being, and social activity were scored on a 0 (worst) - 10 (best) scale and dichotomized as 0-5 (deficit/worse/lower) or 6-10 (no deficit/better/higher). We also assessed employment and health-related work experience. A difference of 1.0 unit or more in score was considered clinically meaningful along with a 2-sided test P-value less than 0.05; Chi-square or Fisher’s Exact Test and Kruskal-Wallis test were used accordingly. Results: Compared to controls, cases were younger (mean age: 62 vs. 65 years, P<0.05); more women than men reported worse financial concerns (56% vs. 38%, P<0.01) and lower spiritual well-being (43% vs. 26%, P=0.04) at first response; this sex disparity reduced but remained significant at long-term. Worse dyspnea (4.4-4.8 vs. 5.6) and fatigue (4.7-4.8 vs. 5.8-5.9) were seen in cases with more financial and legal concerns compared to those with fewer concerns (Ps<0.01). Cases with higher family and friends support, spiritual well-being and social activity had less dyspnea (5.7-6.0 vs. 4.2-4.5) and fatigue (5.5-5.9 vs. 3.9-4.4) than those with lower scores (Ps<0.04). Employment and health-related work experience data were provided by 2213 responders: Comparing 1735 cases to 478 controls, a lower proportion of cases was employed (28% vs. 39%), more cases “often felt tired after work” (49% vs. 28%), and more cases had “quite a lot” of difficulty in daily work or could not work at all” (26% vs. 9%, Ps<0.01). Conclusions: We found that in lung cancer survivors, more women than men reported worse financial concerns, and lower SDoH status was correlated with worse dyspnea and fatigue, indicating SDoH-driven interventions are important to improve lung cancer survivors’ PROs.

Background: Prescription opioids are a major cause of the opioid epidemic. Despite the minimally invasive nature of medical thoracoscopy (MT), data on the efficacy of non-opioid-based pain control after MT is lacking. The purpose of this study is to assess the feasibility and efficacy of a non-opioid-based pain management strategy in patients who underwent MT. Methods: We performed a retrospective analysis of all patients who underwent MT in the Mayo Clinic (Minnesota and Arizona) outpatient setting. We assessed their pain level and the need for analgesia post-MT from August 1, 2019, to May 24, 2021. Results: Forty patients were included. In the first 24 hours, 5/40 (12.5%) reported no pain. Twenty-eight patients out of 40 (70%) reported minor pain (pain scale 1-3), and 7/40 (17.5%) reported moderate pain (pain scale 4-6). No patients reported severe pain. Twenty-two out of 35 patients who experienced discomfort (63%) required acetaminophen, 6/35 patients (17%) required nonsteroidal anti-inflammatory drug, and 7/35 patients (20%) did not require analgesia. Of the 7 patients who had moderate pain, 5 (71%) reported that the moderate pain improved to mild at 72 hours post-MT. Zero patients required opioids, and none reported contacting any provider to manage the pain post-MT. Fourteen patients (78%) who had both parietal pleural biopsies and tunneled pleural catheter placed reported minor pain, 3 patients (17%) reported moderate pain, and 1 patient (6%) experienced no discomfort. Conclusion: MT is well-tolerated by patients with non-opioid-based pain management strategy as needed if there is no absolute contraindication.

Background: Patients with advanced emphysema experience breathlessness due to impaired respiratory mechanics and diaphragm dysfunction. Bronchoscopic lung volume reduction (BLVR) is a minimally invasive bronchoscopic procedure done to reduce hyperinflation and air trapping, promoting atelectasis in the targeted lobe and allowing improved respiratory mechanics. Real-world data on safety and complications outside of clinical trials of BLVR are limited. Methods: We queried the US Food and Drug Administrations (FDA) Manufacturers and User Device Experience database from May 2019 to June 2020 for reports involving BLVR with endobronchial valve (EBV) placement. Events were reviewed for data analysis. Results: We identified 124 cases of complications during BLVR with EBV implantation. The most-reported complication was pneumothorax (110/124, 89%), all of which required chest tube placement. A total of 54 of these cases (54/110, 49%) were complicated by persistent air leak requiring additional interventions. Repeat bronchoscopy was needed to remove the valves in 28 patients, 12 were discharged with a Heimlich valve, and 10 had an additional pleural catheter placed. The other complications of BLVR with EBV placement included respiratory failure (6/124, 5%), pneumonia (4/124, 3%), hemoptysis (2/124, 1.6%), valve migration (1/124, 1%), and pleural effusion (1/124, 1%). A total of 14 deaths were reported during that year. Conclusion: Pneumothorax is the most-reported complication for BLVR with EBV placement, and in 65% of cases, pneumothorax is managed without removing valves. Importantly, 14 deaths were reported during that timeframe. Further studies are needed to estimate the true magnitude of the complications associated with BLVR.