Alqalyoobi, S

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 Introduction/Rationale Hepatic sarcoidosis (HS) is a rare manifestation of sarcoidosis occurring in 6 to 11% of patients with sarcoidosis. Little is known about the clinical characteristics and overall outcome of patients who present with HS. Two recent single center studies from Europe and the U.S. outlined the clinical characteristics, outcomes, and therapeutic interventions in these patients. Our study aims to add to this body of knowledge; in particular, highlighting the clinical presentation, therapeutic interventions, comorbid disease burden, and overall outcomes in patients with HS presenting to a large tertiary care sarcoidosis center of excellence in the U.S. Methods We performed a retrospective review of all patients with hepatic sarcoidosis (HS) seen at the Sarcoidosis Center of Excellence at East Carolina University (ECU) between 2000 and 2025. Data including demographics, clinical manifestations, diagnostic tests, treatments and outcomes, were extracted. Results A total of 794 patients with sarcoidosis were identified. Twenty-one (21) patients had over 50% of missing data and were excluded. Of the 773 patients included in the study, 62 patients (8%) had hepatic sarcoidosis. Of these, 73% were female and 79% were African American. The mean age at diagnosis was 40 years. Thirty-nine percent (39%) of patients underwent a liver biopsy and majority (64%) had a Scadding CXR I or II at diagnosis. There was no significant difference in the age (at diagnosis), race, gender, BMI, smoking status, or pulmonary function when patients with HS were compared to the rest of the cohort (n = 711) (Table 1). CXR Scadding stage at presentation was also similar except that 5% of non-HS patients had a stage IV CXR at presentation compared to 0 patients with HS (Table 1). Evaluation for other organ involvement revealed that patients with HS were more likely to have splenic, Calcium-Vit D dysregulation, extra-thoracic lymphadenopathy, and bone/bone marrow involvement than non-HS patients (Table 2/Figure 1). Treatment patterns including use of methotrexate and infliximab were identical except that only 5% of patients were treated with ursodiol. This was unexpectedly low (Table 3). Outcomes including fatigue at any point during the follow-up and mortality are presented in Table 4. 10.4% of non-HS patients died versus 4.9% of HS patients. No patients in our cohort underwent a liver transplant. Conclusion Hepatic sarcoidosis was found in 8% of patients and was associated with multi-organ disease. Patients received similar medications, including methotrexate, as patients without hepatic sarcoidosis. This abstract is funded by: None