Pulmonary veno-occlusive disease (PVOD) is a rare and aggressive subtype of pulmonary arterial hypertension characterized by fibroproliferative obstruction of post-capillary pulmonary venules leading to increased pulmonary vascular resistance and progressive right ventricular failure. Heritable forms are linked to eukaryotic translation initiation factor 2 alpha kinase 4 (EIF2AK4) mutations, while acquired cases have been associated with environmental exposures, including organic solvents and chemotherapeutic agents like mitomycin-C. PVOD is classified under Group 1 pulmonary hypertension in the current World Symposium on Pulmonary Hypertension classification; however, pulmonary vasodilator therapy has limited efficacy due to the frequent occurrence of acute pulmonary edema. 1 Lung transplantation remains the only definitive and effective therapy for PVOD, and in its absence, the prognosis is uniformly poor. Given the therapeutic and prognostic implications, establishing an accurate diagnosis and early referral for lung transplantation evaluation is imperative. In this single transplant center case series of seven cases, we highlight the clinical presentation and the importance of early recognition and referral to specialized centers for optimal management.

Introduction:An emerging body of evidence suggests that extended static preservation of donor lung allografts at 10°C yields clinical outcomes comparable to standard storage, while enabling transplantation to be performed in a semi-elective manner. We conducted a retrospective analysis to determine whether a mid-sized lung transplant program could replicate outcomes reported in a recent clinical trial and larger transplant centers, while assessing the feasibility and real-world applicability of extended static cold storage. Methods:We conducted a retrospective single-center study of lung transplants performed between January 1 and December 31, 2025. Recipients were categorized by preservation strategy into standard storage or extended static cold storage at 10°C. Clinical and demographic data were obtained from the electronic health record. The primary outcome was the incidence of primary graft dysfunction (PGD) grades 1 to 3 at 0, 24, 48, and 72 hours following transplantation. Secondary outcomes included duration of mechanical ventilation, length of hospitalization, length of ICU stay, postoperative extracorporeal membrane oxygenation (ECMO) requirement, and 30-day survival. Results:Among 59 lung transplants, 40 underwent extended static cold storage at 10°C and 19 underwent standard preservation. Idiopathic pulmonary fibrosis was the most common indication; 55 of 59 recipients received bilateral lung transplantation. Four cases utilized ex vivo lung perfusion (EVLP) (Lung Bioengineering Inc., Silver Spring, MD, USA). Mean ischemia times were longer in the extended group (right 573.9±237.8 vs 343.5±168.1 min; left 613.8±237.3 vs 364.1±151.2 min). There were no significant between-group differences in PGD at 0, 24, 48, or 72 hours. Length of hospitalization, mechanical ventilation duration, and ICU stay were similar. ECMO was required in 4 (10%) of extended storage cases, but none in standard preservation group. There was no difference in the incidence of PGD based on the donor type (DCD vs DBD), regardless of the extended storage at 10°C. Conclusion:Extended static lung preservation at 10 °C was not associated with a statistically significant increase in PGD or adverse short-term clinical outcomes, despite significantly prolonged ischemia times in our cohort. These findings support the feasibility of extended 10 °C preservation without compromising early post-transplant outcomes.

The common cause of porto-pulmonary hypertension and hepato-pulmonary syndrome is portal hypertension. Porto-pulmonary hypertension (PPHTN) is a form of pulmonary arterial hypertension, and hepato-pulmonary syndrome (HPS) occurs as a consequence of hepatic injury or vascular disorders. Demographic characteristics, pathophysiology, screening, differential diagnosis, and treatment of both disorders are treated in this review. Oxygen supply and other medical managements combined with vasodilator drugs are adopted for PPHTN and HPS treatment, but these two clinical conditions also represent an indication for liver transplantation. Despite poor evidence, PPHTN is treated as idiopathic pulmonary arterial hypertension. The latter is combined with improved pulmonary hemodynamics permitting lung transplant. Lung transplant improves PPHTN in one-half of patients and has been associated with longer survival in selected patients. However, the risk of the latter procedure can be relevant as it is closely related to PPHTN severity. Large clinical trials and international guidelines may have a predominant role in increasing our knowledge of both PPHNT and HPS and in improving their outcome by favoring an early diagnosis and more accurate treatment.

Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial lung disease with a highly variable clinical course. Forced vital capacity (FVC) is widely used as a marker of disease severity and progression, yet its variability and dependence on patient effort raise concerns regarding its reliability. Given these limitations, we investigated the clinical significance of slow vital capacity (SVC) as a potential alternative measure of lung function in IPF. In a retrospective cohort of 89 IPF patients who underwent pulmonary function testing with concomitant SVC measurements, we observed a strong correlation between FVC and SVC (r = 0.973 at baseline, r = 0.978 at follow-up). However, in 99

Interstitial lung disease (ILD) complicated by pulmonary hypertension (PH) is associated with poor outcomes. However, real-world data characterizing the hemodynamic profiles of ILD patients undergoing right heart catheterization (RHC) remain limited. We retrospectively analyzed ILD patients who underwent RHC between 2006 and 2024. Hemodynamic profiles were assessed according to the 5th, 6th, and 7th World Symposium on Pulmonary Hypertension (WSPH) definitions and for severe PH (pulmonary vascular resistance [PVR] > 5 Wood units). Correlations between pulmonary function testing (PFT) variables and PVR were explored, and baseline characteristics were compared across groups stratified by PH severity. There were 3541 ILD patients evaluated of whom 12.2% underwent RHC. Among 371 patients with available RHC data and pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg, 49.6%, 54.4%, and 69.4% met the 5th, 6th, and 7th WSPH criteria for precapillary PH, respectively, while 31.3% exhibited severe PH. Correlations between diffusing capacity for carbon monoxide (DLco)%, and forced vital capacity (FVC)%/DLco% ratio with PVR were weak. Our findings highlight the heterogeneous hemodynamic landscape of ILD-PH in clinical practice and underscore the need for heightened vigilance and lower thresholds for RHC. These real-world data can inform future clinical trial design, screening strategies, and management decisions for ILD-PH.

Rationale: Pulmonary hypertension (PH) commonly complicates idiopathic pulmonary fibrosis (IPF). However, the rate of change in pulmonary hemodynamics in IPF remains poorly defined. Objectives: To examine the rate of change in pulmonary hemodynamics among patients with IPF. Methods: The rate of change in mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) was examined in patients with IPF listed for lung transplantation. The fifth and seventh World Symposium on Pulmonary Hypertension definitions for precapillary PH were used in this analysis. Measurements and Main Results: There were 496 patients with IPF who had at least two right heart catheterizations (RHCs) while listed for lung transplantation. The median time between repeated RHCs was 9 months (interquartile range [IQR], 6 to 14 mo). PH was present in 25.8% and 46.8% at the first RHC, whereas 42.9% and 64.3% had PH by the two definitions, respectively, at the time of the final RHC. The median rate of change in the mPAP and PVR were 3.8 mm Hg/yr (IQR, -0.9 to 11.8) and 0.8 Wood units/yr (IQR, -0.2 to 2.4), respectively. The rate of PVR change was slower for those with established PH than those without PH. A total of 28.6% of the patients had accelerated progression of their hemodynamics, arbitrarily defined as an increase in PVR of ⩾2 Wood units/yr. Conclusions: PH associated with IPF tends to progress in an unpredictable fashion, with some patients demonstrating an accelerated phenotype. Among patients with RHC hemodynamics below the threshold for therapy, close vigilance is warranted, with consideration for an early repeat RHC.

Rationale: Little is known about hospitalization in other types of interstitial lung disease (ILD) besides idiopathic pulmonary fibrosis (IPF). Objectives: To determine the frequency of hospitalizations in various types of ILD and elucidate the association of hospitalization with outcomes. Methods: An analysis of the Pulmonary Fibrosis Foundation Patient Registry data was performed. Inpatient hospitalization rates and survival posthospitalization were compared for various types of ILD. Measurements and Main Results: Hospitalization rates were similar across ILD types: 40.6% of participants with IPF, 42.8% of participants with connective tissue disease-related ILD (CTD-ILD), 44.9% of participants with non-IPF idiopathic interstitial pneumonia (IIP), 46.5% of participants with chronic hypersensitivity pneumonitis (CHP), and 53.3% of participants with "other" ILD. All-cause hospitalization was not associated with decreased transplant-free survival (adjusted hazard ratio [AHR], 1.20; 95% confidence interval [CI] = 0.98, 1.46; P = 0.0759) after adjusting for comorbidities and severity of illness; however, respiratory-related hospitalization was (AHR, 1.53; 95% CI = 1.23, 1.90; P = 0.0001). Participants with CTD-ILD (HR, 0.43; 95% CI = 0.25, 0.75; P = 0.0031) and non-IPF IIP (HR, 0.3; 95% CI = 0.15, 0.58; P = 0.005) had a lower risk of death posthospitalization compared with those with IPF, whereas those with chronic hypersensitivity pneumonitis (HR, 0.67; 95% CI = 0.37, 1.20; P = 0.1747) or other ILD (HR, 0.54; 95% CI = 0.19, 1.54; P = 0.25) had a risk comparable with that for IPF. Conclusions: Rates of hospitalization are similar across ILD subtypes. The risk of death or transplant after posthospitalization is lower in patients with CTD-ILD and non-IPF IIP, compared with patients with IPF. In a mixed population of participants with ILD, all-cause hospitalizations were not associated with decreased transplant-free survival; however respiratory-related hospitalizations were.

Background:Right heart catheterization (RHC) is the gold standard for diagnosing pulmonary hypertension (PH) in patients with interstitial lung disease (ILD). However, discrepancies between pulmonary arterial wedge pressure (PAWP) and left ventricular end-diastolic pressure (LVEDP) remain understudied in this population. Methods:We conducted a retrospective analysis of data from ILD patients who underwent RHC and had concomitant LVEDP measurements. Pulmonary vascular resistance (PVR) was calculated using both PAWP and LVEDP. Patients were categorized based on PAWP and LVEDP values using a threshold of 15 mm Hg and PVR values using a threshold of 2 or 3 Wood Units. After that patients were categorized as concordant or discordant if both values were on the same or opposite sides of these thresholds, respectively. A discordantly higher PAWP group (left atrial dysfunction, LAD) was defined as patients with a PAWP-LVEDP difference of more than 3 mm Hg. Results:Among 87 ILD patients, 9 patients (10.3%) showed discordance between PAWP and LVEDP. LAD was observed in 12 patients (13.8%) and was associated with lower forced vital capacity (Odd ratio [OR]: 0.956, p = 0.049) and a larger left atrium diameter (OR: 3.205, p = 0.033). Discordance in PVR values was also noted, with potential treatment targets for PH-specific therapy differing in 9 patients (22.0%) depending on whether PAWP or LVEDP was used. Conclusions:This study highlights the clinical significance of PAWP-LVEDP discrepancies in suspected PH-ILD patients, emphasizing the need for comprehensive assessments incorporating LVEDP and clinical context for accurate diagnosis, risk stratification, and treatment decisions.