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.

Background:Cytopenias are the hallmark manifestation of myelodysplastic syndrome (MDS) as patients frequently suffer from transfusion-dependent anemia. Luspatercept is part of a new family of drugs approved for transfusion-dependent MDS that bind transforming growth factor-β (TGF-β) family ligands to decrease SMAD signaling, resulting in an increase of circulating mature red cells. However, disruption of the TGF-β/SMAD pathway carries risks given its wide-ranging influence on the development and structure of blood vessels. Here we present the case of a patient with MDS presenting with refractory hypoxia from an intrapulmonary shunt secondary to the development of microvascular pulmonary arteriovenous malformations (PAVM) after starting luspatercept. Case Description:The patient reported progressive shortness of breath while receiving luspatercept and was eventually referred to a pulmonary clinic who discovered the shunt via a bubble echocardiogram. A broad cardiac and autoimmune work-up was unremarkable. Her hypoxia was out of proportion to spirometric and imaging findings. A computed tomography (CT) pulmonary angiogram did not reveal any large vascular malformations. Conclusions:The decision was made to stop the drug and her hypoxemia resolved within weeks in parallel with resolution of the shunt as the patient returned to room air and her prior functional baseline. In this report, the complex mechanism of luspatercept is discussed with a literature review on the clinical trials leading to its approval in MDS patients. Furthermore, we connect the pathophysiology between the formation of this patient's telangiectasias leading to her PAVM with the disruption of the TGF-β/SMAD pathway from luspatercept use.

Introduction: Hermansky-Pudlak syndrome (HPS) is a rare, autosomal recessive disorder characterized by abnormalities in lysosome-related organelles. Early onset pulmonary fibrosis is a progressive, fatal manifestation in many patients. Challenges exist in transplanting this population due to their underlying bleeding diathesis and potential alloimmunization. Here we report the clinical course and outcomes for three patients transplanted with HPS.

A spontaneous pneumothorax may be the heralding manifestation of diffuse cystic lung disease (DCLD). Historically, these diagnoses were differentiated by unique clinical, radiographic and tissue pathology characteristics. With recent advancements in genomics, several forms of DCLD can now be diagnosed through genetic testing and patients can thereby avoid undergoing an invasive lung biopsy. We present a case of a young patient with recurrent spontaneous pneumothoraces associated with a rare DCLD, Birt-Hogg-Dubé syndrome, that exemplifies the manifestations of this disease through classic history, imaging and pathology, along with the diagnostic utility of novel genotypic technology in the modern era.

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.

Purpose: Bilateral lung transplantation (BLTx) can be performed either via clamshell thoracotomy (CT) or median sternotomy (MS). CT has been the incision of choice for the last few decades due to easier access. MS, however, has the benefits of less postoperative pain and reduced length of hospitalization. We sought to evaluate differences in postoperative pain, hospital length of stay, and risk of postoperative infection in patients who underwent BLTx at our center.

Introduction: Hyperammonemia syndrome (HS) is a rare complication in lung transplantion associated with increased ammonia production in the context of infection by Ureaplasma and Mycoplasma spp. Cases typically occur within two weeks of transplant, manifested by encephalopathy, cerebral edema, and seizure. Mortality is high, even with early recognition and treatment. Here we report a case of delayed HS four months after a bilateral lung transplant (BLTx) for idiopathic pulmonary fibrosis (IPF).

Introduction: Post-Transplant Lymphoproliferative Disorder (PTLD) is a common complication seen after solid organ transplantation. Manifestations can vary widely based on the degree of disease and organ involvement. We present a case of an asymptomatic lung transplant patient who was diagnosed with PTLD after discovery of a liver lesion on chest imaging surveillance.