Orenitram (treprostinil diethanolamine oral) / United Therapeutics Corp, Supernus Pharma - LARVOL DELTA

DOUBLE TROUBLE: A CASE OF DASATINIB-INDUCED PULMONARY ARTERIAL HYPERTENSION AND RECURRENT CHYLOTHORAX (CHEST 2025) - "She was started on tadalafil, macitentan, and orenitram...Bosutinib was discontinued, and she was started on asciminib, with no further recurrence of effusions... While generally well tolerated, this case highlights rare manifestations of TKI side effects and underscores importance of evaluating pulmonary symptoms that coincide with the initiation of these therapies."

Clinical • Cardiovascular • Chronic Myeloid Leukemia • Congestive Heart Failure • Diabetes • Genetic Disorders • Heart Failure • Hematological Malignancies • Leukemia • Metabolic Disorders • Obesity • Obstructive Sleep Apnea • Pulmonary Arterial Hypertension • Respiratory Diseases • Sleep Disorder • Type 2 Diabetes Mellitus

United Therapeutics Corporation Reports Record Third Quarter 2025 Financial Results (United Therapeutics Press Release) - "Total Tyvaso revenues grew by 10 percent to $478.0 million in the third quarter of 2025, compared to $433.8 million in the third quarter of 2024...Total Tyvaso revenues grew by 10 percent to $478.0 million in the third quarter of 2025, compared to $433.8 million in the third quarter of 2024...The growth in Orenitram revenues resulted primarily from an increase in quantities sold of $11.7 million and, to a lesser extent, a price increase."

Commercial • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Arterial Hypertension

Sotatercept in Severe Pulmonary Arterial Hypertension and Right Heart Failure: Transforming Care and Outcomes (HFSA 2025) - "He was initiated on triple therapy with IV treprostinil, sildenafil, and macitentan...Based on this, we transitioned him from IV treprostinil to oral treprostinil while maintaining sotatercept therapy for long-term disease control. This case underscores the transformative role of sotatercept in managing severe PAH and right-sided heart failure. This case underscores the transformative role of sotatercept in managing severe PAH and right-sided heart failure. By enabling a transition from IV to subcutaneous therapy, sotatercept improved patient's quality of life, 6MWT and mitigated complications of prolonged IV prostacyclin use. The case highlights the need for personalized approaches in PAH care and supports sotatercept as a promising therapeutic adjunct."

Cardiovascular • Congestive Heart Failure • Heart Failure • Hematological Disorders • Infectious Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Thrombosis

Perioperative Optimization for Tonsillectomy and Adenoidectomy in Pediatric Patient with Severe Pulmonary Hypertension and Moderate Obstructive Sleep Apnea (ASA 2025) - "Patient's pulmonary hypertension and right heart failure was managed with a reverse Potts shunt, continuous subcutaneous treprostinil, oral ambrisentan, oral sildenafil, and oral digoxin. Perioperative optimization of this patient required continuation of pulmonary anti-hypertensives, adequate premedication to facilitate controlled intravenous induction, and postoperative sedation to minimize the risk of worsening right heart function during emergence from general anesthesia."

Clinical • Anesthesia • Cardiovascular • Congestive Heart Failure • Heart Failure • Obstructive Sleep Apnea • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sleep Disorder

Reversal of Severe Dasatinib-Induced Pulmonary Arterial Hypertension (HFSA 2025) - "Treatment included immediate dasatinib cessation and guideline directed PAH therapy: subcutaneous treprostinil, oral macitentan, and tadalafil. This case underscores the importance of high suspicion for dasatinib induced PAH in CML patients, particularly those on long-term therapy. Prompt RHC diagnosis, dasatinib discontinuation, and comprehensive targeted therapy are essential for significant clinical improvement and disease reversal."

Cardiovascular • Chronic Myeloid Leukemia • Congestive Heart Failure • Heart Failure • Hematological Malignancies • Interstitial Lung Disease • Leukemia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

United Therapeutics Corporation Reports Record Second Quarter 2025 Financial Results (Businesswire) - "Tyvaso DPI record total revenue of $315 million, reflecting 22 percent growth over the second quarter of 2024....Double-digit, year-over-year revenue growth for Nebulized Tyvaso, Orenitram, and Unituxin with record quarterly revenue for Orenitram; Phase 3 TETON 2 study of Nebulized Tyvaso in idiopathic pulmonary fibrosis is complete; data expected in September 2025; phase 3 TETON 1 data expected in the first half of 2026; Enrollment for the phase 3 ADVANCE OUTCOMES study of ralinepag in pulmonary arterial hypertension is complete; data expected in the first half of 2026."

Enrollment closed • P3 data • Sales • Idiopathic Pulmonary Fibrosis • Neuroblastoma • Neuroendocrine Tumor • Pulmonary Arterial Hypertension

Open Label Treprostinil Raynaud's Study (clinicaltrials.gov) - P4 | N=30 | Not yet recruiting | Sponsor: Brigham and Women's Hospital

New P4 trial • Cardiovascular • Rheumatology

Plain language summary: oral treprostinil (Orenitram) for the treatment of pulmonary arterial hypertension (PAH). (PubMed, Future Cardiol) - No abstract available

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

ARTISAN: A Study of a Mean Pulmonary Artery Pressure-Targeted Approach With Early and Rapid Treprostinil Therapy to Reverse Right Ventricular Remodeling in Participants With Pulmonary Arterial Hypertension (clinicaltrials.gov) - P4 | N=50 | Active, not recruiting | Sponsor: United Therapeutics | Recruiting ➔ Active, not recruiting | Trial completion date: Dec 2027 ➔ Apr 2028 | Trial primary completion date: Dec 2025 ➔ Apr 2026

Enrollment closed • Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

A Case of Pulmonary Tumor Thrombotic Microangiopathy Complicated by Cardiogenic Shock as First Presentation of Metastatic Gastric Adenocarcinoma (ISHLT 2025) - "Due to the presence of severe pulmonary hypertension as well as debility the patient was not a candidate for chemotherapy and opted for inpatient hospice.Summary This patient with PTTM was stabilized with the utilization of VA-ECMO, IV treprostinil and oral sildenafil. However, due to the metastatic signet cell carcinoma, low performance status and severe PH the patient died in hospice few weeks later."

Clinical • Metastases • Cardiovascular • Gastric Adenocarcinoma • Gastric Cancer • Gastroenterology • Gastrointestinal Disorder • Heart Failure • Lung Cancer • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Solid Tumor

TUMORAL PULMONARY HYPERTENSION SECONDARY TO GI MALIGNANCY - Rachel Wester (ACC 2025) - "She was started on milrinone and inhaled epoprostenol and transitioned to IV treprostinil and oral sildenafil. The precise mechanism of PH in patients with malignancy remains to be explored. Histopathological diagnosis with tissue sampling is the gold standard. The optimal management of PTTM is unclear but prior case reports discuss supportive care, chemotherapy, steroids, oxygen and PAH medications."

Cardiovascular • Gastrointestinal Cancer • Hematological Disorders • Lung Cancer • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Rare Diseases • Respiratory Diseases • Thrombocytopenia

ADAPT - a Patient Registry of the Real-world Use of Orenitram® (clinicaltrials.gov) - P=N/A | N=300 | Active, not recruiting | Sponsor: United Therapeutics | Trial completion date: Dec 2023 ➔ Dec 2025 | Trial primary completion date: Apr 2022 ➔ Dec 2025

Real-world evidence • Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

United Therapeutics Corporation Reports Fourth Quarter and Full Year 2024 Financial Results (Businesswire) - "'both TETON studies in idiopathic pulmonary fibrosis are enrolled, facilitating data starting the second half of this year; ralinepag, our potentially best-in-class once-daily oral prostacyclin agonist, will generate data next year'...Total Tyvaso revenues grew by 19 percent to $415.9 million in the fourth quarter of 2024, compared to $350.6 million in the fourth quarter of 2023...Total Tyvaso revenues grew by 31 percent to $1,620.4 million in 2024, compared to $1,233.7 million in 2023...The growth in Remodulin revenues resulted from an increase in U.S. Remodulin revenues, driven by an increase in quantities sold. The growth in Orenitram revenues resulted from an increase in quantities sold and, to a lesser extent, a price increase."

Clinical data • Commercial • P3 data • Idiopathic Pulmonary Fibrosis • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Respiratory Diseases

Transition from parenteral treprostinil to selexipag aiming at hemodynamic targets in pulmonary arterial hypertension. (PubMed, Clin Hypertens) - "Three of these 5 patients reached the hemodynamic target by 12 months and successfully transitioned from parenteral treprostinil to oral selexipag. This study showed that 3 of 5 patients (60.0%) with high-risk PAH reached PVR ≤ 4 WU and/or mPAP ≤ 40 mmHg by receiving intensive parenteral treprostinil therapy with significant improvements in 6MWD and NT-proBNP levels, and successfully transitioned to oral selexipag. We proposed that transition strategies from parenteral treprostinil to selexipag aiming at hemodynamic targets."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

ARTISAN: A Study of a Mean Pulmonary Artery Pressure-Targeted Approach With Early and Rapid Treprostinil Therapy to Reverse Right Ventricular Remodeling in Participants With Pulmonary Arterial Hypertension (clinicaltrials.gov) - P4 | N=50 | Recruiting | Sponsor: United Therapeutics | Trial primary completion date: Dec 2024 ➔ Dec 2025

Trial primary completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

MULTIDISCIPLINARY APPROACH TO PULMONARY ARTERIAL HYPERTENSION THERAPY TRANSITIONS (CHEST 2024) - "Patient was started on epoprostenol IV...Due to gastrointestinal side effects related to treprostinil oral therapy, the decision was made to transition from treprostinil oral therapy to treprostinil inhaled therapy... Multidisciplinary approach and teamwork are the key for successful transition of PAH therapies as PAH therapies are complex, have lots of side effects, and difficult insurance approval processes."

Cardiovascular • Gastrointestinal Disorder • Immunology • Pulmonary Arterial Hypertension • Respiratory Diseases • Scleroderma • Systemic Sclerosis

ORENITRAM's decadal journey: unveiling safety profiles and adverse event through a real-world pharmacovigilance study of FAERS events. (PubMed, Expert Opin Drug Saf) - "These findings highlight the necessity for careful clinical monitoring and effective risk management, particularly with observed gender differences in AE profiles. The study's retrospective nature and reliance on spontaneous reports may affect result generalizability."

Adverse events • Journal • Real-world • Real-world evidence • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

ARTISAN: A Study of a Mean Pulmonary Artery Pressure-Targeted Approach With Early and Rapid Treprostinil Therapy to Reverse Right Ventricular Remodeling in Participants With Pulmonary Arterial Hypertension (clinicaltrials.gov) - P4 | N=50 | Recruiting | Sponsor: United Therapeutics | Trial completion date: Sep 2024 ➔ Dec 2027 | Trial primary completion date: Sep 2024 ➔ Dec 2024

Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Practical management of oral treprostinil in patients with pulmonary arterial hypertension: Lessons from ADAPT, EXPEDITE, and expert consensus. (PubMed, Respir Med) - P=N/A | "Patient selection for, and initiation and titration of oral treprostinil should be individualized and may include parenteral treprostinil induction-transition for faster titration. Assertive side effect management may help reach higher and more efficacious doses of oral treprostinil."

Journal • Cardiovascular • Hypertension • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Pharmaceutical Industry Payments to Physicians and Prescriptions of Oral Pulmonary Arterial Hypertension Medications (ATS 2024) - "Physicians receiving non-research payments from industry related to oral treprostinil and oral selexipag were more likely to prescribe these costly medications associated with high Medicare expenditures, despite payments being overall low value. Investigation is needed to understand the role of industry in shaping physician behavior and practice related to treating PAH."

Cardiovascular • Hypertension • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Sotatercept (Winrevair) for pulmonary arterial hypertension. (PubMed, Med Lett Drugs Ther) - No abstract available

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Comparison of treprostinil and oral sildenafil for the treatment of persistent pulmonary hypertension of the newborn: a retrospective cohort study. (PubMed, Front Pediatr) - "We conducted a retrospective cohort study of 42 neonates with PPHN treated with continuous intravenous treprostinil or oral sildenafil from January 2020 to October 2022 in China. Treprostinil effectively lowers pulmonary artery pressure and improves oxygenation in neonates with PPHN, without being associated with severe complications. It may serve as a beneficial adjunct therapy for neonates with PPHN."

Journal • Retrospective data • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

ADAPT - A Patient Registry of the Real-world Use of Orenitram® (clinicaltrials.gov) - P=N/A | N=300 | Active, not recruiting | Sponsor: United Therapeutics | Trial completion date: Jun 2023 ➔ Dec 2023

Real-world • Real-world evidence • Trial completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Long-Term Experience and Safety of Transitioning from Subcutaneous Treprostinil to Oral Selexipag in the High-Risk Pulmonary Hypertension Patient: A Case Report. (PubMed, Acta Cardiol Sin) - No abstract available

Journal • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Safety Study of UT-15C SR (Oral Treprostinil) in Patients Undergoing a Lower Limb Endovascular Procedure (clinicaltrials.gov) - P2 | N=7 | Completed | Sponsor: Ochsner Health System | N=20 ➔ 7

Enrollment change • Cardiovascular • Peripheral Arterial Disease