Uptravi (selexipag) / J&J, Nippon Shinyaku - LARVOL DELTA

Simultaneous Liver-Kidney Transplantation in the Setting of Severe Port-Pulmonary Hypertension (ACG 2026) - "She was on tadalafil 40 mg daily and inhaled treprostinil 80 mcg; prior trials of selexipag and macitentan were discontinued due to intolerance and worsening anemia, respectively. Sotatercept was contraindicated given thrombocytopenia...Despite intolerance or contraindication to several standard pulmonary vasodilators, the patient was bridged to transplant with intravenous treprostinil, renal replacement therapy and careful volume management, to meet hemodynamic listing criteria. This case illustrates the importance of individualized, dynamic risk assessment in PoPH patients."

Cardiovascular • Fibrosis • Genetic Disorders • Hematological Disorders • Hepatology • Immunology • Nephrology • Polycystic Kidney Disease • Portal Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Thrombocytopenia • Thrombosis • Transplantation

Sotatercept Versus Other Add-on Therapies in Pulmonary Arterial Hypertension: a Bayesian Network Meta-Analysis (ERS 2026) - "Oral Treprostinil (MD 17m), Inhaled Iloprost (MD 26.0m), Selexipag (MD 8.3m), Ralinepag (MD 7.3m), and MK-5475 (MD -5.7m) showed non-significant effects (Figure 1). Sotatercept demonstrated the highest probability of being the most effective add-on therapy for improving 6MWD in PAH, supporting its positioning as preferred add-on for patients not achieving treatment goals."

Retrospective data • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Real-world safety and efficacy of selexipag in pulmonary arterial hypertension with pulmonary disease (ERS 2026) - "Selexipag improved pulmonary hemodynamics in patients with PAH and concomitant lung disease, and improved exercise capacity in those with ILD. With careful dose titration, selexipag was well tolerated without severe adverse events."

Clinical • Real-world • Real-world evidence • Bronchiectasis • Cardiovascular • Chronic Obstructive Pulmonary Disease • Immunology • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

A CASE OF PULMONARY VENOOCCLUSIVE DISEASE EXACERBATED BY SELEXIPAG THERAPY FOR PULMONARY ARTERIAL HYPERTENSION (CHEST 2026) - No abstract available

Clinical • Cardiovascular • Hepatology • Pulmonary Arterial Hypertension • Respiratory Diseases

Selexipag in systemic sclerosis-associated pulmonary arterial hypertension: long-term real-world data from a multicentre Italian cohort. (PubMed, Rheumatology (Oxford)) - "Selexipag demonstrated a favourable long-term safety profile in SSc-PAH patients. A preserved right ventricular function represents a key determinant for survival, while early therapeutic combination strategies including selexipag are associated with more favourable COMPERA 2.0 mortality risk."

Journal • Real-world evidence • Cardiovascular • Hypertension • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

Real-World Experience of Midodrine in Hospital Setting in Pulmonary Arterial Hypertension. (PubMed, CHEST Pulm) - "Patients on midodrine during hospitalization received higher doses of epoprostenol (P < .001), treprostinil (P < .05), and selexipag (P < .05). This study, to our knowledge the first large-scale analysis of PAH data, investigated the use of midodrine in hospitalized patients with PAH. In this single-center study, we share real-world experience of using midodrine to mitigate systemic hypotension, thereby facilitating the uptitration of PAH-targeted therapies."

Journal • Real-world evidence • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Hypotension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Outcomes of transition from selexipag to parenteral prostacyclin analogues in patients with pulmonary arterial hypertension. (PubMed, ERJ Open Res) - "Outcomes are poor among those within this population who do not achieve a low-risk status after the transition. https://bit.ly/4sMY3aF."

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

Comparable exercise hemodynamic improvements with riociguat and selexipag under multimodal therapy including balloon pulmonary angioplasty in inoperable chronic thromboembolic pulmonary hypertension (ESC 2026) - "In patients with inoperable CTEPH undergoing multimodal treatment including BPA, both riociguat and selexipag significantly improved resting and exercise hemodynamics. Selexipag demonstrated efficacy comparable to riociguat, suggesting that effective hemodynamic improvement can also be achieved with selexipag in this clinical setting."

Cardiovascular • Pulmonary Embolism

Extended prospective follow-up of a randomised comparison of riociguat and selexipag under multimodal therapy in inoperable chronic thromboembolic pulmonary hypertension (ESC 2026) - "The mean pulmonary arterial pressure–cardiac output slope decreased from baseline to post-BPA in both groups (riociguat: 11.59 [IQR 6.91–16.68] to 4.4 [3.74–7.94]; selexipag: 9.87 [6.76–14.9] to 4.68 [4.02–5.88]; both p = 0.031).ConclusionBoth riociguat and selexipag demonstrated significant hemodynamic improvements in treatment-naïve patients with inoperable CTEPH before and after BPA. Selexipag showed efficacy comparable to riociguat within a multimodal treatment strategy, supporting its role as an effective pulmonary vasodilator in this setting."

Clinical • Cardiovascular • Pulmonary Embolism

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3 | N=280 | Recruiting | Sponsor: Actelion | Trial completion date: Jul 2029 ➔ Sep 2030

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

Implementation and use of risk minimization control tools in the EU: A multistakeholder survey. (PubMed, Br J Clin Pharmacol) - "RMCT implementation was generally perceived as feasible, although regulatory implementation was more challenging than integration into healthcare. Successful implementation may benefit from flexible design, alignment with existing systems, feasibility assessment, stakeholder engagement and clear guidance."

Journal

PHoenix: Pulmonary Hypertension: Intensification and Personalisation of Combination Rx (clinicaltrials.gov) - P4 | N=70 | Active, not recruiting | Sponsor: Sheffield Teaching Hospitals NHS Foundation Trust | Recruiting ➔ Active, not recruiting | N=40 ➔ 70 | Trial completion date: Jan 2027 ➔ Jan 2028 | Trial primary completion date: Jan 2027 ➔ Jan 2028

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

Transitions From Parenteral Prostacyclin Analogues to Selexipag in Patients With Pulmonary Arterial Hypertension. (PubMed, Pulm Circ) - "Pulmonary arterial hypertension (PAH) is a progressive illness that may require therapy with parenteral prostacyclin pathway agents (PPA) (epoprostenol and treprostinil). All patients survived to outpatient pulmonary hypertension follow up (median 5.5 weeks, IQR 1.4-9.6 weeks), and the majority were alive at 1-year post-transition (n = 32, 91%). Based on these results, we conclude that transition from parenteral PPA's to selexipag can be done in the inpatient setting safely, quickly, and effectively."

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

A Study of Inhaled Treprostinil (LIQ861) in Adults With Pulmonary Arterial Hypertension Transitioning From Oral Selexipag (clinicaltrials.gov) - P4 | N=20 | Not yet recruiting | Sponsor: Liquidia Technologies, Inc.

New P4 trial • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Pharmacovigilance assessment of gout: a real-world study using the FAERS database. (PubMed, Front Pharmacol) - "These include Lenalidomide, Sacubitril valsartan, Ruxolitinib, Treprostinil, Octreotide, Selexipag, Rosuvastatin, Sitagliptin, Riociguat, Epoprostenol, Patiromer, Dasabuvir ombitasvir paritaprevir ritonavir, Tafamidis, Sparsentan, and Iloprost. These findings suggest the potential for targeted monitoring of drug-associated gout in clinical practice. When administering these medications, it may be crucial to regularly assess patients' uric acid levels and maintain heightened awareness for the possible onset of gout."

Adverse events • Journal • Real-world evidence • Cardiovascular • Gout • Hematological Disorders • Hematological Malignancies • Hypertension • Inflammatory Arthritis • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology

ASCEND-PAH: Randomized Study of Triple Therapy vs Sildenafil Dose Optimization in Pulmonary Arterial Hypertension (clinicaltrials.gov) - P4 | N=196 | Recruiting | Sponsor: University of Sao Paulo General Hospital

New P4 trial • Cardiovascular • Congestive Heart Failure • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Towards an integrated and proactive management of pulmonary hypertension in systemic sclerosis: a practical approach for early diagnosis and optimal patient management. (PubMed, Rheumatol Adv Pract) - "In SSc patients already receiving a dual combination, escalation to triple therapy including selexipag, or in selected cases switching to riociguat, should be promptly considered. Structured referral networks, implementation of the DETECT algorithm and involvement of a dedicated case manager and/or nurse can further improve timely diagnosis and continuity of care. Optimizing SSc-PAH management requires a proactive, integrated approach that bridges rheumatology and cardiology expertise."

Journal • Review • Cardiovascular • Hypertension • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Scleroderma • Systemic Sclerosis

Aging-driven transcriptional programs in diabetic kidney disease: multi-omics discovery of diagnostic biomarkers and drug-repurposing targets. (PubMed, Clin Epigenetics) - "In this study, we identified age-related hub genes, constructed a reliable diagnostic signature, and suggested SYK-fostamatinib and APOC3-selexipag as potential drug-repurposing candidates for DKD, which requires further experimental and clinical validation."

Biomarker • Journal • Chronic Kidney Disease • Diabetes • Diabetic Nephropathy • Nephrology • Renal Disease • EGF • RB1 • RPA1 • SYK

Withdrawal of Prostacyclin Pathway Therapy in Patients With Pulmonary Arterial Hypertension Receiving Sotatercept (WATERLOO) (clinicaltrials.gov) - P=N/A | N=78 | Not yet recruiting | Sponsor: University of Alberta

Head-to-Head • New trial • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3 | N=252 | Recruiting | Sponsor: Actelion | Not yet recruiting ➔ Recruiting

Enrollment open • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3; N=252; Not yet recruiting; Sponsor: Actelion

Clinical • New P3 trial • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3 | N=280 | Recruiting | Sponsor: Actelion | Trial completion date: Feb 2028 ➔ Sep 2029

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

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3 | N=230 | Recruiting | Sponsor: Actelion | Trial completion date: Jan 2027 ➔ May 2027

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

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3 | N=280 | Recruiting | Sponsor: Actelion | Trial completion date: Mar 2029 ➔ Sep 2030

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

PLATYPUS: A Study Providing Treatment Access in Participants With Pulmonary Hypertension Completing a Parent Study and Having no Other Option (clinicaltrials.gov) - P3 | N=230 | Recruiting | Sponsor: Actelion | Trial completion date: May 2027 ➔ Feb 2028

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