Adempas (riociguat) / Bayer, Merck (MSD) - LARVOL DELTA

Long-Term Prospective Observation Study of Patients with Pulmonary Arterial Hypertension Associated with Connective Tissue Diseases. (PubMed, Kardiologiia) - "The use of macitentan and/or riociguat, as monotherapy or in combination with another PAH-specific drug, significantly reduced the 5-year risk of fatal outcome (OR 0.38 [0.16; 0.89], p=0.027).Conclusion    The survival of patients with PAH-IIRD remains low. Further studies aimed at finding new pathogenetic targets are needed; the use of modern PAH-specific drugs (macitentan and/or riociguat) modifies the course of the disease, increasing the survival."

Journal • Observational data • Cardiovascular • Hypertension • Immunology • Inflammatory Arthritis • Lupus • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatoid Arthritis • Rheumatology • Scleroderma • Sjogren's Syndrome • Systemic Lupus Erythematosus • Systemic Sclerosis

Expert consensus on approaches to risk stratification and choice of therapy in patients with inoperable and residual chronic thromboembolic pulmonary hypertension (PubMed, Ter Arkh) - "It was considered that a strong consensus was achieved with a mean score of M±m 2.5±2, and with a mean score below 2.5, consensus was not achieved. A strong consensus was reached on all issues."

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

Soluble Guanylate Cyclase Stimulator Mediated Tgf-β Induced Autophagy Related to Chronic Thromboembolic Pulmonary Hypertension (ATS 2025) - "These findings suggest that targeting autophagy may offer novel therapeutic strategies for CTEPH.These findings suggest autophagy is central to CTEPH pathogenesis and may serve as a therapeutic target. Modulating autophagy through agents like riociguat or targeting the TGF-β/Smad3 pathway may offer novel treatment strategies for CTEPH."

Cardiovascular • Fibrosis • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases • BECN1 • SMAD3 • TGFB1

Pulmonary Arteriovenous Malformations in BMPR2-Associated Pulmonary Arterial Hypertension (ATS 2025) - "At the time of her relocation, she was on triple oral therapy with riociguat, selexipag, and macitentan with low-risk by REVEAL score and mildly decreased right ventricular function on echocardiography. Whether sotatercept in these patients can worsen pre-existing AVMs is unknown. Figure 1."

Cardiovascular • CNS Disorders • Heart Failure • Hematological Disorders • Myasthenia Gravis • Pulmonary Arterial Hypertension • Respiratory Diseases • ACVR2A

Complex Case of Melena and Epistaxis in a Patient With Advanced Pulmonary Sarcoidosis and Pulmonary Hypertension: A Multidisciplinary Approach to Management (ATS 2025) - "Two weeks prior to admission he was initiated on apixaban for pulmonary emboli...CTEPH warrants evaluation for surgical endarterectomy or pulmonary arterial balloon angioplasty, or if patient is not a candidate for either, utilization of riociguat to stimulate soluble guanylate cyclase and subsequent vasorelaxation...This case highlights the multifactorial nature of pulmonary hypertension in some patients. As such, a tailored approach to therapy is essential to address the underlying causes, and in refractory cases lung transplant may be necessary."

Clinical • Metastases • Cardiovascular • Congestive Heart Failure • Gastroenterology • Gastrointestinal Disorder • Heart Failure • Hepatology • Immunology • Peptic Ulcer • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases • Sarcoidosis • Vasculitis

Initial Experience and Short-term Outcomes of Pulmonary Endarterectomy for Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Patients in a New CTEPH Program in the United Arab Emirates (ATS 2025) - "Prior to PEA, all patients were treated with dual PH therapy - Riociguat and Macitentan...All patients were successfully weaned off PH medications and continued on anticoagulation with Warfarin. Our result shows an excellent outcome with no perioperative mortality and excellent improvement in hemodynamics and echocardiographic findings. PEA is a potentially curable treatment option for carefully selected CTEPH patients by skilled multidisciplinary team."

Clinical • Cardiovascular • Fibrosis • Genetic Disorders • Hematological Disorders • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Amphetamine Induced Group I Pulmonary Hypertension: A Case Report of Unforseen Consequences (ATS 2025) - "This abstract is funded by: None Introduction: Pulmonary hypertension (PH) is a medical condition in which there is hypertension in the lungs. In patients classified as intermediate-high risk based on their REVEAL score, PAH therapy should be initiated with endothelin receptor antagonist (bosentan), phosphodiesterase type 5 inhibitor (sildenafil), soluble guanylate cyclase stimulator (riociguat), prostanoid (epoprostenol), and/or prostacyclin agents (selexipag, and treprostinil). Treatment of PAH can be challenging as hemodynamics can be compromised with induction of these vasodilative agents; however, is necessary to prevent disease progression."

Case report • Clinical • Asthma • Cardiovascular • CNS Disorders • Congestive Heart Failure • Endocrine Disorders • Gastrointestinal Disorder • General Anxiety Disorder • Heart Failure • Hypertension • Immunology • Mood Disorders • Nephrology • Nicotine Addiction • Psychiatry • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases • Septic Shock • Tobacco Addiction

Patients With History of Methamphetamine Use Are More Likely to Have Risk Evaluation and Mitigation Strategy Medications for Pulmonary Arterial Hypertension Stopped Due to Non-adherence (ATS 2025) - "Patients with PAH were included if they were prescribed at least one of the REMS drugs (riociguat, macitentan, ambrisentan) and had an office visit with a pulmonary hypertension specialist during the study period. Methamphetamine-associated PAH remains challenging to manage. Patients with Meth-PAH are more likely to be non-adherent to REMS drugs and active use further increases the likelihood of non-adherence. Males with Meth-PAH appear more likely to be non-adherent to REMS drugs than females."

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

Invasive Hemodynamics in the Management of Sarcoidosis Associated Pulmonary Arterial Hypertension (ATS 2025) - "Notably this was her sixth admission since February 2023 when she was started on macitentan and riociguat. The patient's PAH medications were discontinued. Sarcoidosis, especially with mixed cardio-pulmonary disease, is difficult to manage without hemodynamic assessment."

Cardiovascular • Congestive Heart Failure • Heart Failure • Hematological Disorders • Immunology • Obstructive Sleep Apnea • Pulmonary Arterial Hypertension • Pulmonary Embolism • Respiratory Diseases • Sarcoidosis • Sleep Disorder • Ventricular Tachycardia

Persistence of Pulmonary Hypertension Symptoms After Two PTEs in a Patient With CTEPH (ATS 2025) - "In April 2016, she had another pulmonary embolism on rivaroxaban and was diagnosed with positive lupus anticoagulant...She started macitentan and riociguat and continued enoxaparin, but symptoms continued to worsen...Our case demonstrates that patients can still have CTEPH symptoms after a repeat PTE while on anticoagulation and PH medications. These patients might require interventions like balloon pulmonary angioplasty while continuing medical therapy and anticoagulation."

Clinical • Cardiovascular • Immunology • Infectious Disease • Inflammatory Arthritis • Novel Coronavirus Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

The Phourth Pathway in PAH: Experience in the "Underserved" Central Valley of California (ATS 2025) - "Sotatercept (SOT) is the new activin inhibitor recently added as the fourth pathway...Case 1: A 45-year-old African-American female with WHO group 1 CTD-PAH reported worsening symptoms despite triple therapy (Parenteral prostacyclin therapy (PPT) + Endothelin receptor agonist (ERA) + Tadalafil (TAD)...Case 3: A 53-year-old Hispanic male with IPAH was on Triple combo treatment (Oral prostacyclin + ERA + Riociguat (Rio))... These cases illustrate favorable responses to SOT in patients with progressive PAH. Often, Symptom improvement was perceived as soon as after 1st dose. Of 4 patients, SOT had to be stopped in 1 patient due to significant Thrombocytopenia despite favorable clinical response."

Cardiovascular • Congestive Heart Failure • Heart Failure • Hematological Disorders • Immunology • Inflammatory Arthritis • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sjogren's Syndrome • Thrombocytopenia

ATMOS, A Proof-of-Concept Trial of Inhaled Mosliciguat, A First-in-Class Soluble Guanylate Cyclase Activator - A Novel Approach to Address Unmet Needs in PH (ATS 2025) - "Phosphodiesterase 5 inhibitors and riociguat, an sGC stimulator, are approved for PH, but highly synergistic with functional, NO-responsive, heme-containing sGC. Mosliciguat had favorable tolerability and resulted in a sustained, substantial reduction in PVR after a single inhaled dose."

Cardiovascular • Fatigue • Inflammation • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Comparative Efficacy of Pharmacological Treatments for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Network Meta-analysis (ATS 2025) - " A comprehensive search was conducted on PubMed, Cochrane, PloSOne, ScienceDirect, Google Scholar, clinicaltrials.gov up to october 2024.Seven randomized control trials comparing seven treatments: Bosentan(A), Sildenafil(B), Macitentan(C), Riociguat(D), Ambrisentan(E) and Selexipag(F) to placebo were included. This network meta-analysis compares pharmacological treatments for inoperable CTEPH. The findings indicate that Bosentan is the most effective treatment for reducing pulmonary vascular resistance (PVR) and NT-proBNP levels, while Riociguat shows the most significant improvement in 6MWD. These results provide insights into the efficacy of available therapies, guiding clinical decision-making for patients with inoperable CTEPH."

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

Different Effects of Riociguat and Vericiguat on Pulmonary Vessels and Airways. (PubMed, Biomedicines) - "These properties could make sGC stimulators useful for treating diseases associated with endothelial dysfunction. In the future, vericiguat may provide an alternative treatment to induce bronchodilation in respiratory diseases."

Journal • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Inflammation • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • EDN1 • IL6

Riociguat-Discontinue Effects on Right HEART in CTEPH (RED-HEART) (clinicaltrials.gov) - P3 | N=68 | Recruiting | Sponsor: Suqiao Yang | Active, not recruiting ➔ Recruiting | Phase classification: P4 ➔ P3

Enrollment open • Phase classification • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

CHMP post-authorisation summary of positive opinion for Adempas (X-41) (European Medicines Agency) - "On 25 April 2025, the Committee for Medicinal Products for Human Use (CHMP) adopted a positive opinion, recommending a change to the terms of the marketing authorisation for the medicinal product Adempas....The CHMP adopted the addition of a new pharmaceutical form, with a new strength (Adempas 0.15 mg/ml granules for oral suspension), along with a change to an existing indication to include treatment of children aged 6 years and older with pulmonary arterial hypertension..."

CHMP • Pulmonary Arterial Hypertension

Riociguat-Discontinue Effects on Right HEART in CTEPH (RED-HEART) (clinicaltrials.gov) - P4 | N=68 | Active, not recruiting | Sponsor: Suqiao Yang

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

Vericiguat: A Promising Drug for the Treatment of Heart Failure. (PubMed, Curr Cardiol Rev) - "Riociguat was the first sGC stimulator used in pulmonary hypertension therapy, but its short half-life required multiple dosing, making it unsuitable for cardiovascular diseases. Its compatibility with other heart failure therapies without significant drug-drug interactions further highlights its potential as a cornerstone treatment. Ongoing studies continue to explore its benefits, suggesting that vericiguat may enable more comprehensive and effective management of heart failure, reducing the burden of this debilitating condition."

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

Giant Pulmonary Artery Aneurysm Compressing Left Lung and Pulmonary Drainage (ISHLT 2025) - "History was notable for pulmonary hypertension (PH), attributed to chronic thromboembolism, treated with selexipag 1600 mcg BiD, riociguat 2.5 mg TiD, and macitentan 10 mg. Palliative medicine was engaged, and she decided to pursue transplant evaluation elsewhere.Summary Giant PAAs carry major implications for transplant surgery and potential need for multiorgan transplant. Secondary PR is often associated with rapid deterioration and timely referral for advanced therapies should be considered in PH patients with giant PAA."

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

Effect of discontinuation of riociguat on right heart function in patients with chronic thromboembolic pulmonary hypertension after combination therapy (ChiCTR) - P4 | N=68 | Not yet recruiting | Sponsor: Beijing Chao-Yang Hospital, Capital Medical University; Beijing Chao-Yang Hospital, Capital Medical University

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

CONSIDERATION OF CLOSURE OF A PATENT DUCTUS ARTERIOSUS COMPLICATED BY EISENMENGER SYNDROME - Eric Bailey (ACC 2025) - "Given the presence of severe PH she was initiated on riociguat, ambrisentan, and selexipag. Eisenmenger physiology is not an absolute contraindication to PDA closure in the right clinical setting. The shunt in this case reversed evidenced by her recurrent pulmonary edema and significant left-to-right shunting, facilitating the feasibility of this procedure."

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

EFFECTS OF COMBINED RIOCIGUAT AND ENDOTHELIN RECEPTOR ANTAGONIST THERAPY IN CHINESE PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION - Anqi Duan (ACC 2025) - "Combination therapy with riociguat and ERA enhanced exercise capacity, hemodynamic status, and risk stratification in patients with PAH."

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

STROKE IN RESOLVING CTEPH: CLINICAL IMPLICATIONS OF A PERSISTENT RIGHT-TO-LEFT SHUNT - Ayesha T. Jalal (ACC 2025) - "Patient was discharged on riociguat and dose-reduced apixaban. Our case highlights a gap in literature for managing CTEPH with right to left shunting. Initial TTE in cases of massive PE should be done with bubble study. Intra cardiac shunting should be followed up with repeat EBS in 2-3 months."

Clinical • Anemia • Cardiovascular • Gynecology • Hematological Disorders • Ischemic stroke • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Discovery of the first isoform-specific sGC activator: selective activation of GC-1 by runcaciguat. (PubMed, Eur J Pharmacol) - "Drug research and development programmes targeting soluble guanylyl cyclase (sGC) have been highly successful, leading to the launch of the sGC stimulators riociguat for pulmonary hypertension (2014) and vericiguat for chronic heart failure (2021). Notably, runcaciguat is the first sGC activator identified to selectively target GC-1 at therapeutic concentrations. This selective targeting of isoforms not only opens avenues for new therapeutic strategies but also provides an alternative to knockout animal models for investigating isoform-specific functions."

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

Management of Pulmonary Arterial Hypertension: Current Strategies and Future Prospects. (PubMed, Life (Basel)) - "Current therapies are categorized based on targeting different pathways known to contribute to PAH, including endothelin receptor antagonists (ERAs), phosphodiesterase-5 inhibitors (PDE-5 inhibitors), prostacyclin analogs, soluble guanylate cyclase stimulators, and activin signaling inhibitors such as Sotatercept. The latest addition to treatment options is soluble guanylate cyclase stimulators, such as Riociguat, which directly stimulates the nitric oxide pathway, facilitating vasodilation...Besides prevailing therapies, emerging PH treatments target growth factors and inflammation-modulating pathways, with ongoing trials assessing their long-term benefits and safety. Hence, this review explores current therapies that delay progression and improve survival, as well as future treatments with curative potential."

Journal • Review • Cardiovascular • Gene Therapies • Hypertension • Inflammation • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • TGFB1