riociguat / Generic mfg. - LARVOL DELTA

A study on the accessibility and utilisation of targeted drugs for pulmonary arterial hypertension in China. (PubMed, Front Pharmacol) - "Among all drugs, treprostinil had the highest availability (56.1% across hospitals), while newer drugs such as selexipag had very low availability (3,700 CNY)...Drug accessibility exhibited significant structural imbalances, with some drugs being "affordable but hard to obtain" (e.g., riociguat) and others "highly burdensome and poorly accessible" (e.g., treprostinil). The findings demonstrate significant improvements in the affordability and availability of PAH-targeted therapies over the study period; however, notable inequalities persist in accessibility improvements across urban and rural areas and income groups. Future policies should be tailored to address specific accessibility challenges for different drug categories and focus on overcoming medication access barriers for low-income rural populations to foster health equity."

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

Case Report: Immune checkpoint inhibitor-associated pulmonary hypertension. (PubMed, Front Cardiovasc Med) - "We report a case of a 65-year-old male who developed severe pulmonary arterial hypertension (PAH) following treatment with the programmed cell death protein 1 inhibitor tislelizumab. Following the initiation of a triple combination targeted therapy for PAH comprising macitentan, riociguat, and treprostinil, the patient's condition improved significantly...To date, no specific diagnostic or treatment guidelines exist for this condition due to its rarity. Therefore, there is an urgent need for more case reports, shared experiences, and clinical research to assist clinicians in identifying optimal strategies for the diagnosis and management of this complication."

Checkpoint inhibition • Journal • Cardiovascular • Gastrointestinal Disorder • Hypertension • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Treatment of Exercise-Induced Pulmonary Vascular Dysfunction After Pulmonary Thromboendarterectomy or Balloon Pulmonary Angioplasty (clinicaltrials.gov) - P2 | N=10 | Completed | Sponsor: University of Colorado, Denver | Recruiting ➔ Completed | N=20 ➔ 10 | Trial completion date: Dec 2024 ➔ Jan 2026 | Trial primary completion date: Dec 2024 ➔ Jan 2026

Enrollment change • Trial completion • Trial completion date • Trial primary completion date • Cardiovascular

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

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

REAPPRAISED: Riociguat in Triple Therapy for Pulmonary Arterial Hypertension in Real-World Practice (clinicaltrials.gov) - P=N/A | N=15 | Not yet recruiting | Sponsor: Caio Júlio César dos Santos Fernandes

New trial • Real-world evidence • Cardiovascular • Congestive Heart Failure • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

COMPARATIVE EFFECTIVENESS OF RIOCIGUAT VERSUS SILDENAFIL IN EXERCISE INDUCED PULMONARY HYPERTENSION: A SINGLE-CENTER RETROSPECTIVE STUDY (ACC 2026) - "Abstract is embargoed at this time."

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

Targeting the Stromal Switch: Single-cell profiles implicate the cGMP-PKG pathway in driving fibrosis and cancer progression in Ulcerative Colitis (ECCO-IBD 2026) - "We established a murine model mimicking UC progression through cyclic dextran sulfate sodium (DSS) exposure with azoxymethane (AOM) initiation, evaluating the therapeutic effects of riociguat and sildenafil...Conclusion Our study provides a comprehensive single-cell atlas of the fibrotic and neoplastic UC microenvironment, establishing stromal cGMP-PKG signaling as a pivotal driver of disease progression. We demonstrate the therapeutic potential of repurposing cGMP-elevating drugs, offering a novel strategy to combat devastating UC complications."

Stroma • Immunology • Inflammatory Bowel Disease • Ulcerative Colitis • CD4 • CD8

Current Perspectives on Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension. (PubMed, J Clin Med) - "Recent randomized controlled trials, including the RACE and MR-BPA trials, have demonstrated that BPA results in greater reduction in pulmonary vascular resistance and mean pulmonary arterial pressure compared to riociguat, although with a higher incidence of procedure-related complications...This review synthesizes current evidence on the efficacy, safety, and practical aspects of BPA, while highlighting ongoing challenges, including long-term outcome data, standardization of treatment endpoints, and the role of combination therapy. BPA is poised to play an increasingly central role in personalized care strategies for CTEPH."

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

Global Resource Disparities Between Pulmonary Hypertension Centers: Results From the International Survey by the PVRI IDDI Access to Care Workstream. (PubMed, Pulm Circ) - "Phosphodiesterase type 5 (PDE-5) inhibitors were available in almost all centers but there was limited access to oral, inhaled, and parenteral prostacyclin therapy, riociguat, and selexipag. Survey results suggest significant disparities across the globe. Further research is needed to understand access to PH care and therapies in non-expert/academic centers and regional disparities within countries."

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

Haemodynamic and clinical impacts of switching phosphodiesterase-5 inhibitors to riociguat in patients with chronic thromboembolic pulmonary hypertension (CTEPH) after balloon pulmonary angioplasty (BPA) - a prospective cohort study. (PubMed, BMC Pulm Med) - P4 | "In CTEPH patients after completion of BPA, replacing PDE5i with riociguat significantly enhanced pulmonary haemodynamics and functional capacity but was accompanied by a considerable risk of treatment-related adverse events."

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

Design and Optimization of LOXL2 and sGC Dual-Target Regulators Targeting Extracellular Matrix Dysregulation and Vasodilation for the Treatment of Pulmonary Arterial Hypertension. (PubMed, J Med Chem) - "In a hypoxia-induced PAH rat model, its esterified derivative 9k markedly alleviated vascular remodeling and reduced pulmonary artery pressure, showing efficacy comparable to the combination of riociguat and PAT-1251. Consequently, this study proposes a novel strategy for the development of bifunctional PAH drugs, with a focus on extracellular matrix dysfunction and vasoconstriction, and has identified a promising lead compound."

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

Exercise capacity and oxygen saturation on six-minute walk testing relate to markers of organ dysfunction in adults with sickle cell disease (ASH 2025) - "We used the baseline clinical data from the STERIO-SCDprospective, randomized placebo-controlled Phase 1-2 clinical trial of riociguat for treatment of high-riskpatients with SCD to investigate this hypothesis. Data were obtained as part of the multi-center STERIO-SCD clinical trial... Patients with SCD and systemic hypertension, an elevated TRV and/or proteinuria reflectiveof end-organ damage exhibit lower exercise capacity and ambulatory oxygen saturations. Reducedexercise capacity and oxygen desaturation are associated with a greater degree of organ dysfunction.This suggests that end-organ disease plays a role in limitations of the observed reduced functionalcapacity in patients with SCD and that 6MWT may be a way of identifying higher risk patients who requirefurther evaluation for evidence of cardiopulmonary and renal disease."

Clinical • Cardiovascular • Genetic Disorders • Hematological Disorders • Hypertension • Nephrology • Pulmonary Disease • Renal Disease • Sickle Cell Disease

Survival in patients with chronic thromboembolic pulmonary hypertension treated with riociguat versus other medications: a COMPERA analysis. (PubMed, ERJ Open Res) - "This analysis from the COMPERA registry suggests that in medically treated patients with CTEPH, the soluble guanylate cyclase stimulator riociguat may confer a survival benefit over other PH medications https://bit.ly/3Zq3AIu."

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

Long-term effects of medical treatment in patients with chronic thromboembolic pulmonary hypertension. (PubMed, ERJ Open Res) - "The objective of the current pre-specified analysis was to evaluate the effect of medical treatment, including the approved oral guanylate cyclase stimulator (sGC) riociguat, in patients with or without mechanical intervention...This international CTEPH registry suggests that, although an increasing proportion of patients with CTEPH benefited from mechanical treatment, medical treatment with sGC may be associated with a survival advantage in patients undergoing BPA or no intervention. These observations confirm the results of previous randomised controlled trials in a real-world setting."

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

Vascular diameter and responsiveness to soluble guanylate cyclase modulators: A systematic review of preclinical and clinical evidence. (PubMed, Microvasc Res) - "Current evidence supports a size-dependent pattern of vascular responsiveness to sGC modulators, but inference strength is constrained by heterogeneous methodologies and inconsistent reporting. Future work should implement standardized vessel classification, rigorous biospecimen handling, and transparent methodological documentation to clarify the clinical significance of vessel diameter in sGC-based therapy."

Journal • Preclinical • Review • Cardiovascular

Soluble guanylate cyclase induces cranial vasodilation and headache in adults: a randomized trial. (PubMed, Brain Commun) - "We evaluated whether oral ingestion of riociguat, a stimulator of soluble guanylate cyclase, could elicit cranial vasodilation and headache in healthy adults...These findings support the hypothesis that cranial vasodilation mediated via direct soluble guanylate cyclase stimulation contributes to headache pathogenesis. Further research is warranted to delineate the relative contributions of nitric oxide-dependent versus nitric oxide-independent soluble guanylate cyclase activation, and to evaluate guanylate cyclase signalling as a potential therapeutic target in headache disorders."

Clinical • Journal • CNS Disorders • Migraine • Pain

Chronic thromboembolic pulmonary hypertension - the hidden disease (PubMed, Orv Hetil) - "Early diagnosis and evidence-based management of CTEPH can significantly improve both quality of life and survival. As general practitioners and internists are on the frontline, their awareness and education are crucial for early disease recognition. Orv Hetil. 2025; 166(48): 1908-1915."

Journal • Review • Cardiovascular • Cognitive Disorders • Congestive Heart Failure • Heart Failure • Hematological Disorders • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Comparing bridging therapies prior to balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension (BTS WM 2025) - "Medical therapies including phosphodiesterase-5 inhibitors (PDE5i), endothelin receptor antagonists (ERA) and riociguat are commonly used as bridging therapies to BPA, but data comparing these therapies are limited...These differences persisted following correction for baseline differences. As improvements in haemodynamic parameters are associated with improved BPA safety profile, bridging with dual therapy may enhance BPA safety, although more work is needed to validate this hypothesis."

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

Balloon Pulmonary angioplasty versus riociguat in inoperable chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis of randomised controlled trials (BTS WM 2025) - "Conclusion BPA offers superior hemodynamic and biomarker improvements over riociguat in inoperable CTEPH, whereas riociguat enhances cardiac output. A patient-specific approach considering feasibility and treatment goals may optimise outcomes."

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

Life-Threatening Hypotension From Supplement-Riociguat Interaction: A Clinical Caution. (PubMed, Am J Ther) - No abstract available

Journal • Hypotension

A Case Report of Pulmonary Arterial Hypertension Associated With Hereditary Hemorrhagic Telangiectasia Successfully Treated With Riociguat. (PubMed, Cureus) - "In this case, treatment with riociguat improved pulmonary vascular resistance (PVR) without exacerbating epistaxis or causing other adverse effects. These findings suggest that riociguat may represent a promising therapeutic option for HHT-associated pre-capillary PH."

Journal • Cardiovascular • CNS Disorders • Hematological Disorders • Hypertension • Portal Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Insights from systematic reviews (2019-2024) and drug interaction database analysis in people with HIV and comorbidities. (PubMed, Int J STD AIDS) - "Severe adverse drug reactions associated with ARTs, including efavirenz, darunavir, nevirapine, and atazanavir-ritonavir, especially when combined with treatments for TB and malaria. Key interactions included reduced drug levels from rifampicin and QT prolongation from artemether-lumefantrine...Database discrepancies were noted, especially for riociguat interactions and ritonavir through inhibition of P-gp or OATP1B1 functions.ConclusionsDDIs in PWH receiving ART with comorbidities have highlighted the crucial need for personalized treatment. Incorporating pharmacokinetic, pharmacodynamic, and pharmacogenomic factors is essential for optimizing therapy outcomes."

Journal • Cardiovascular • Human Immunodeficiency Virus • Hypertension • Infectious Disease • Malaria • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Tuberculosis

Marked improvement in severe pulmonary arterial hypertension following airway infection in a patient with a heterozygous BMP9 nonsense mutation: a case report. (PubMed, Eur Heart J Case Rep) - "Despite receiving combination therapy with macitentan, riociguat, and intravenous epoprostenol, she continued to have severe pulmonary hypertension with suprasystemic pressure [mean pulmonary artery pressure (PAP) = 65 mmHg] for 4 years following treatment initiation. A patient with severe, treatment-resistant pulmonary hypertension exhibiting a dramatic improvement in pulmonary pressure following an AI is exceedingly rare. The present case may provide new insights into the pathophysiology of PAH."

Journal • Cardiovascular • Hypertension • Infectious Disease • Inflammation • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Riociguat Alleviates Cisplatin-Caused Kidney Injury by Suppressing Oxidative Stress and Inflammation. (PubMed, Biology (Basel)) - "These results indicate that riociguat exerts protection of the kidneys from CP-caused kidney damage by antioxidation and anti-inflammation. Riociguat may have potential as an adjunct therapy to mitigate CP-associated nephrotoxicity."

Journal • Acute Kidney Injury • Inflammation • Nephrology • Oncology • Renal Disease • CAT • IL1B • IL6 • TNFA

SICKLE CELL DISEASE LEADING TO DEBILITATING PULMONARY ARTERIAL HYPERTENSION (CHEST 2025) - "Managing SCD-associated PH primarily focuses on treating the underlying disease with hydroxyurea or chronic blood exchange transfusions, along with anticoagulation for thromboembolism and treatment of left heart failure in those with post-capillary PH...Given clinical volume overload, she was started on torsemide...Although intended to be started on macitentan (an endothelin receptor antagonist), she returned to the hospital with worsening dyspnea before she could get the medication approved...She then became increasingly hypotensive riociguat was uptitrated, requiring initiation of midodrine as well as downtitration of diuresis... SCD-associated PH carries a high mortality risk with a notable lack of evidence on effective therapy options that demonstrate significantly improved outcomes."

Cardiovascular • Congestive Heart Failure • Genetic Disorders • Heart Failure • Hematological Disorders • Hypotension • Pulmonary Arterial Hypertension • Respiratory Diseases • Sickle Cell Disease