riociguat / Generic mfg. - LARVOL DELTA

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

DE-ESCALATION OF THERAPY IN THE SETTING OF SOTATERCEPT IN PULMONARY ARTERIAL HYPERTENSION PATIENTS ON PREVIOUSLY STABLE PARENTERAL PROSTACYCLIN (CHEST 2025) - "CASE PRESENTATION: A 38-year-old female with Ehlers-Danlos and World Health Organization (WHO) group 1 PAH on triple therapy (subcutaneous treprostinil 30 ng/kg/min, macitentan, riociguat) was started on sotatercept...Six minute walk distance (6MWD) improved (460m) as did her functional class (II).A 55-year-old female with methamphetamine-associated PAH on triple therapy (epoprostenol 16.5ng/kg/min, macitentan, riociguat) was started on sotatercept...Epoprostenol was tapered off over 2 days and was transitioned selexipag... These cases highlight the importance of regular follow-up, monitoring, and dose modifications to PAH therapies. Further studies are needed to evaluate the long-term impact of sotatercept and its interactions with other PAH therapies."

Clinical • Cardiovascular • Endocrine Disorders • Infectious Disease • Nephrology • Novel Coronavirus Disease • Pneumonia • Pulmonary Arterial Hypertension • Renal Disease • Respiratory Diseases

Molecular Aspects of Soluble Guanylate Cyclase Activation and Stimulator Function. (PubMed, Biochemistry) - "High-resolution cryo-electron microscopy structures were solved for CYR715 bound to Ms sGC βC122S revealing that CYR715 occupies the same binding site as the characterized sGC stimulators YC-1 and riociguat...Conformational extension of sGC following NO, YC-1, or CYR715 binding was characterized using small-angle X-ray scattering, revealing that while ligand binding results in sGC extension this extension does not directly correlate to observed activity. This suggests that not all conformational extensions of sGC result in increased catalytic activity, and that effective stimulators assist in converting extension into catalytic function."

Journal • Cardiovascular

BRIDGE TO TRANSPLANT: A CASE OF SEVERE IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION MANAGED WITH VENOARTERIAL EXTRACORPOREAL MEMBRANE OXYGENATION AND DOUBLE-LUNG TRANSPLANTATION (CHEST 2025) - "CASE PRESENTATION: We have a 26-year-old female with cor pulmonale secondary to IPAH, listed for lung transplantation while on maximal medical therapy, including Ambrisentan, Adempas, intravenous epoprostenol, and had just started on Soteracept. DLT remains a feasible and life-saving option for patients with severe PAH requiring mechanical support, and it continues to be preferred over HLT under specified conditions. This makes the use of rescue therapies, like ECMO, critical for survival while awaiting transplantation(8)."

Clinical • Cardiovascular • Hematological Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases • Thrombocytopenia • Transplantation

OPTIMIZING RIOCIGUAT THERAPY FOR PULMONARY HYPERTENSION: A SYSTEMATIC REVIEW AND META-ANALYSIS OF DOSE VARIABILITY, SAFETY, AND EFFICACY (CHEST 2025) - "Riociguat demonstrates dose-dependent efficacy in improving functional and hemodynamic outcomes in PH. The 2.5 mg dose administered three times daily showed consistent benefits across outcomes. BMI influenced functional capacity but not hemodynamic responses."

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

A RARE ROUTE: GERBODE DEFECT AND SEVERE UNTREATED PULMONARY HYPERTENSION LEADING TO EISENMENGER SYNDROME (CHEST 2025) - "A Hickman catheter was placed and her final regimen was treprostinil 20 ng/kg/min, ambrisentan 10mg daily, riociguat 0.5mg three times a day, and warfarin 7.5mg daily. The rarity of GD, especially in adults of advanced age with no obvious acquired risk factors, leads to a scarcity of literature on practice guidelines. More cases are needed to establish appropriate screening and management."

Cardiovascular • Chronic Kidney Disease • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Renal Disease • Respiratory Diseases

HEART AND LUNG TRANSPLANT IN GROUP I/IV PULMONARY ARTERY HYPERTENSION WITH MASSIVE PULMONARY ARTERY AND RECURRENT CARDIAC TAMPONADE (CHEST 2025) - "CASE PRESENTATION: A 57-year-old female with a history of Group I/IV PAH (treated with Treprostinil, Riociguat, Macitentan, and experimental therapy from the SOTERIA trial), a large PAA, chronic pulmonary embolism, atrial fibrillation, bilateral intracranial aneurysms, and an unspecified connective tissue disorder that was hospitalized for cardiac tamponade following a routine surveillance echocardiogram. Patients with PAH and associated PAA with debilitating hypoxic respiratory failure and evidence of progressive right ventricular dysfunction, despite optimal medical management, should be considered for transplant. The decision to complete a BLT or HLT remains complex and patient-dependent. For patients with a suspected connective tissue disorder, a HLT should be considered."

Atrial Fibrillation • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Embolism • Rheumatology • Transplantation • Vascular Neurology

Comparative Effectiveness And Safety Of Soluble Guanylate Cyclase Stimulator Monotherapy Versus Combination With Endothelin Receptor Antagonists In Pulmonary Arterial Hypertension: A Multi-center Real-world Cohort Study (HFSA 2025) - "While guidelines recommend combination therapy, data comparing soluble guanylate cyclase stimulators (sGCs) alone versus in combination with endothelin receptor antagonists (ERAs) are limited. Using the TriNetX Research Network, we conducted a propensity score-matched cohort study of PAH patients receiving either sGC monotherapy (riociguat/vericiguat) or sGC+ERA combination therapy (with bosentan/ambrisentan/macitentan) between 2015-2023. In this real-world analysis, sGC+ERA combination therapy was associated with higher risks of MACE and hypotension compared to sGC monotherapy. However, after accounting for competing risks and patient characteristics, combination therapy showed potential mortality benefits. These findings emphasize the complex risk-benefit profile of combination therapy and underscore the importance of individualized treatment approaches in PAH management, with careful consideration of patient-specific factors to optimize outcomes."

Clinical • HEOR • Monotherapy • Real-world • Real-world evidence • Cardiovascular • Hypotension • Pulmonary Arterial Hypertension • Respiratory Diseases

Exploring the potential of soluble guanylyl cyclase stimulators and activators in heart failure. (PubMed, Biochem Pharmacol) - "Over a decade ago, the sGC stimulator riociguat was approved for the treatment of pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH). More recently, the sGC stimulator vericiguat was approved for symptomatic chronic HF...Promising results have been observed in clinical trials of HF with reduced ejection fraction (HFrEF), but not in clinical trials of HF with preserved ejection fraction (HFpEF). Further studies are needed to determine the precise mechanisms of action of sGC agonists in HF and associated cardiorenal diseases to fully leverage their therapeutic potential and address the challenges of implementing these agents in routine clinical practice."

Journal • Review • Cardiovascular • Chronic Kidney Disease • Congestive Heart Failure • Heart Failure • Hypertension • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases

An Unusual Initial Manifestation of Pulmonary Embolism: Aseptic Cavitary Infarction With Pulmonary Hypertension-Case Report and Literature Review. (PubMed, Respirol Case Rep) - "She responded well to anticoagulation and riociguat, showing marked clinical improvement and complete resolution of the cavity on follow-up imaging. Family screening showed that both children carried the same mutation, highlighting the value of genetic testing in patients with unexplained CTEPH."

Journal • Cardiovascular • Cough • Hematological Disorders • Hypertension • Infectious Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Balloon Pulmonary Angioplasty Versus Riociguat in Inoperable Chronic Thromboembolic Pulmonary Hypertension: Systematic Review and Meta-Analysis of Randomized Controlled Trials. (PubMed, Cardiol Rev) - "In summary, BPA delivers stronger pressure and biomarker improvements, while riociguat better boosts heart output. Combining or sequencing these treatments deserves further study in larger trials."

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

RIALTO: A Long-term Extension Study of Riociguat in Patients With Symptomatic Pulmonary Arterial Hypertension. (clinicaltrials.gov) - P4 | N=18 | Completed | Sponsor: Bayer | Active, not recruiting ➔ Completed

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