ambrisentan / Generic mfg. - LARVOL DELTA

Disproportionality analysis of adverse events associated with endothelin receptor antagonists based on the FDA adverse event reporting system (FAERS). (PubMed, Front Cardiovasc Med) - "Endothelin receptor antagonists (ERAs), including bosentan, ambrisentan, and macitentan, are recognized as first-line treatments for pulmonary arterial hypertension (PAH). These results provide valuable insights for clinicians aiming to optimize ERA utilization, minimize associated risks, and improve patient outcomes. Future research should focus on elucidating the underlying mechanisms of these ADEs to further enhance the safety and efficacy of ERA therapies."

Adverse events • Journal • Cardiovascular • Gout • Hematological Disorders • Hypertension • Hypotension • Inflammatory Arthritis • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Thrombosis

EFFICACY AND SAFETY OF BUDESONIDE DELAYED-RELEASE CAPSULES COMBINED WITH AMBRISENTAN FOR IGA NEPHROPATHY (ISN-WCN 2026) - "This combination strategy, acting through dual pathways of "immune modulation + endothelial function protection," offers a novel therapeutic approach for IgAN and holds significant clinical promise. A larger randomized controlled trial will be conducted to further validate its long-term efficacy and renoprotective effects.This abstract was also submitted for CCSN 2025 congress."

Clinical • Glomerulonephritis • IgA Nephropathy • Immune Modulation • Immunology • Lupus Nephritis • Renal Disease

Ambrisentan attenuates cisplatin-related mitochondrial dysfunction in the heart via regulation of p53 and NF-κB signaling. (PubMed, Sci Rep) - "Mechanistically, pharmacological inhibition of p53 and NF-κB enhanced the protective effects of ambrisentan, whereas their activation exacerbated cisplatin-induced cardiac injury. Therefore, ambrisentan may serve as a potential agent for suppressing apoptosis, inflammation, and mitochondrial impairments, offering protection against cisplatin-associated cardiotoxicity through modulation of the p53 and NF-κB pathways."

IO biomarker • Journal • Cardiovascular • Inflammation • Metabolic Disorders • BAX • BCL2 • CASP3 • CASP7 • IL6 • NRF1 • TNFA

Marked clinical and haemodynamic improvement with sotatercept in severe refractory pulmonary arterial hypertension associated with corrected complex congenital heart disease. (PubMed, Respir Med Case Rep) - "Despite treatment with tadalafil, ambrisentan, and high dose subcutaneous treprostinil, she remained at intermediate-high risk, with WHO-FC III, NT-proBNP 3773 pg/mL and a 6-min walk distance (6MWD) of 480 m. Sotatercept was initiated through an expanded access program. A significant and rapid improvement in haemodynamics and functional capacity was observed after starting sotatercept, in a patient with severe PAH-CHD refractory to maximal therapy. Sotatercept may represent an effective rescue therapy and a potential therapeutic option for complex and severe PAH patients."

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

Effects of combined Atorvastatin and Ambrisentan treatment on hemodynamic alterations and liver fibrosis in a translational rat model of advanced MASLD (EASL 2026) - No abstract available

Metastases • Preclinical • Fibrosis • Hepatology • Immunology • Liver Cirrhosis • Metabolic Dysfunction-Associated Steatotic Liver Disease

Outcome of Pediatric Pulmonary Hypertension Patients Requiring ECMO (SCCM 2026) - "100% (31/31) of patients received at least one PAH therapy prior to and during ECMO [31/31 (100%) inhaled nitric oxide or inhaled epoprostenol; 26/31 (84%) sildenafil, tadalafil or riociugat; 18/31 (58%) macitentan, ambrisentan or bosentan; 8/31(26%) selexipag; 10/31(32%) treprostinil. Pediatric PAH is a complex disease due to varied etiologies often requiring multiple medications to effectively manage. Certain subpopulations such as those with single ventricle CHD have higher mortality especially when requiring ECMO. Further study is necessary to determine if earlier identification and management of PAH patients prior to ECMO can improve survival and decrease need for mechanical circulatory support in this high-risk population."

Clinical • Cardiovascular • Heart Failure • Hematological Disorders • Infectious Disease • Pediatrics • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases

Comparative Effectiveness of Endothelin Receptor Antagonists and PDE-5 Inhibitors in PAH (SCCM 2026) - "Adults carrying an ICD-10 code for PAH (I27.0) who started either ERA monotherapy (bosentan, ambrisentan, macitentan) or PDE5I monotherapy (sildenafil, tadalafil) with no prior prostacyclin or combination therapy were eligible. n this large multicentre, propensity-matched analysis of treatment-naive PAH, ERAs and PDE5Is produced indistinguishable 12-month survival, cardiovascular outcomes, and adverse-event rates. These findings support current guidelines that either class is an acceptable first-line option; choice may therefore be guided by individual comorbidity profiles, side-effect tolerance, and cost rather than expectations of differential efficacy."

HEOR • Cardiovascular • Congestive Heart Failure • Heart Failure • Myocardial Infarction • Pulmonary Arterial Hypertension • Respiratory Diseases

WHEN TWO RARE PATHS MEET: BILATERAL CORONARY-LV FISTULAE IN SCLERODERMA-ASSOCIATED PULMONARY HYPERTENSION (ACC 2026) - "Case: A 73-year-old woman with CREST/scleroderma-associated pulmonary hypertension on ambrisentan and home oxygen presented with worsening dyspnea... Bilateral LV-terminating CAF are rare. Their coexistence with severe PAH complicates diagnosis and management, underscoring the importance of distinguishing incidental anomalies from clinically meaningful drivers of disease."

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

DIAGNOSIS AND MANAGEMENT OF PULMONARY HYPERTENSION IN A PATIENT WITH OSLER-WEBER-RENDU SYNDROME (ACC 2026) - "Patient was started on sildenafil and ambrisentan with improvement of symptoms. Right heart catheterization is crucial to differentiate between forms of PHT in patients with OWRS and subsequently to initiate appropriate therapy, since both entities require different treatment. Early initiation of therapy decreases re-admission rate, improves morbidity and mortality."

Clinical • Cardiovascular • Chronic Kidney Disease • CNS Disorders • Hematological Disorders • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases

A Novel Approach to Pulmonary Vasodilator Transitions: Converting Oral Selexipag to Inhaled Treprostinil in Mixed Pulmonary Arterial Hypertension and Covid Associated Interstitial Lung Disease (ISHLT 2026) - "He started on max dose tadalafil, ambrisentan and selexipag. The patient successfully transitioned with this regimen with objective improvement.Summary We present a novel approach of transitioning oral selexipag to inhaled treprostinil in a patient with combined Group 1 and Group 3 PH. Given the risk of oral agents with group 3 PH, our approach to transition was safe and tolerable with clinical improvement."

Cardiovascular • Fibrosis • Hepatology • Immunology • Interstitial Lung Disease • Novel Coronavirus Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

A MICROCOSTING STUDY OF CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION (CTEPH) BASED ON REAL WORLD DATA FROM BRAZIL (ISPOR 2026) - "OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease that has pulmonary endarterectomy (PEA) as the gold standard treatment, while ballon pulmonary angioplasty (BPA) and guanylate cyclase stimulator (riociguat) are recommended for patients who cannot undergo surgery or who relapse after PEA. Patients with CTEPH account for an excessive burden on the system. Strategies to reduce the impact of the disease should be pursued from both clinical and financial perspectives."

Clinical • Real-world • Real-world evidence • Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Rare Diseases • Respiratory Diseases

Pulmonary Artery Denervation for Refractory Sjögren's Syndrome-Associated Pulmonary Arterial Hypertension: A Case Report of Combined Hemodynamic and Renal Recovery. (PubMed, Catheter Cardiovasc Interv) - "Despite initial immunosuppressive therapy and dual oral PAH-targeted treatment with ambrisentan and tadalafil, she remained at high risk. Renal function also improved (24-hour urinary protein 158 mg). This case suggests that PADN may be an effective adjunctive interventional strategy for refractory autoimmune PAH, even with concurrent renal involvement, facilitating simultaneous cardiopulmonary and renal recovery."

Journal • Cardiovascular • Hypertension • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases • Sjogren's Syndrome

Ambrisentan as adjunctive therapy for IgA nephropathy: a retrospective single-center analysis. (PubMed, J Nephrol) - "In this analysis incorporating a matched historical control, adjunctive ambrisentan significantly reduced proteinuria and hematuria in patients with IgAN without affecting kidney function. The proteinuria-lowering effect was more pronounced when ambrisentan was added to an ACEi/ARB or finerenone. Notably, a significant decline in hemoglobin levels was observed, indicating a need for monitoring. These results warrant further prospective trials to confirm the long-term renal benefits and safety of ambrisentan in IgAN."

Journal • Retrospective data • Glomerulonephritis • Hematological Disorders • IgA Nephropathy • Renal Disease

COMBINED EFFECT OF ATORVASTATIN AND AMBRISENTAN ON THE HEMODYNAMIC ALTERATIONS AND LIVER FIBROSIS IN A TRANSLATIONAL RAT MODEL OF MASLD WITH ADVANCED FIBROSIS (AEEH 2026) - No abstract available

Metastases • Preclinical • Fibrosis • Hepatology • Immunology • Liver Cirrhosis • Metabolic Dysfunction-Associated Steatotic Liver Disease

Unilateral Pulmonary Artery Agenesis in an Adult: A Case Report and Review of Literature. (PubMed, Case Rep Radiol) - "The patient was managed conservatively with oxygen therapy and pulmonary vasodilator therapy, including ambrisentan and tadalafil, resulting in clinical stabilization. Unilateral pulmonary artery agenesis is a rare but important cause of pulmonary hypertension and cor pulmonale in adults. Computed tomography pulmonary angiography plays a pivotal role in diagnosis by accurately delineating vascular anatomy and collateral circulation, enabling appropriate management and prevention of complications."

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

Impact of Switching From Ambrisentan to Bosentan on Clinical and Risk Profiles in Pulmonary Arterial Hypertension: Insights From the Action Study (ATS 2026) - No abstract available

Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Endothelin-1 in the failing Fontan: pathobiology, precision therapeutics, and future trial design. (PubMed, Front Pediatr) - "Trials of endothelin-receptor antagonists (bosentan, ambrisentan, macitentan) demonstrate reassuring safety and suggest benefit when outcomes emphasize ventilatory efficiency or hepatic endpoints rather than peak oxygen consumption, which is physiologically constrained in Fontan physiology. Given the mixed results of existing trials, a framework is outlined that stratifies Fontan patients into pulmonary-inefficiency, congestive-hepatic, lymphatic, and arrhythmia-dominant phenotypes, using co-primary endpoints such as VE/VCO2 slope, elastography, and biomarker panels. By linking ET-1 biology to pragmatic trial design, this approach emphasizes targeted strategies that may stabilize the circulation, extend transplant candidacy, and improve long-term outcomes."

Journal • Review • Cardiovascular • Heart Failure • Hepatology • Hypertension • Transplantation • EDN1 • RHOA

Modulation of AKT/GSK-3β signaling by ambrisentan alleviates chronic kidney disease. (PubMed, Pathol Res Pract) - "Furthermore, AMB mitigated rh-TGF-β1-induced apoptosis, as assessed by flow cytometry, and modulated Bax, Bcl-2, and cleaved caspase-3 expression, as revealed by qRT-PCR and immunohistochemistry. Collectively, the findings indicate that the AMB modulates the AKT/GSK-3β axis, halting fibrotic progression in CKD, suggesting its clinical potential."

IO biomarker • Journal • Cardiovascular • Chronic Kidney Disease • Fibrosis • Hypertension • Immunology • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases • BAX • BCL2 • CASP3 • CDH1 • GSK3B • TGFB1 • VIM

Ambrisentan for Early-Stage Low-Risk Pulmonary Arterial Hypertension: Design of the Randomized, Double-Blind, Placebo-Controlled ALEPH Trial. (PubMed, JACC Asia) - P=N/A | "The ALEPH trial aims to generate high-quality evidence regarding the efficacy and safety of ambrisentan monotherapy in early-stage, low-risk PAH. This study may provide valuable insight into early therapeutic intervention for patients with mild hemodynamic disorder. (Ambrisentan for Early Low-Risk Pulmonary Arterial Hypertension [ALEPH]; NCT06987097)."

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

Impact of Pharmacological Intervention on Clinical Outcomes in Borderline High-Altitude Pulmonary Hypertension: A Multicenter, Placebo-Controlled, Randomized, Double-Blind Clinical Trial (ChiCTR) - P=N/A | N=712 | Not yet recruiting | Sponsor: The Second Affiliated Hospital of the Army Medical University; The Second Affiliated Hospital of the Army Medical University

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

Inferior vena cava anomalous drainage into the left atrium with severe pulmonary arterial hypertension: a case report and review of management strategies. (PubMed, Front Cardiovasc Med) - "Following multidisciplinary consultation, a combined "treat and repair" strategy was adopted, initiating targeted PAH therapy with ambrisentan and sildenafil. At the 3-month follow-up, the patient reported significant symptomatic improvement, resumed daily physical activities without cardiorespiratory discomfort, PASP decreased from 146 mmHg to 127 mmHg and demonstrated enhanced functional capacity. This case highlights the diagnostic challenges and therapeutic considerations for IVC anomalies associated with PH, contributing novel insights to the management of complex congenital cardiovascular disorders."

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

Physician Awareness of FDA's Relaxation of REMS-Required Laboratory Testing Requirements and Changes in Prescribing Practices During the COVID-19 Pandemic. (PubMed, Drug Saf) - "Although policy awareness was correlated with laboratory practice changes, fewer than half of physicians who were aware of the FDA's policy reported changing their practices."

Journal • Infectious Disease • Novel Coronavirus Disease • Respiratory Diseases

Isolated trigeminal sensory neuropathy in a patient with mixed connective tissue disease: A diagnostic challenge (EHF-EHC 2025) - "Chronic medication included gabapentin 100mg three times daily, prednisolone 10mg, hydroxychloroquine 400mg, aspirin 150mg, pentoxifylline 400mg, nifedipine 20mg, furosemide, tadalafil and ambrisentan. This case highlights the importance of considering autoimmune mechanisms in isolated trigeminal neuropathies, especially in patients with systemic connective tissue disease. EMG may assist in detecting trigeminal dysfunction, and intrathecal autoantibodies, even without structural lesions, support an immune-mediated process and underscore the value of CSF analysis in atypical neurological presentations."

Clinical • Cardiovascular • Immunology • Melanoma • Pain • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Rheumatology • Solid Tumor

A patient-derived CRISPR platform reveals selective dependencies in Acute Myeloid Leukemia (ASH 2025) - "Our results indicate that EDNRAknockout inhibits AML cell proliferation and growth, highlighting its potential as a therapeutic target.Furthermore, EDNRA is overexpressed in AML compared to normal cells, and since FDA approved EDNRAinhibitors such as Macitentan, Bosentan, Ambrisentan, and Clazosentan are already being used to treatpulmonary arterial hypertension, this presents a promising opportunity to evaluate these inhibitors aspotential chemotherapy-sensitizing agents for high-risk AML.SOD1 plays a critical role in eliminating toxicradicals generated within biological systems and has been associated with poor outcomes in AML. This study demonstrates the establishment of a scalable platform for CRISPR screening inprimary AML cells for identification of more conserved vulnerabilities that may be exploitedtherapeutically, with EDNRA as a potential target. Ongoing work includes validating key targets with thegoal of advancing therapeutic strategies. Future studies will..."

Clinical • Acute Myelogenous Leukemia • Hematological Malignancies • Leukemia • Pulmonary Arterial Hypertension • Respiratory Diseases • EDNRA • HOXA9 • MEIS1 • PPM1D • SUZ12

Upfront combination therapy with sildenafil and ambrisentan in patients with chronic thromboembolic pulmonary hypertension. (PubMed, J Bras Pneumol) - "The combined use of sildenafil and ambrisentan appears to be associated with clinical, functional, and hemodynamic improvement in patients with CTEPH."

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