ambrisentan / Generic mfg. - LARVOL DELTA

Physician Understanding and Perceptions of Risk Communication Related to Drugs Covered by Risk Evaluation and Mitigation Strategy Programs. (PubMed, Pharmacoepidemiol Drug Saf) - "While physicians largely expressed awareness of REMS testing requirements and found REMS programs to be valuable sources of risk communication, they would like more information on managing side effects, more accessible information on REMS requirements, integration of REMS programs with EMRs, and timely updates to REMS materials as new safety information emerges."

Journal

Ambrisentan rescues doxorubicin-induced cardiac apoptosis and mitochondrial bioenergetic dysfunction: Mechanistic insights into p53 and mTOR signaling. (PubMed, Biochem Pharmacol) - "Moreover, inhibition of mTOR signaling with rapamycin attenuated the beneficial effects of ambrisentan on oxidative stress, apoptosis, and mitochondrial dysfunction. Collectively, these findings suggest that ambrisentan protects cardiomyocytes from doxorubicin-mediated injury, with potential involvement of p53- and mTOR-associated signaling in preserving redox homeostasis and cellular bioenergetics."

IO biomarker • Journal • Cardiovascular • Metabolic Disorders • BAX • BCL2 • BECN1 • NRF1

Association between a regional reimbursement policy and treatment escalation in pulmonary arterial hypertension: a real-world cohort study with long-term Markov cost-effectiveness analysis. (PubMed, Front Pharmacol) - "Descriptive analyses and multivariable logistic regression were used to examine factors associated with ambrisentan use and combination therapy...However, under current drug pricing conditions, the post-policy treatment distribution was not cost-effective from the Chinese healthcare system perspective. Substantial reductions in targeted therapy prices may be necessary to improve the economic feasibility of intensified combination treatment strategies."

HEOR • Journal • Real-world evidence • Reimbursement • US reimbursement • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

A novel TRPC6 variant (c.131C>T, p.(Pro44Leu)) associated with focal segmental glomerulosclerosis: a case report. (PubMed, Front Genet) - "He received losartan, dapagliflozin, strict salt restriction, and ambrisentan, achieving proteinuria reduction to 0.7g/24h without immunosuppression. This case demonstrates that genetic testing can guide personalized management in young patients with FSGS, offering a practical framework for avoiding unnecessary treatment-related morbidity. This case also illustrates that cost and psychological barriers can delay genetic diagnosis and highlights the value of early supportive therapy in genetic FSGS."

Journal • Focal Segmental Glomerulosclerosis • Gene Therapies • Glomerulonephritis • Nephrology • Renal Disease • TRPC6

Exploring the therapeutic landscape of pulmonary hypertension associated with interstitial lung disease, with a focus on idiopathic pulmonary fibrosis: a narrative review. (PubMed, Front Pharmacol) - "Most pulmonary arterial hypertension therapies have failed to show benefit in PH-IPF or have raised safety concerns, with ambrisentan and riociguat associated with harm. Inhaled treprostinil is currently the only approved therapy with randomized evidence of efficacy in PH associated with interstitial lung disease, including IPF. Supportive care, optimization of comorbidities, referral to expert centers, and timely lung transplantation evaluation remain essential components of management."

Journal • Review • Cardiovascular • Fibrosis • Hypertension • Idiopathic Pulmonary Fibrosis • Immunology • Infectious Disease • Interstitial Lung Disease • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Transplantation

Sepsis reporting signals associated with endothelin receptor antagonists and IFITM3-Centered interferon-responsive monocyte features: a pharmacovigilance and transcriptomic study. (PubMed, Front Pharmacol) - "FAERS analysis identified Maitentan and ambrisentan as PAH-targeted therapies with positive reporting signals for the MedDRA Preferred Term "Sepsis," with adjusted reporting associations persisting after adjustment for available demographic variables. These findings should be interpreted as reporting associations and transcriptomic hypotheses rather than evidence of causal drug-induced sepsis. The predicted riociguat-IFITM3 interaction provides a computational hypothesis for future experimental validation."

Adverse events • Journal • Cardiovascular • Hypertension • Infectious Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Septic Shock • IFIT1 • IFITM3 • IFNG • ISG15

Comparative clinical evaluation of endothelin receptor antagonists in pulmonary arterial hypertension using a multi-criteria decision analysis framework. (PubMed, Int J Clin Pharm) - "Within the proposed MCDA framework, ambrisentan achieved the highest overall score across multiple clinical and practical dimensions. The framework provides a transparent tool for structured evaluation, although economic and accessibility findings should be interpreted within the Chinese healthcare context."

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

Next-generation therapeutics and renaissance of legacy drugs targeting the endothelin system. (PubMed, Can J Physiol Pharmacol) - "Endothelin-1 (ET-1) was discovered in 1988, followed by identification of ETA and ETB receptors in 1990, enabling rapid development of the first endothelin receptor antagonists (bosentan, ambrisentan, and macitentan) for pulmonary arterial hypertension, a condition marked by elevated ET-1. Nearly a decade later, a new therapeutic wave began with the ETB agonist sovateltide (2021) for cerebral ischemic stroke, demonstrating the benefits of ETB activation...Clazosentan (ETA) for cerebral vasospasm and aprocitentan (ETA/ETB) for resistant hypertension extended endothelin-targeted therapy into more common diseases. In kidney disease, sparsentan (AT1/ETA) and atrasentan (ETA) have both been approved for IgA nephropathy, with atrasentan succeeding after earlier failed trials...This review outlines approaches for identifying legacy endothelin compounds suitable for new indications, using zibotentan, now combined with dapagliflozin to reduce fluid retention as an example. Kidney..."

Journal • Review • Cardiovascular • Glomerulonephritis • Hypertension • IgA Nephropathy • Ischemic stroke • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases • EDN1

In vitro and In vivo Based Multi-Target Therapy and Prognostic Bio-modeling in Liver Cancer. (PubMed, Curr Pharm Des) - "The findings of this study enlightened identified targets as the scientific foundation for HCC therapeutics using integrated network analysis and molecular dynamics simulations."

Journal • Preclinical • Hepatocellular Cancer • Liver Cancer • Oncology • Solid Tumor

Development of a population pharmacokinetic model using combined paediatric and adult data for four pulmonary arterial hypertension drugs. (PubMed, Br J Clin Pharmacol) - "This study clarified the pharmacokinetic parameters and their variability for sildenafil, tadalafil, bosentan and ambrisentan using combined data from Japanese paediatric and adult patients with PAH. The results of Bayesian estimation suggest that at current clinical dosages, plasma concentrations of these drugs may be lower in paediatric than adult patients."

Journal • PK/PD data • Cardiovascular • Hypertension • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

ACTION: The Impact of ERA Switching on Risk Stratification in Pulmonary Arterial Hypertension (clinicaltrials.gov) - P=N/A | N=183 | Recruiting | Sponsor: University of Sao Paulo General Hospital | N=121 ➔ 183

Enrollment change • Cardiovascular • Congestive Heart Failure • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

The invisible hide : Vasculopathy in disguises (ISTH 2026) - "The patient was initiated on pulmonary arterial hypertension–targeted therapy with ambrisentan and tadalafil, resulting in symptomatic improvement and stabilization of right heart failure. Clinicians should maintain a high index of suspicion for systemic sclerosis sine scleroderma in patients with unexplained vasculopathy and pulmonary arterial hypertension. DOI*10.1016/j.rpth.2026.103936"

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

A Rare Case of Pulmonary Arterial Hypertension in a Patient With Breast Cancer Treated With Trastuzumab Emtansine. (PubMed, Cureus) - "The patient was treated with sildenafil and ambrisentan, resulting in symptomatic and haemodynamic improvement. This case highlights the importance of recognising PAH as a potential adverse effect of T-DM1. Clinicians should maintain a high index of suspicion in patients presenting with unexplained dyspnoea or right ventricular dysfunction to enable early diagnosis and appropriate management."

Journal • Breast Cancer • Cardiovascular • Heart Failure • HER2 Breast Cancer • HER2 Positive Breast Cancer • Hypertension • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Solid Tumor

Targeting PI3K/Akt/mTOR Pathway, Ki-67 and Endothelin Receptors by Ambrisentan in Juvenile Rat Intestinal Ischemia. (PubMed, Int J Mol Sci) - "However, AMB treatment increased immune expressions of phosphatidylinositol 3-kinase (PI3K), protein kinase B (Akt), Ki-67, and mammalian target of rapamycin (mTOR) and showed remarkable improvement in the histopathological changes. AMB could be considered as an adjuvant medical treatment for cases of JII/R."

Journal • Preclinical • Cardiovascular • Pediatrics • Reperfusion Injury • CASP3 • mTOR • NFKB1 • PI3K

ET1/ETAR/NOX signaling enhances invadopodia formation, thereby promoting cell invasion in gallbladder cancer cells (EACR 2026) - "This question is addressed in the present study.Material and The GBC cell line NOZ was treated with ET1 and/or Ambrisentan (ETAR antagonist), and/or Diphenyleneiodonium (pan-NOX proteins inhibitor), and/or Saracatinib (Src phosphorylation inhibitor). ET1/ETAR signaling promotes an increase in NOX-dependent ROS production, which is associated with increased invasiveness of GBC cells through enhanced invadopodia formation."

Gallbladder Cancer • Oncology • Solid Tumor • EDN1

Efficacy and Safety of Oral Budesonide Targeted-Release Formulation Combined with Ambrisentan in the Treatment of IgA Nephropathy (ERA 2026) - "Nefecon, a targeted-release oral budesonide formulation, influences the gut-associated lymphoid system, regulates immune response and attenuates Gd-IgA1 production. (b)Percent change in 24-hour urinary protein from baseline at different visits. (c) Percent change in eGFR from baseline at different visits."

Clinical • Glomerulonephritis • IgA Nephropathy • Lupus Nephritis • Renal Disease

Not All Pulmonary Vascular Dilations Are AVMs: A Case of Pulmonary Venous Varix in Pulmonary Hypertension (PHA 2026) - "He had completed a prolonged course of prednisone for sarcoidosis, which remained in remission with residual fibrotic changes...His home medications included ambrisentan, tadalafil, torsemide, and abiraterone...The distinction is increasingly relevant as emerging PAH therapies such as sotatercept, which modulates TGF ß signaling, may induce or unmask intrapulmonary shunts and are not suitable in patients with true AVMs... Multi-phase pulmonary angiography with careful evaluation of arterial and venous phases, combined with detailed review of cross-sectional imaging, remains essential for accurate diagnosis of pulmonary venous varix. Clinicians must maintain heightened awareness of this rare entity, especially in the context of pulmonary hypertension, to ensure appropriate conservative management. Figures/Images: Figure 1: CT chest angiography images; A and B - Sagittal and axial sections showing dilated tortuous vein crossing the major fissure."

Clinical • Cardiovascular • CNS Disorders • Fibrosis • Genito-urinary Cancer • Hypertension • Immunology • Prostate Cancer • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sarcoidosis • Solid Tumor • Varicose Veins

Severe Pulmonary Hypertension Due to Undiagnosed Congenital Heart Disease Presenting in Late Pregnancy (PHA 2026) - "Right heart catheterization showed pulmonary artery pressures of 104/61 mmHg (mean 84 mmHg), normal pulmonary capillary wedge pressure (6 mmHg), Qp:Qs 2.1, preserved cardiac index, and pulmonary vascular resistance of 4.0 Wood units The patient was initiated on inhaled Epoprostenol via high-flow nasal cannula and intravenous Treprostinil starting at 2 ng/kg/min with daily up-titration to 8 ng/kg/min...Postpartum, she was transitioned to oral triple therapy with Sildenafil, Ambrisentan, and Selexipag (600 μg twice daily) and discharged in stable condition... This case highlights late-pregnancy presentation of severe PH due to previously unrecognized CHD with systemic-to-pulmonary shunting. Rapid hemodynamic assessment, early initiation of parenteral prostacyclin therapy, and coordinated multidisciplinary delivery planning enabled favorable maternal and fetal outcomes. Early consideration of occult CHD is essential in pregnant patients presenting with unexplained..."

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

Dose-Anchored Conversion of IV Treprostinil to Oral Selexipag: A Case Report with Literature Review (PHA 2026) - "She was transferred to the cardiac ICU for initiation of IV treprostinil on background ambrisentan and sildenafil. PHA 2026 Conference: Abstracts This case illustrates that a structured, dose-anchored conversion from IV treprostinil to oral selexipag may be feasible in carefully selected PAH patients who demonstrate rapid improvement on intensive therapy but are unsuitable for long-term parenteral administration. This approach provides a reproducible, quantitative framework to guide oral prostacyclin initiation and addresses an important gap in adult PAH management. Successful implementation requires multidisciplinary expertise, close outpatient monitoring, and individualized titration to maintain hemodynamic stability and clinical benefit."

Case report • Clinical • Review • Cardiovascular • Hypertension • Peripheral Arterial Disease • Pulmonary Arterial Hypertension • Respiratory Diseases

Use of ambrisentan in the management of refractory pulmonary arterial hypertension following occlusion of a bidirectionally shunting patent ductus arteriosus in a dog. (PubMed, J Vet Cardiol) - "Combination medical therapy using ambrisentan, sildenafil, pimobendan and amlodipine resulted in resolution of right-sided congestive heart failure and oxygen dependency. At the last follow-up, the patient remained stable without complications associated with ductal occlusion. This case highlights the success of bidirectional ductal occlusion with postoperative use of ambrisentan for management of refractory PAH."

Journal • Anorexia • Cardiovascular • Congestive Heart Failure • 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) - "The combination of AtoAmb demonstrated a synergistic antifibrotic effect in a translational rat model of MASLD but failed to reduce PH, likely due to persistent severe steatosis and sinusoidal compression. These findings highlight the dissociation between fibrosis and portal pressure in advanced MASLD and emphasize the need for multi-targeted strategies addressing both lipid accumulation and fibrosis for effective PH management."

Metastases • Preclinical • Cardiovascular • Fibrosis • Hepatology • Immunology • Inflammation • Liver Cirrhosis • Metabolic Disorders • Metabolic Dysfunction-Associated Steatohepatitis • Metabolic Dysfunction-Associated Steatotic Liver Disease • Portal Hypertension • COL1A1 • NOS3 • TIMP1

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

Uncovering immune related mechanisms in drug-induced liver toxicity of sitaxsentan using human ex vivo precision-cut liver slices (EASL 2026) - "HPCLiS were treated with sitaxsentan, ambrisentan, or acetaminophen (APAP) for 48 h. Release of lactate dehydrogenase into culture supernatant and cellular metabolic activity measurement by WST-1 turnover were used as indicators for general cytotoxicity... In summary, our data suggest early activation of Kupffer cells after sitaxsentan treatment, which could trigger further immune cell activation leading to hepatotoxicty. Shedding light on the yet unknown irDILI effects of sitaxsentan, the herein presented results advocate for hPCLiS as an invaluable pre-clinical model to assess safety of novel therapies, particularly addressing concerns about immune-mediated liver toxicity."

Preclinical • Hepatology • Liver Failure • CD4 • CD8 • IL13

A Rare Case of Multi-System Involvement and Hereditary Pulmonary Hypertension Caused by De Novo Heterozygous CAV1 Mutation in a Pediatric Patient. (PubMed, Children (Basel)) - "She received sequential treatment with digoxin, hydrochlorothiazide, tadalafil, ambrisentan, selexipag, and treprostinil. Early genetic diagnosis, regular follow-up, and individualized treatment are crucial. It requires the joint efforts of patients, parents, and healthcare providers."

Journal • Cardiovascular • Cataract • Gastroenterology • Gastrointestinal Disorder • Hypertension • Lipodystrophy • Metabolic Disorders • Ophthalmology • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • CAV1

Ambrisentan Exhibits Hepatoprotective Effects Against NASH-Associated Hepatic Injury in Dexamethasone-Treated Rats Through Regulation of Inflammation, Ferroptosis and Autophagy. (PubMed, Pharmaceuticals (Basel)) - "Moreover, AMB ameliorated DEXA-induced changes in endothelial and vascular function by increasing hepatic PGI2 and cGMP and lowering ET-1 and iNOS. AMB improved DEXA-induced NASH, primarily through its action on endothelin pathways, with associated reductions in inflammation and the downstream processes of ferroptosis, ferritinophagy, lipophagy, and autophagy."

Journal • Preclinical • Hematological Disorders • Hepatology • Inflammation • Liver Failure • Metabolic Dysfunction-Associated Steatohepatitis • BECN1 • FTH1 • GPX4 • IL6 • NCOA4 • TGFB1