ambrisentan / Generic mfg. - LARVOL DELTA

Combination of Orai1 inhibitor CM5480 with specific therapy mitigates pulmonary hypertension and its cardiac dysfunction. (PubMed, JCI Insight) - "Standard PAH therapies, ambrisentan or sildenafil, achieved modest improvements in experimental PAH. Finally, we found enhanced Orai1 expression/function in PASMCs and pulmonary vein SMCs from patients with pulmonary veno-occlusive disease. These findings suggest that Orai1 inhibition represents a potentially novel and complementary therapeutic strategy for PAH by acting at pulmonary vascular and RV levels."

Journal • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • RAI1

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

WHEN TREATMENT TURNS TOXIC: A CAUTIONARY TALE OF POMALIDOMIDE-ASSOCIATED PULMONARY COMPLICATIONS (CHEST 2025) - "INTRODUCTION: Pomalidomide, an immunomodulatory drug much more potent than thalidomide, has significantly improved treatment outcomes for relapsed or refractory multiple myeloma (MM) but its side effect profile remains poorly understood...CASE PRESENTATION: A 79-year-old man with a history of multiple myeloma complicated by paraneoplastic pulmonary hypertension managed with three vasodilators (ambrisentan, sildenafil, and treprostinil) presented with activity-limiting shortness of breath...For multiple myeloma, he was initially treated with lenalidomide and transitioned to bortezomib...He unfortunately had relapsed disease several years later prompting treatment with bortezomib which was subsequently transitioned to a regimen consisting of daratumumab, bortezomib, dexamethasone. Shortly before evaluation for dyspnea, he was being treated with elotuzumab and pomalidomide...Pomalidomide was stopped and he was started on a tapered course of prednisone over four weeks with..."

Cardiovascular • Coronary Artery Disease • Hematological Malignancies • Hypertension • Immunology • Infectious Disease • Interstitial Lung Disease • Multiple Myeloma • Obstructive Sleep Apnea • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sleep Disorder

Efficacy and safety of Ambrisentan on idiopathic membranous nephropathy----a prospective, multi-center, randomized and controlled study (ChiCTR) - P=N/A | N=80 | Not yet recruiting | Sponsor: The Second Affiliated Hospital of Army Medical University; Army Medical University

New trial • Glomerulonephritis • Renal Disease

Case Report: Bevacizumab-induced renal-limited TMA and FSGS-like lesions in a kidney transplant recipient. (PubMed, Front Oncol) - "We present a case of nephrotic syndrome and renal insufficiency arising from small intestinal mucinous adenocarcinoma 17 years post-renal transplantation, treated with bevacizumab and sindilizumab. Upon discontinuation of the antitumor drugs and initiation of treatment with telmisartan, fenoldopam, and ambrisentan, proteinuria markedly decreased and eGFR improved. No tumor recurrence was observed over a follow-up period exceeding six months."

Journal • Focal Segmental Glomerulosclerosis • Glomerulonephritis • Nephrology • Oncology • Renal Disease • Transplantation

CHRONIC THROMBOEMBOLIC PULMONARY HYPERTENSION IN A PATIENT WITH SYSTEMIC SCLEROSIS (CHEST 2025) - "CASE PRESENTATION: A female patient in her 40s with a history of SSc with diffuse cutaneous involvement on mycophenolate mofetil therapy, severe chronic obstructive pulmonary disease (COPD)/asthma, hypertension, former smoker, and remote substance use, presented for management of pulmonary hypertension (PH)...Right heart catheterization (RHC) was delayed due to issues with vascular access secondary to her diffuse cutaneous SSc, but eventually confirmed severe precapillary pulmonary hypertension with a mean pulmonary artery pressure of 44 mm Hg responsive to inhaled nitric oxide, pulmonary vascular resistance of 15 Wood units, and pulmonary capillary wedge pressure of 12 mm Hg, She was anticoagulated with apixaban, though therapy was interrupted due to recurrent epistaxis. PH-specific therapy was started with sildenafil, titrated to 20 mg three times daily. Though the addition of ambrisentan was poorly tolerated, her right ventricular parameters did normalize with the..."

Clinical • Asthma • Cardiovascular • Chronic Myeloid Leukemia • Chronic Obstructive Pulmonary Disease • Congestive Heart Failure • Heart Failure • Hematological Disorders • Hematological Malignancies • Hypertension • Immunology • Interstitial Lung Disease • Leukemia • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases • Scleroderma • Systemic Sclerosis • Thrombocytosis

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

EVALUATING THE COMPARATIVE EFFECTIVENESS OF ENDOTHELIN INHIBITORS IN IDIOPATHIC PULMONARY FIBROSIS: A COMPREHENSIVE BAYESIAN NETWORK META-ANALYSIS WITH INTERVENTION RANKING (CHEST 2025) - "This study suggests that bosentan and macitentan are the most effective endothelin inhibitors for managing idiopathic pulmonary fibrosis (IPF) in terms of improving disease progression and lung function, with higher SUCRA values indicating superior efficacy. Ambrisentan showed the least benefit across several outcomes, including FVC and DLCO. CLINICAL IMPLICATIONS: Clinicians may consider bosentan and macitentan as preferred options for managing IPF due their better efficacy profiles, particularly in slowing disease progression and improving lung function."

HEOR • Retrospective data • Fibrosis • Idiopathic Pulmonary Fibrosis • Immunology • Pulmonary Disease • Respiratory Diseases • EDN1

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

Pathogenic Glomulin Gene Variant in a Patient with Idiopathic Pulmonary Arterial Hypertension: A Novel Association Case Report. (PubMed, Reports (MDPI)) - "The patient was treated with oxygen therapy, diuretics, non-invasive ventilation, and dual oral pulmonary arterial hypertension therapy (ambrisentan and tadalafil), with stabilization but persistent hypercapnia. While causality cannot be inferred, glomulin's role in vascular smooth-muscle maturation provides a plausible link to pulmonary vascular remodeling. This case underscores the importance of assessing respiratory muscle function in idiopathic pulmonary arterial hypertension patients with hypercapnia and highlights the potential relevance of extended genetic testing in rare pulmonary vascular disease."

Journal • Cardiovascular • Hypertension • Immunology • Myositis • Pulmonary Arterial Hypertension • Pulmonary Disease • Rare Diseases • Respiratory Diseases

Additive Antihypertensive and Renoprotective Effects of a Mineralocorticoid Receptor Antagonist Combined with an Endothelin Receptor Type A-Selective Antagonist in Salt-Loaded Spontaneously Diabetic Torii Rats (KIDNEY WEEK 2025) - "Kerendia (KER), a mineralocorticoid receptor antagonist (MRA), is approved for treating chronic kidney disease in type 2 diabetes...We investigated the effects of KER combined with the ERA, ambrisentan (AMB) in salt-loaded SDT fatty rats...Although exudative glomerular lesions were observed in several rats, no significant difference in incidence was detected between the experimental groups. Conclusion MRA provides antihypertensive and renoprotective effects in a salt-loaded SDT fatty rat model of diabetic nephropathy with hyporeninemic hypertension, with additive benefits when combined with ERA."

Preclinical • Cardiovascular • Chronic Kidney Disease • Diabetes • Diabetic Nephropathy • Fibrosis • Hypertension • Immunology • Metabolic Disorders • Nephrology • Renal Disease • Type 2 Diabetes Mellitus • EDN1 • EDNRA • FABP1 • GAPDH • KIM1 • POSTN • SPP1 • TIMP1

IgAN with Orthostatic Proteinuria: A Case Report (KIDNEY WEEK 2025) - "Over this period, due to poorly controlled proteinuria, he had been given various treatment regimens on top of RASi support therapy, including steroids, Tacrolimus, leflunomide, hydroxychloroquine, mycophenolate mofetil, ambrisentan, and Tripterygium wilfordii polyglycoside, but his spot urine protein levels remained unsatisfactory...We thus considered that the patient had IgAN coexisting with orthostatic proteinuria and withdrew immunosuppressive medications, retaining only irbesartan at 150 mg/day...This case underscores the importance of considering this diagnosis in adult IgAN patients with discordant findings or poor response to treatment. Routine use of split urine collections, especially in patients with variable home test results, can help avoid misdiagnosis and overtreatment."

Case report • Clinical • Glomerulonephritis • IgA Nephropathy • Ophthalmology • Renal Disease

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

Patient Perceptions of and Experiences with Risk Evaluation and Mitigation Strategies. (PubMed, Clin Pharmacol Ther) - "To understand patients' and caregivers' perception of and experiences with REMS programs with elements to assure safe use, we conducted semi-structured interviews from March 2022 to July 2023 with 135 patients or caregivers of patients who had used alemtuzumab (Lemtrada), ambrisentan (Letairis), clozapine (Clozaril), isotretinoin (Accutane), lenalidomide (Revlimid), pegvaliase (Palynziq), or sodium oxybate (Xyrem/Xywav). Among 135 participants, five key themes emerged: (1) Participants were knowledgeable about REMS-associated drug risks and requirements; (2) some participants had difficulty finding prescribers, particularly for clozapine; (3) administrative burdens related to REMS implementation were a source of frustration, with satisfaction higher when care coordination support was provided; (4) emotional and logistical challenges were identified with pregnancy- and clozapine-related testing; and (5) participants desired similar engagement around risks not..."

Journal

Perioperative Optimization for Tonsillectomy and Adenoidectomy in Pediatric Patient with Severe Pulmonary Hypertension and Moderate Obstructive Sleep Apnea (ASA 2025) - "Patient's pulmonary hypertension and right heart failure was managed with a reverse Potts shunt, continuous subcutaneous treprostinil, oral ambrisentan, oral sildenafil, and oral digoxin. Perioperative optimization of this patient required continuation of pulmonary anti-hypertensives, adequate premedication to facilitate controlled intravenous induction, and postoperative sedation to minimize the risk of worsening right heart function during emergence from general anesthesia."

Clinical • Anesthesia • Cardiovascular • Congestive Heart Failure • Heart Failure • Obstructive Sleep Apnea • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Sleep Disorder

Tailored Perioperative Approach for High-Risk iPAH Patient Undergoing Hemorrhoidectomy Pre-Lung Transplant (ASA 2025) - "A 51-year-old with BMPR2-associated idiopathic pulmonary arterial hypertension (iPAH) on intravenous epoprostenol, sildenafil, and ambrisentan, awaiting lung transplantation, presented for pre-transplant hemorrhoidectomy...The patient was pretreated with vasopressin and epinephrine; sedation was achieved with midazolam, propofol, and remifentanyl. ECMO and cardiothoracic surgery backup were available. This case highlights perioperative considerations in pre-lung-transplant iPAH patients presenting for non-cardiac surgery."

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

Airway Lasering with Precision: Juggling Systemic Pulmonary Hypertension and Multiple Cardiac Arrests from Airway Collapse (ASA 2025) - "Medications include tadalafil, ambrisentan, nitric oxide (NO) and oxygen for pulmonary hypertension. Significant pulmonary hypertensive crises occurred and near cardiac arrest. After completing successful DLB, patient had persistent bronchomalacia requiring further intervention and cardiac arrest a week later."

Cardiovascular • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

COMBINED EFFECT OF ATORVASTATIN AND AMBRISENTAN ON THE HEMODYNAMIC ALTERATIONS AND LIVER FIBROSIS IN A TRANSLATIONAL RAT MODEL OF MASLD WITH ADVANCED FIBROSIS (AASLD 2025) - "The combination of Ato and Amb demonstrated a synergistic effect reversing liver fibrosis 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

Pseudo-pseudo Meigs syndrome in a patient with overlap syndrome: A case report and literature review. (PubMed, Medicine (Baltimore)) - "This case emphasizes the importance of considering PPMS in autoimmune disease patients with unexplained ascites and elevated CA-125 levels, while suggesting a potential association between PAH medications and PPMS development."

Journal • Review • Cardiovascular • Hypertension • Immunology • Inflammatory Arthritis • Lupus • Multiple Sclerosis • Oncology • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Lupus Erythematosus • Systemic Sclerosis • MUC16

Targeting Endothelin A and Angiotensin type 1 receptors with Ambrisentan and Losartan ameliorates cisplatin-induced injury in human tubuloids. (PubMed, Nephrol Dial Transplant) - No abstract available

Journal

ACTION: The Impact of ERA Switching on Risk Stratification in Pulmonary Arterial Hypertension (clinicaltrials.gov) - P=N/A | N=121 | Recruiting | Sponsor: University of Sao Paulo General Hospital | Not yet recruiting ➔ Recruiting | Trial completion date: Jun 2026 ➔ Dec 2026 | Trial primary completion date: Aug 2025 ➔ Aug 2026

Enrollment open • Trial completion date • Trial primary completion date • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Initial Body Weight as an Important Factor for Improving the Reliability and Translational Relevance of the Preclinical Monocrotaline-Induced Rat Pulmonary Hypertension Model. (PubMed, Int J Mol Sci) - "Thus, our study aimed to examine the influence of initial BW on the severity of PH development induced by monocrotaline (MCT) and the effectiveness of the reference combined therapy (ambrisentan and tadalafil given for 21 days). The effectiveness of the reference therapy was also most evident in Set II, where the reversal of PH-related aggravations was best documented. We demonstrated that both the severity of MCT-induced PH in rats and the effectiveness of the reference combined therapy strongly depend on the animals' initial BW."

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

Characterization of the Intermediate-Risk Population in Patients with Pulmonary Arterial Hypertension Group I: CAPRI Registry in a Latin American country (ERS 2025) - "The use of bosentan decreased from 36% (63/175) to 28% (46/164) (p < 0.001), while ambrisentan increased from 17.7% (31/175) to 28.7% (47/164). Median survival was slightly higher in the low-risk group, and an improvement in functional capacity was observed, along with an increase in the use of parenteral prostanoids."

Clinical • Cardiovascular • Congestive Heart Failure • Heart Failure • Pulmonary Arterial Hypertension • Respiratory Diseases

BAIN: Nefecon and Ambrisentan in IgA Nephropathy (clinicaltrials.gov) - P4 | N=129 | Recruiting | Sponsor: The First Hospital of Jilin University | Not yet recruiting ➔ Recruiting

Enrollment open • Chronic Kidney Disease • Glomerulonephritis • IgA Nephropathy • Nephrology • Renal Disease

In brief: Embryotoxicity REMS removal for endothelin receptor antagonists. (PubMed, Med Lett Drugs Ther) - No abstract available

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