ambrisentan / Generic mfg. - LARVOL DELTA

Ambrisentan retains its pro-autophagic activity on human pulmonary artery endothelial cells exposed to hypoxia in an in vitro model mimicking diabetes (ESC-WCC 2025) - "In hPAEC exposed to Hx, AMB retains its pro-autophagic effects in an in vitro model mimicking diabetes. miR124-3p and, to a lesser extent miR191-3p, may act as biomarkers of disease and treatment response to specific drugs in patients with PAH and diabetes."

Preclinical • Cardiovascular • MIR124-3 • MIR191

Causal Inference of Adverse Drug Events in Pulmonary Arterial Hypertension: A Pharmacovigilance Study. (PubMed, Pharmaceuticals (Basel)) - " Signal detection revealed that Ambrisentan, Bosentan, and Iloprost were associated with serious AEs, including death, dyspnea, pneumonia, and edema. This study quantifies causal drug-event relationships in PAH using Bayesian causal networks. The findings offer valuable evidence regarding the clinical safety of PAH medications, thereby improving patient health outcomes."

Adverse events • Journal • Cardiovascular • Endocrine Disorders • Hematological Disorders • Hypertension • Infectious Disease • Pneumonia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Characterization of the Population With Intermediate-Risk Status in Pulmonary Arterial Hypertension: Patients in a Latin American Country: The CAPRI Registry. (PubMed, Pulm Circ) - "The use of bosentan decreased from 36% (63/175) to 28% (46/154) (p < 0.001), while ambrisentan increased from 18% (31/175) to 30% (47/154), and macitentan increased from 25% (44/175) to 36% (57/154). The use of parenteral prostanoids and selexipag increased by 20% (4% vs. 24%) and 3% (1% vs. 4%) during clinical follow-up, respectively...At baseline, most patients were classified as intermediate-low-risk. However, during clinical follow-up, risk distribution changed, with a notable proportion shifting across different risk categories."

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

Phosphodiesterase type 5 inhibitor plus endothelin receptor antagonist compared to either alone for group 1 pulmonary arterial hypertension. (PubMed, Cochrane Database Syst Rev) - "Combination therapy for PAH offers benefits over monotherapies, reducing clinical worsening compared to ERA alone (high certainty). Their benefits over PDE5i are less certain, although potentially favourable when studies at high risk of bias are excluded. Hospitalisation rates are likely reduced with combination therapy compared to ERA, but the effect is very uncertain when combination therapy is compared to PDE5i. Uncertainty also persists regarding its impact on mortality and functional outcomes, such as 6MWD and WHO functional class. Serious adverse events and withdrawal rates are similar between combination therapy and monotherapies, with varying levels of certainty, although withdrawals may favour combination therapy over PDE5i. Comparative analyses of PDE5i and ERA provided mixed results with varying levels of certainty. These findings could inform whether initial combination therapy should become the standard of care in people with group 1 PAH with WHO functional..."

Clinical • Journal • Review • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Rare Diseases • Respiratory Diseases

ORIENTATION: Comparison of Sequential to Initial Combination Therapy in PAH (clinicaltrials.gov) - P=N/A | N=376 | Recruiting | Sponsor: Second Affiliated Hospital, School of Medicine, Zhejiang University | Not yet recruiting ➔ Recruiting

Enrollment open • Head-to-Head • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

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

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

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

Statement of retraction: clinical adverse events to letairis: a real-world drug safety study based on FDA Adverse Event Reporting System (FAERS). (PubMed, Expert Opin Drug Saf) - No abstract available

Adverse events • Journal • Real-world evidence

Hereditary hemorrhagic telangiectasia with pulmonary hypertension: a report of three cases. (PubMed, J Thorac Dis) - "Following diagnosis, Case 1 received treatment consisting of bevacizumab in conjunction with tadalafil and macitentan, Case 2 was treated with thalidomide in combination with sildenafil and macitentan, and Case 3 received a combination of sildenafil and ambrisentan. It is imperative for healthcare professionals to exercise heightened suspicion of the diagnosis in patients with PH and telangiectasia of the skin and mucous membranes, and to undertake a comprehensive investigation into the family history. The implementation of genetic testing is crucial to mitigate the occurrence of missed diagnoses and misdiagnoses of this disease."

Journal • Cardiovascular • Genetic Disorders • Hematological Disorders • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • ACVRL1

Reverse Bernheim Phenomenon - A True Enigma. (PubMed, Respir Med Case Rep) - "He was treated with ambrisentan, sildenafil and selexipag. The patient's PAH therapy was subsequently escalated with addition of intravenous treprostinil, which led to improvement of exercise capacity and resolution of syncopal episodes. A repeat stress echocardiogram revealed resolution of the acute exercise-induced RV dilatation and septal bulging without compressing the LVOT."

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

Brain Weight Effect of Ambrisentan in Juvenile Rat Toxicity Studies Associated With Breathing Sounds, Apnea, and Sustained Hypoxemia. (PubMed, Birth Defects Res) - "It's postulated that the brain weight decrease was mediated by sustained hypoxemia during a period of rapid brain growth. Improper interaction of rat laryngeal tissues, in close apposition during early postnatal stages, may constitute a sensitive period. Clinical relevance is unknown; palatal/laryngeal maturation timing in healthy children supports up to ~2 years as the period for any potential risk. However, for children with PAH, chronic hypoxemia and/or concomitant conditions associated with developmental delay could hypothetically extend the sensitive age period for potential risk through the third year of life."

Journal • Preclinical • Cardiovascular • Developmental Disorders • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • EDN1

Prostacyclin Therapy as Adjunctive Treatment for Pulmonary Hypertension in Pediatric Heart Transplantation: A Case Report. (PubMed, Pediatr Transplant) - "This case highlights that aggressive medical management, including prostacyclin therapy, can render even high-risk pediatric patients with elevated pulmonary vascular resistance eligible for heart transplant and improve post-operative outcomes. It supports emerging evidence that elevated pulmonary vascular resistance, particularly when responsive to vasodilators, should not be viewed as an absolute contraindication to heart transplantation and emphasizes the importance of tailored, ongoing pulmonary hypertension management in children with congenital heart disease."

Journal • Cardiovascular • Congestive Heart Failure • Heart Failure • Hypertension • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Transplantation

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

New P4 trial • Chronic Kidney Disease • Glomerulonephritis • IgA Nephropathy • Nephrology • Renal Disease

Thermodynamic and Extra-Thermodynamic Investigations of Bioactive Compounds With Endothelin A Receptor by Nonlinear Chromatography. (PubMed, Biomed Chromatogr) - "Using endothelin receptor A (ETAR) as an example, ETAR was immobilized on ibrutinib-modified silica gels to prepare affinity stationary phase...The four ligands were ambrisentan, paeoniflorin (PAE), ferulic acid (FA), and salvianolic acid B (SAB)...This result was also confirmed by a survey of DrugBank. Together, the method may become an alternative for predicting the druglike property of a newly discovered bioactive compound."

Journal • Cardiovascular • Hypertension • 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=121 | Not yet recruiting | Sponsor: University of Sao Paulo General Hospital

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

Efficacy and safety of ambrisentan in maintenance hemodialysis patients with secondary pulmonary hypertension: a multicenter, randomized, controlled, open-label study (ChiCTR) - P4 | N=220 | Not yet recruiting | Sponsor: The Second Affiliated Hospital of Army Medical University (Xinqiao Hospital); The Second Affiliated Hospital of Army Medical University (Xinqiao Hospi

New P4 trial • Cardiovascular • Hypertension • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases

Juvenile Systemic Sclerosis Presenting With Severe Pulmonary Vascular Disease: ECMO Stabilization During Aggressive Therapy Resulting in a Favorable Outcome. (PubMed, Pulm Circ) - "Veno-arterial extracorporeal membranous oxygenation (VA-ECMO) and atrial stent placement facilitated treatment with pulse-dose steroids, mycophenolate mofetil, and B-cell depleting therapy to treat the underlying autoimmune inflammation and triple therapy with treprostinil, ambrisentan, and tadalafil for her pulmonary hypertension. At 9-month follow-up, her jSSc is well-controlled with complete resolution of her PH. This case demonstrates that multi-disciplinary treatment, including upfront multi-drug therapy for jSSC and PAH, that included VA-ECMO, may improve outcomes, particularly when treatment for underlying causes (in this case, jSSc) is just being initiated."

Journal • Cardiovascular • Critical care • Hypertension • Immunology • Inflammation • Pediatrics • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

Transitioning From Parenteral Treprostinil to LIQ861 in a Patient With PAH (ATS 2025) - "This is the first case report describing transitioning a patient with PAH from intravenous treprostinil to LIQ861.Case description:Herein, we present a case of a 42-year-old female with idiopathic PAH, oxygen dependent, initially NYHA II, who was enrolled in the INSPIRE study on August 23, 2018 and transitioned from Tyvaso 12 breaths QID to LIQ861 79.5ug QID in combination with sildenafil and macitentan. On Day 2174, NYHA II, 6MWD 320 meters in combination with LIQ861 212ug QID, Ambrisentan 10mg and Sildenafil 40mg TID. Inhaled Treprostinil therapy can offer comparable efficacy to parenteral routes in the management of PAH, while reducing systemic side effects and eliminating the need for subcutaneous or intravenous access. To our knowledge, this is the first case report of transitioning between LIQ861 and I.V. Treprostinil."

Clinical • Cardiovascular • Congestive Heart Failure • Heart Failure • Pain

Maternal Pulmonary Arterial Hypertension Treated With IV Epoprostenol - A Case Report (ATS 2025) - "Her pre-gestation medications included Ambrisentan, Selexipag, and Sildenafil...The patient was then transitioned from IV Epoprostenol to inhaled Treprostinil via dry powder inhaler...IV Epoprostenol may be used adjunctively in the management of pregnant PAH patients and could improve peri and postpartum outcomes. This case adds to a growing literature base on this subject; the difficulties of performing a blinded, randomized clinical trial in this cohort are acknowledged."

Case report • Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Discussion on Role of Imatinib Therapy in Scleroderma with Suspected PVOD (ATS 2025) - "We report a case of a patient with cardiogenic shock and suspected PVOD related to scleroderma, managed with Imatinib.Case Report: A 55-year-old male with CREST syndrome-associated Group 1 PAH, on home subcutaneous Remodulin, presented with dyspnea and leg edema...Due to worsening hypoxemia, he was cannulated for venoarterial extracorporeal membrane oxygenation (VA ECMO), diuresed, and started on Ambrisentan and inhaled Veletri...PVOD remains a challenge in pulmonary hypertension due to diagnostic difficulties, limited treatment options, and poor prognosis. Continued research into its pathogenesis, improved diagnostics, and targeted therapies, supported by advances in genetics and molecular biology, is essential for better management of this condition."

Cardiovascular • Fibrosis • Heart Failure • Hepatology • Immunology • Interstitial Lung Disease • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • Scleroderma • Systemic Sclerosis

A Real-world Pharmacovigilance Study on Endothelin Receptor Antagonists for Treatment of Pulmonary Artery Hypertension (ATS 2025) - "We collected majorly reported serious adverse events for Macitentan, Bosentan, and Ambrisentan since their introduction in the market following FDA approval. Real-world data for adverse events differs between currently available ERAs. FAERS database is useful for clinicians and researchers to identify adverse events."

Adverse events • Clinical • Real-world • Real-world evidence • Cardiovascular • Hypertension • Infectious Disease • Pneumonia • Pulmonary Arterial Hypertension

Patients With History of Methamphetamine Use Are More Likely to Have Risk Evaluation and Mitigation Strategy Medications for Pulmonary Arterial Hypertension Stopped Due to Non-adherence (ATS 2025) - "Patients with PAH were included if they were prescribed at least one of the REMS drugs (riociguat, macitentan, ambrisentan) and had an office visit with a pulmonary hypertension specialist during the study period. Methamphetamine-associated PAH remains challenging to manage. Patients with Meth-PAH are more likely to be non-adherent to REMS drugs and active use further increases the likelihood of non-adherence. Males with Meth-PAH appear more likely to be non-adherent to REMS drugs than females."

Adherence • Clinical • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

Effective Pharmacotherapy in Hereditary Pulmonary Arterial Hypertension: A Case of BMPR2 Mutation (ATS 2025) - "An initial vasodilator challenge with Flolan improved cardiac output and reduced PVR, but pulmonary pressures remained high, necessitating a comprehensive treatment plan.The patient was started on tadalafil, a phosphodiesterase-5 inhibitor, known for reducing pulmonary pressures and enhancing exercise capacity in PAH. However, initial trials with the endothelin receptor antagonist macitentan were unsuccessful due to side effects, including significant facial and lower extremity swelling. Consequently, ambrisentan, another endothelin receptor antagonist, was considered as a potential alternative for better tolerance...Additionally, the patient's treatment included Bumex diuretics to manage fluid overload and losartan for blood pressure control...The addition of selexipag significantly improved her quality of life. This case highlights the critical role of pharmacotherapy in managing PAH associated with BMPR2 mutation."

Clinical • Cardiovascular • Congestive Heart Failure • Gastrointestinal Disorder • Heart Failure • Hematological Disorders • Hypertension • Immunology • Inflammatory Arthritis • Lupus • Obesity • Pulmonary Arterial Hypertension • Pulmonary Embolism • Respiratory Diseases

Unmasked Pulmonary Hypertension After Successful Liver Transplantation (ATS 2025) - "Case Descriptions: Patient 1: A 60-year-old woman with cirrhosis secondary to metabolic dysfunction-associated fatty liver disease and mild PoPH on sildenafil underwent uncomplicated LT...She was treated with ambrisentan and tadalafil with clinical improvement, and eventually underwent renal transplantation with fistula ligation...Early recognition of PH in the post-operative period, especially in the setting of pre-transplant HPS, is vital to ensure timely treatment of these patients. Further cases should be identified and described to help clarify the pathophysiological basis for this phenomenon."

Cardiovascular • Congestive Heart Failure • Fibrosis • Heart Failure • Hepatitis C • Hepatology • Immunology • Infectious Disease • Inflammation • Metabolic Disorders • Metabolic Dysfunction-Associated Steatotic Liver Disease • Nephrology • Pulmonary Arterial Hypertension • Pulmonary Disease • Renal Disease • Respiratory Diseases • Transplantation