ambrisentan / Generic mfg. - LARVOL DELTA

ORIENTATION: Comparison of Sequential to Initial Combination Therapy in PAH (clinicaltrials.gov) - P=N/A | N=372 | Not yet recruiting | Sponsor: Second Affiliated Hospital, School of Medicine, Zhejiang University

Head-to-Head • New trial • Cardiovascular • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

Mechanistic insights into the protective potential of ambrisentan against L-arginine induced acute pancreatitis and multiorgan damage (role of NRF2/HO-1 and TXNIP/NLRP3 pathways). (PubMed, Biomed Pharmacother) - "AMB effectively improved the AP and MOD produced by L-Arg through its anti-inflammatory and antioxidant properties. NRF2/HO-1 and TXNIP/NLRP3 pathways play major roles in these protective effects."

Journal • Oncology • Pancreatitis • AMPK • CASP3 • CD68 • NLRP3 • TNFA • TXNIP

Combination of Captopril, Ambrisentan and Sildenafil mitigates long-term side effects of combined heart and lung irradiation (ESTRO 2025) - P | "In rats the combination of the ACE-inhibitor Captopril with the PH medications Ambrisentan and Sildenafil effectively reduced radiation-induced loss of cardiopulmonary function even when heart and lung were both irradiated. This combination of medications is a promising intervention to prevent cardiopulmonary side effects in patients treated with thoracic radiotherapy, improving their quality of life and possibly even survival."

Adverse events • Cardiovascular • Fibrosis • Immunology • Pulmonary Arterial Hypertension • Pulmonary Disease • 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

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

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

Refractory Pulmonary Arterial Hypertension as a Presentation for a Type 1 Interferonopathy in a Toddler (ATS 2025) - "While on ECMO, he continued to have refractory PAH despite being on aggressive triple therapy (ambrisentan, sildenafil, and treprostinil)...He began to improve clinically, and he was discharged on Baricitinib and triple PAH therapy for continual close observation...While there have been case reports of Type 1 interferonopathies causing PAH and interstitial lung disease, this patient's causative gene, 9p, has only been reported to have interferonopathy-related lupus and GI involvement. This further illustrates the need for continued investigation into these newly discovered interferonopathies."

Cardiovascular • CNS Disorders • Developmental Disorders • Fatigue • Immunology • Inflammatory Arthritis • Interferonopathies • Interstitial Lung Disease • Lupus • Pulmonary Arterial Hypertension • Respiratory Diseases • Ventriculomegaly

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

Pulmonary Veno-occlusive Disease (PVOD) Contributing to Pulmonary Arterial Hypertension in an 11-year Old Boy With a Heterozygous Pathogenic Variant in EIF2AK4 (ATS 2025) - "He was hospitalized in Mexico where a repeat ECHO confirmed PH, and he was started on Sildenafil and Budesonide...He had worsening heart function on ECHO and started on triple therapy with treprostinil, ambrisentan, tadalafil and nitric oxide therapy. He was discharged home August 2024 on 1L of supplemental oxygen via nasal cannula, furosemide daily, and triple therapy with ambristentan, tadalafil and selexipag...Lung transplant remains the only definitive treatment, therefore early diagnosis and management is vital. As seen in this case, close outpatient follow up played a key role in the optimal management of PVOD despite fragmented care."

Asthma • Cardiovascular • Fatigue • Hepatology • Immunology • Infectious Disease • Influenza • Pediatrics • Pulmonary Arterial Hypertension • Respiratory Diseases

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

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

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

Comparative Efficacy of Pharmacological Treatments for Inoperable Chronic Thromboembolic Pulmonary Hypertension: A Network Meta-analysis (ATS 2025) - " A comprehensive search was conducted on PubMed, Cochrane, PloSOne, ScienceDirect, Google Scholar, clinicaltrials.gov up to october 2024.Seven randomized control trials comparing seven treatments: Bosentan(A), Sildenafil(B), Macitentan(C), Riociguat(D), Ambrisentan(E) and Selexipag(F) to placebo were included. This network meta-analysis compares pharmacological treatments for inoperable CTEPH. The findings indicate that Bosentan is the most effective treatment for reducing pulmonary vascular resistance (PVR) and NT-proBNP levels, while Riociguat shows the most significant improvement in 6MWD. These results provide insights into the efficacy of available therapies, guiding clinical decision-making for patients with inoperable CTEPH."

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

Bridging knowledge gaps: A case report of pulmonary Veno-occlusive disease in systemic sclerosis. (PubMed, Lung India) - "She was started on a combination of Ambrisentan and Tadalafil. This case emphasizes the importance of early clinical suspicion and multidisciplinary discussion in managing rare pulmonary complications associated with systemic sclerosis. Additionally, it highlights the urgent need for further research to delineate the underlying pathophysiological mechanisms and optimize therapeutic strategies for this challenging clinical entity."

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

A Budget Impact Analysis of Introducing OPSYNVI for the Treatment of Adult Patients Diagnosed With Pulmonary Arterial Hypertension (ISPOR 2025) - "Comparators included macitentan, ambrisentan, bosentan, tadalafil, sildenafil, and an ERA + PDE5i loose dose combination. Introducing OPSYNVI results in a minimal budget impact for both commercial and Medicare advantage plans. Despite its higher monthly costs, our model demonstrates that OPSYNVI could reduce hospitalization days, readmissions, and patients' progression to prostanoid. Given that PAH is a rare, progressive, fatal disease, adding OPSYNVI to a plan's formulary has negligible costs while potentially improving outcomes."

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

A real-world comparative effectiveness analysis of macitentan versus ambrisentan and bosentan on hospitalizations and healthcare resource utilization in patients with pulmonary arterial hypertension. (PubMed, Respir Med) - "Macitentan was associated with a significantly reduced risk of PAH-related and all-cause hospitalization, with lower ICU healthcare resource utilization, versus other ERAs."

HEOR • Journal • Real-world evidence • Cardiovascular • Critical care • Hypertension • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

ASPIRE: Ambrisentan Sotagliflozin and Prevention of Renal Injury; a Randomized Evaluation (clinicaltrials.gov) - P2 | N=36 | Not yet recruiting | Sponsor: University Medical Center Groningen | Trial completion date: Mar 2025 ➔ Dec 2027 | Trial primary completion date: Mar 2025 ➔ Sep 2027

Trial completion date • Trial primary completion date • Chronic Kidney Disease • Diabetes • Diabetic Nephropathy • Metabolic Disorders • Nephrology • Renal Disease • Type 1 Diabetes Mellitus

Endothelin System Blockade Extenuates Sepsis-Induced Acute Heart and Kidney Injuries via Modulating ET-1/Klotho/p38-MAPK. (PubMed, Clin Exp Pharmacol Physiol) - "Endothelin receptor antagonists (ERAs); bosentan and ambrisentan, or endothelin converting enzyme inhibitor (ECE-i) phosphoramidon, are promising agents against sepsis-induced organ damage. This was evident in their cardiorenal protective effects, up-regulation of klotho, suppression of inflammation, oxidation, apoptosis, and enhancement of the antioxidant status."

Journal • Infectious Disease • Inflammation • Septic Shock • CASP3 • EDN1 • IL6 • KL • TNFA

Ambrisentan Retains Its Pro-Autophagic Activity on Human Pulmonary Artery Endothelial Cells Exposed to Hypoxia in an In Vitro Model Mimicking Diabetes. (PubMed, J Cell Mol Med) - "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."

Journal • Preclinical • Cardiovascular • Diabetes • Hypertension • Metabolic Disorders • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases • MIR124-3 • MIR191

Healthcare Resource Utilization and Progression in Patients with Pulmonary Arterial Hypertension Initiated on Monotherapy with Macitentan versus Other Endothelin-Receptor Antagonists (ISHLT 2025) - "Time to PAH progression (first of PAH-related hospitalization, injectable prostacyclin pathway agents other than selexipag, atrial septostomy, lung transplant, and death) was evaluated using weighted Kaplan-Meier (KM) analyses.Results A total of 357 and 322 patients were included in the macitentan and other ERA cohorts (ambrisentan [92%]; or bosentan [8%]), respectively. During the study period, the other ERA cohort had higher PAH-related outpatient visits (1.04 vs 0.73 PPPM, IRR: 1.42), hospitalizations (0.06 vs 0.05 PPPM, IRR: 1.41), and re-hospitalizations within 30 days (0.01 vs 0.01 PPPM, IRR: 2.07) compared to the macitentan cohort (all p<0.05). In the KM analysis, numerically higher proportions of patients in the macitentan cohort were free of any progression endpoints at 6 months (80% vs 73%), 12 months (68% vs 61%), and 24 months (54% vs 49%).Conclusion In this retrospective US claims analysis, patients initiated on macitentan had lower PAH-related HRU and..."

Clinical • HEOR • Monotherapy • Cardiovascular • Pulmonary Arterial Hypertension • Respiratory Diseases

CONSIDERATION OF CLOSURE OF A PATENT DUCTUS ARTERIOSUS COMPLICATED BY EISENMENGER SYNDROME - Eric Bailey (ACC 2025) - "Given the presence of severe PH she was initiated on riociguat, ambrisentan, and selexipag. Eisenmenger physiology is not an absolute contraindication to PDA closure in the right clinical setting. The shunt in this case reversed evidenced by her recurrent pulmonary edema and significant left-to-right shunting, facilitating the feasibility of this procedure."

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

VALVE-IN-VALVE TRANSCATHETER MITRAL VALVE REPLACEMENT FOR SEVERE BIOPROSTHETIC MITRAL VALVE STENOSIS WITH SUPRASYSTEMIC PULMONARY HYPERTENSION - Akshitha Yarrabothula (ACC 2025) - "He was continued on sildenafil and discharged on ambrisentan for treatment of group 1 pulmonary hypertension per Pulmonology and Adult Congenital Heart Disease team recommendations. Our patient successfully underwent valve-in-valve TMVR to alleviate symptoms and improve hemodynamics. This case underscores the complexities of treating patients with adult congenital heart disease and the value of multispecialty care."

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

FIRST IMPELLA RP FLEX PLACED DURING OPEN HEART SURGERY IN THE USA - Laura Muller (ACC 2025) - "There are no prior reports of RP Flex being placed intraoperatively.Case: A 51-year-old male with a history of diabetes mellitus type 2, idiopathic pulmonary arterial hypertension (on Ambrisentan and Tadalafil), chronic right sided heart failure complicated by congestive hepatopathy with recurrent ascites, torrential tricuspid regurgitation (TR), and preserved LVEF underwent transesophageal echocardiogram and right heart catheterization (RHC) (RAP mean 26 with V waves up to 36, RVP 40/20, PAP 50/20, PAPI 1.2). The use of the RP Flex during TV replacement to support the failing RV is a novel idea that proved successful."

Surgery • Anesthesia • Cardiovascular • Congestive Heart Failure • Diabetes • Heart Failure • Hepatology • Metabolic Disorders • Pulmonary Arterial Hypertension • Respiratory Diseases • Type 2 Diabetes Mellitus

TIMING IS EVERYTHING: MANAGEMENT OF A SWISS-CHEESE INTERVENTRICULAR SEPTUM - Daniel O'Meara (ACC 2025) - "Upon diagnosis, high PVRi precluded immediate surgical repair, so she was started on tadalafil and ambrisentan to improve her surgical candidacy. If left unrepaired, multiple VSD's can cause pulmonary vascular disease with increased PVRi. Pulmonary vasodilator therapy may allow for future septation if PVRi declines to an acceptable level."

Cardiovascular • Hematological Disorders • Hemophilia • Pulmonary Arterial Hypertension • Pulmonary Disease • Respiratory Diseases

PULMONARY ARTERY ANEURYSM COMPLICATED BY PULMONARY ARTERY DISSECTION - Charles Wanna (ACC 2025) - "She was adherent with her tadalafil, ambrisentan, and subcutaneous Remodulin. PA dissection is a rare complication of chronic PAH. PA dissections have a high morbidity and mortality. For patients with advanced PAH who have too high a risk for surgical repair or monitoring, combined heart-lung transplantation is a curative option."

Cardiovascular • Congestive Heart Failure • Heart Failure • Immunology • Inflammatory Arthritis • Lupus • Pulmonary Arterial Hypertension • Pulmonary Disease • Pulmonary Embolism • Respiratory Diseases • Systemic Lupus Erythematosus