- |||||||||| ambrisentan / Generic mfg., cisplatin / Generic mfg.
Journal, IO biomarker: Inhibition of p75NTR/p53 axis by ambrisentan suppresses apoptosis and oxidative stress-mediated renal damage in a cisplatin AKI model. (Pubmed Central) - Feb 2, 2025
Ambrisentan also significantly restored oxidative balance in renal tissues as evidenced by reduced malondialdehyde and increased total antioxidant capacity and superoxide dismutase activity, in addition to decreasing nitric oxide levels (p<0.05 vs CP group). This protective effect of ambrisentan might be mediated through the downregulation of death receptor, P75NTR that in turn restores renal blood flow and oxidative balance and regulates p53, VEGF/eNOS, NF-?B, and bcl-2/Bax/caspase-3 signaling.
- |||||||||| ambrisentan / Generic mfg.
Trial completion date, Trial primary completion date: Endothelin Receptor Antagonism with Ambrisentan to Treat Hepatorenal Syndrome (clinicaltrials.gov) - Jan 30, 2025
P2, N=54, Recruiting,
This protective effect of ambrisentan might be mediated through the downregulation of death receptor, P75NTR that in turn restores renal blood flow and oxidative balance and regulates p53, VEGF/eNOS, NF-?B, and bcl-2/Bax/caspase-3 signaling. Trial completion date: Jun 2025 --> Feb 2026 | Trial primary completion date: Mar 2025 --> Dec 2025
- |||||||||| Opsumit (macitentan) / Nippon Shinyaku, J&J
Journal, Adherence: Treatment patterns and factors associated with adherence in pulmonary arterial hypertension. (Pubmed Central) - Dec 7, 2024
According to SMAQ, female sex may have a negative impact on adherence in this cohort, but PDC revealed no factors influencing it. No notable differences in adherence between treatment types were found and generally patients felt the treatments were effective in controlling their disease.
- |||||||||| ambrisentan / Generic mfg.
Journal: Enhanced Vasoconstriction in Sickle Cell Disease is Dependent on ETA Receptor Activation. (Pubmed Central) - Nov 26, 2024
Treatment with ambrisentan, a selective ETA receptor antagonist, and a ROS scavenger normalized the aortic vasoconstrictive response in HbSS mice...Taken together, these data suggest that the ETA receptor pathway interaction with the adrenergic receptor pathway contributes to enhanced aortic vasoconstriction in SCD. Findings indicate the potential of ETA antagonism as a therapeutic avenue for improving vascular health in SCD.
- |||||||||| ambrisentan / Generic mfg., sildenafil / Generic mfg., epoprostenol intravenous / Generic mfg.
Recovery from Kidney Failure Associated with Pulmonary Hypertension following Use of Vasodilators () - Oct 12, 2024 - Abstract #KIDNEYWEEK2024KIDNEY_WEEK_4967;
Prostanoids like epoprostenol demonstrate renal protective effects, improving outcomes in various kidney dysfunction contexts. Overall, these targeted therapies offer promising renal benefits in patients with PH and CKD..
- |||||||||| Review, Journal: New and Emerging Therapeutic Drugs for the Treatment of Pulmonary Arterial Hypertension: A Systematic Review. (Pubmed Central) - Sep 30, 2024
Conversely, udenafil, racecadotril, sotatercept, anastrozole, riociguat, tacrolimus, and ralinepag were found to be safe, well-tolerated, and effective in improving hemodynamic measures and 6-MWDs. This study aims to summarize the developing treatment options currently under clinical trials, highlighting the need for further trials before their application in clinical practice.
- |||||||||| ambrisentan / Generic mfg.
Protective Effect of Ambrisentan on Proteinuria in Patients with Alport Syndrome (Exhibit Hall, Convention Center) - Sep 23, 2024 - Abstract #KIDNEYWEEK2024KIDNEY_WEEK_4128;
This study aims to summarize the developing treatment options currently under clinical trials, highlighting the need for further trials before their application in clinical practice. The pilot study suggests that introducing of ERA on top of RAASi with or without SGLT2i/MRA provides an additional antiproteinuric effect in AS.
- |||||||||| epoprostenol intravenous / Generic mfg.
BREAKING BOUNDARIES: OVERCOMING TYROSINE KINASE INHIBITOR HURDLES IN CHRONIC MYELOID LEUKEMIA: A LIFESAVING ODYSSEY FROM RV RESCUE TO RENAL RECOVERY WITH IV EPOPROSTENOL (Convention Center Exhibit Hall: Rapid Fire Area 4C) - Jul 31, 2024 - Abstract #CHEST2024CHEST_4519;
CASE PRESENTATION: A 53-year-old female with a history of CML on bosutinib (previously on imatinib, dasatinib, and nilotinib), progressive kidney disease (biopsy-proven TKI-induced nephrotoxicity), diabetes mellitus and fibromyalgia presented with dyspnea with minimal exertion, orthopnea, 20+lb weight gain and significant peripheral edema...She was started on norepinephrine and dobutamine infusion for cardiogenic shock from RV failure but failed aggressive IV diuresis and was started on renal replacement therapy (RRT)...She was eventually started on ponatinib for her CML but subsequent RHC revealed near-normalization of hemodynamics while on IV epoprostenol and Ambrisentan monotherapy (Table 1)... Early treatment of PAH and RV failure with IV epoprostenol in patients with TKI-induced PAH and nephrotoxicity should be considered in conjunction with removal of the provoking agent to avoid and minimize long term comorbidities and sequela.
- |||||||||| Uptravi (selexipag) / J&J
CHARTING THE COURSE: PULMONARY HYPERTENSION AND THE DECISION TO INITIATE THERAPY (Convention Center Exhibit Hall: Poster Area 4) - Jul 31, 2024 - Abstract #CHEST2024CHEST_3024;
Group 1 PAH patients' who benefit from triple therapy at baseline, may not tolerate the same degree of pulmonary vasodilation when there is an acute pulmonary process ongoing (i.e. pneumonia, significant atelectasis, acute CHF). In these circumstances, VQ mismatch will be heightened leading to respiratory decompensation.
- |||||||||| Trevyent (treprostinil PatchPump) / Correvio, United Therapeutics Corp, treprostinil inhaled dry powder (LIQ861) / Liquidia Technologies
A MISSED DIAGNOSIS OF CONGENITAL HEART DISEASE (Convention Center Exhibit Hall: Poster Area 1) - Jul 31, 2024 - Abstract #CHEST2024CHEST_2475;
Nevertheless, cases like the one illustrated emphasize that even in the US CHD can be missed. Sustained attention and advancements in treatment strategies are crucial to enhance outcomes for individuals navigating this intricate constellation of conditions.
- |||||||||| Tracleer (bosentan) / J&J, Roche
SUSPECTED PLATYPNEA-ORTHODEOXIA IN A COMPLICATED EISENMENGER SYNDROME: RAPID DETERIORATION FOLLOWING RESPIRATORY ARREST (Convention Center Exhibit Hall: Poster Area 5) - Jul 31, 2024 - Abstract #CHEST2024CHEST_1731;
Although the exact mechanism of POS remains partially understood, its correlation with intracardiac and intrapulmonary shunts, occurring in the context of CHD such as VSD and PAH, implies a complex interaction of hemodynamic factors. The patient's rapid deterioration with suspected POS emphasizes the crucial need for careful management and preventing systemic volume depletion and hypotension, which can exacerbate right-to-left shunting resulting in hemodynamic instability.
- |||||||||| ambrisentan / Generic mfg.
Trial completion: Safety and Efficacy Study of Ambrisentan in Subjects with Pulmonary Hypertension (EUDRACT) - Jul 26, 2024
P3, N=200, Completed,
The patient's rapid deterioration with suspected POS emphasizes the crucial need for careful management and preventing systemic volume depletion and hypotension, which can exacerbate right-to-left shunting resulting in hemodynamic instability. Recruiting --> Completed
- |||||||||| ambrisentan / Generic mfg.
Journal: Removing the FDA's Boxed Hepatotoxicity Warning and Liver Function Testing Requirement for Ambrisentan. (Pubmed Central) - Jul 18, 2024
In this serial cross-sectional study of ambrisentan, labeling changes and removal of the REMS-related LFT requirement were associated with shifts in prescribing and testing behavior for ambrisentan but not bosentan. Further clinician education may be needed to maximize the benefits of REMS programs and labeling warnings designed to ensure the safe administration of high-risk medications.
- |||||||||| Tryvio (aprocitentan) / J&J
Journal: Aprocitentan (Tryvio) for hypertension. (Pubmed Central) - Jun 21, 2024
Further clinician education may be needed to maximize the benefits of REMS programs and labeling warnings designed to ensure the safe administration of high-risk medications. No abstract available
- |||||||||| Retrospective data, Review, Journal: Treatment of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review and meta-analysis. (Pubmed Central) - Apr 23, 2024
Treatment for CTD-PAH had favorable effects on clinical and hemodynamic outcomes but not on survival and NT-proBNP levels. Different from the previous meta-analyses that focused on 6-MWD, time to clinical worsening, and CW as outcomes, this meta-analysis additionally reports the pooled analysis of change in FC, hemodynamic measurements (RAP, PVR, CI), and NT-proBNP, some of which have prognostic value for PAH.
- |||||||||| Ventavis (iloprost) / Bayer, J&J, University of Copenhagen, Opsumit (macitentan) / Nippon Shinyaku, J&J, Tracleer (bosentan) / J&J, Roche
A PAH Drug Ranking Strategy for Individualized Combination Therapy Via Pulmonary Endoarterial Biopsy (San Diego Convention Center, Room 25A-C (Upper Level)) - Mar 17, 2024 - Abstract #ATS2024ATS_8762;
This study demonstrates the utility of combining an endoarterial biopsy technique with genetic expression studies to generate a ranking system that can be used to select a potentially more effective drug or drug combination regimen for an individual patient at a certain a stage of PAH disease. Human studies will be necessary to demonstrate the utility of this approach.
- |||||||||| ambrisentan / Generic mfg.
Journal: Active Anchoring Stimuli-responsive Nano-craft to relieve Pulmonary Vasoconstriction by Targeting Smooth Muscle Cell for Hypoxic Pulmonary Hypertension Treatment. (Pubmed Central) - Feb 27, 2024
Additionally, we modified the surface of the nanocarrier with sialic acid (SA) and encapsulated the clinically used drug ambrisentan (Am), which enabled the anchoring of E-selectin and subsequent drug delivery...Moreover, compared with free Am, nanoencapsulation and SA-PEG2000 -NH2 prolonged the blood circulation time, achieving better therapeutic outcomes in preventing vascular remodeling and reversing systolic dysfunction. The originality and contribution of our work reveal the promising value of this pulmonary arterial anchoring stimuli-responsive nanocraft as a novel therapeutic strategy for satisfactory PAH treatment.
- |||||||||| methamphetamine / Generic mfg.
Case Series: Marked Attenuation of Methamphetamine-associated Pulmonary Arterial Hypertension With Methamphetamine Cessation and Pharmacologic Therapy (San Diego Convention Center, Area C (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_6468;
Disease progression led to the addition of tadalafil and macitentan...She was initiated on ambrisentan, but intolerant of selexipag and PDE5i...Sildenafil and ambrisentan were initiated, with simultaneous methamphetamine cessation...He was initiated on subcutaneous Treprostinil infusion and PDE5i, with total methamphetamine cessation...Methamphetamine cessation combined with pulmonary vasodilator therapies appear to unmask a possible endotype within meth-APAH that demonstrate capability for greatly ameliorated disease. Further studies to better phenotype meth-APAH and investigate the role of methamphetamine cessation combined with PAH pharmacotherapy in altering disease course are warranted.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Pulmonary Hypertension Due to Unilateral Absence of Pulmonary Artery: A Series of 2 Cases (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_3875;
She refused parenteral therapy for PAH and was started on oral therapy with tadalafil 40 mg daily, ambrisentan 10 mg daily and Selexipag...She was started on inhaled nitric oxide transitioned to IV Treprostinil and sildenafil 40 mg TID...Therefore, pharmacotherapy with vasodilators is largely pursued. These cases highlight the need for awareness of this condition as treatment varies based on the age of diagnosis.
- |||||||||| Adempas (riociguat) / Bayer, Merck (MSD), Uptravi (selexipag) / J&J, Nippon Shinyaku
"Pop n' Drop": Two Cases of Recreational Amyl Nitrite Use Among Patients With Pulmonary Arterial Hypertension on Phosphodiesterase-5 Inhibitors Leading to Syncope (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_3872;
There are major drug interactions when amyl nitrites are taken with phosphodiesterase-5 inhibitors (PDE5i) such as sildenafil or tadalafil, resulting in profound hypotension and resultant loss of consciousness...Case Presentations: Case 1: 49-year-old male with history of WHO group I PAH secondary to HIV and methamphetamine use, on tadalafil, ambrisentan and selexipag, notes ongoing light-headedness and pre-syncope during sexual intercourse but not during other vigorous activities...Although the drug interaction between nitrates and PDE5i and riociguat is well-established, it is not commonplace to discuss the avoidance of poppers in patients with PAH. MSM on PD5i or riociguat for PAH should be screened for amyl nitrite exposure and informed of potential adverse drug interactions to prevent syncope.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Improvement in PAH After Semaglutide-induced Weight Loss (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_3871;
She was initiated on intravenous Epoprostenol and had improvement in symptoms to WHO FC II...She was transitioned to oral therapy with selexipag and ambrisentan...Table 1. Clinical and echocardiogram-derived markers of pulmonary hypertension severity by date.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Unique Presentation of Pulmonary Veno-occlusive Disease in Patient Whose Mother Had Familial Pulmonary Arterial Hypertension (San Diego Convention Center, Area K (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_3870;
At age 29 he was receiving tadalafil, ambrisentan, and selexipag...Selexipag was discontinued and IV treprostinil was initiated...The pathology in the patient's mother was consistent with PAH while his pathology revealed PVOD. This case raises the question of a "crossover" genetic variant within pulmonary vascular disease with a PAH phenotype in the proband and a PVOD phenotype in the subsequent generation vs. a novel spectrum of disease evolution.
- |||||||||| Pulmonary Capillary Hemangiomatosis: A Rare Disorder With a Nonspecific Presentation (San Diego Convention Center, Area J (Hall H, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_1895;
She was treated with empiric therapies for presumed interstitial lung disease (ILD) including exogenous steroids, methotrexate, hydroxychloroquine and later cyclophosphamide and tofacitinib...Repeat cardiac catheterization after 9 months demonstrated persistent PH, and sildenafil was started with improvement...During her admission, she had waxing and waning of her PH and she was converted to tadalafil, ambrisentan and amlodipine therapy...Identifying PCH is important as some PH therapies may be detrimental in these patients. When diagnosed, prompt referral for lung transplant is indicated.
- |||||||||| ambrisentan / Generic mfg., epoprostenol intravenous / Generic mfg., tadalafil / Generic mfg.
Cardiac Arrest After a Diagnostic Pericardiocentesis in a Patient With Severe Pulmonary Arterial Hypertension (San Diego Convention Center, Area F (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_1798;
Description: A 61-year-old woman with idiopathic PAH on epoprostenol (76 ng/kg/min), tadalafil, and ambrisentan presented with dyspnea and fluid overload...Intravenous epoprostenol was weaned to 30 ng/kg/min due to suspected over-vasodilation...It is suggested that continuous monitoring of intracardiac and pericardial pressures, combined with a gradual removal of pericardial fluid, may mitigate this risk. Our case underscores the necessity for careful patient selection when severe PAH is present and the value of invasive hemodynamic monitoring during pericardiocentesis procedures.
- |||||||||| ambrisentan / Generic mfg., azithromycin / Generic mfg., tadalafil / Generic mfg.
Azith and the Myth (San Diego Convention Center, Area A (Hall A-B2, Ground Level)) - Feb 20, 2024 - Abstract #ATS2024ATS_1604;
The mechanism of action for this process is unknown. However, discontinuation of azithromycin should be considered when extensive workup for evaluation of DAH is unrevealing.
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