ambrisentan / Generic mfg. 
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  • ||||||||||  ambrisentan / Generic mfg., sildenafil / Generic mfg.
    CPET-DIRECTED OXYGEN THERAPY: A BREATH OF FRESH AIR FOR PATIENTS WITH PAH (Hall B4-5) -  Jan 26, 2024 - Abstract #ACC2024ACC_4122;    
    She showed limited symptom relief and continued disease progression despite the use of ambrisentan and sildenafil. Further investigations are warranted to ascertain the role of CPET in aiding physicians to identify patients who would benefit from oxygen therapy, and to further explore the benefits of oxygen therapy in patients with PAH.
  • ||||||||||  ambrisentan / Generic mfg., tadalafil / Generic mfg.
    UNEXPECTED EISENMENGER'S: COMPENSATED LARGE PATENT DUCTUS ARTERIOSUS (Hall B4-5) -  Jan 26, 2024 - Abstract #ACC2024ACC_875;    
    Left-sided chamber dilation was notably absent despite years of pulmonary over-circulation. This implies that the increases in pulmonary vascular resistance developed gradually with subsequent attenuation of the left to right shunt.
  • ||||||||||  Ventavis (iloprost) / Bayer, J&J, University of Copenhagen, Opsumit (macitentan) / Nippon Shinyaku, J&J, Tracleer (bosentan) / J&J, Roche
    P4 data, Journal, Real-world evidence, Real-world:  Liver injury due to endothelin receptor antagonists: a real-world study based on post-marketing drug monitoring data. (Pubmed Central) -  Jan 9, 2024   
    According to the results of the study, bosentan had the highest risk of liver injury and the combination regimens bosentan?+?sildenafil, bosentan?+?epoprostenol, and bosentan?+?iloprost had a stronger risk of liver injury. From the early stages of treatment, we need to regularly monitor the liver function of patients, especially for females and the elderly, and discontinue the suspected drug as soon as the liver injury occurs.
  • ||||||||||  Treatment of Pulmonary Arterial Hypertension in Patients with Connective Tissue Diseases: A Meta-analysis (Poster Hall; in person) -  Sep 23, 2023 - Abstract #ACRConvergence2023ACR_Convergence_3585;    
    This is the first meta-analysis on CTD-PAH that reported the pooled analysis of change in functional class, hemodynamic measurements (RAP, PVR, CI), and NT-proBNP, some of which have important prognostic value for PAH. Improvement in exercise capacity and reduction in risk of CW in CTD-PAH patients were less pronounced compared to idiopathic PAH (IPAH).
  • ||||||||||  Tracleer (bosentan) / J&J, Roche
    Journal, HEOR:  Cost-utility of ambrisentan and bosentan for pediatric pulmonary arterial hypertension. (Pubmed Central) -  Jul 5, 2023   
    The QALYs per person estimated for ambrisentan were 0.39 (95% CI 0.381-0.382), whereas for bosentan it was 0.40 (95% CI 0.401-0.403). Our economic evaluation shows that ambrisentan is not cost-effective regarding bosentan to in treating pulmonary arterial hypertension in C.
  • ||||||||||  Adempas (riociguat) / Bayer, Merck (MSD), Nplate (romiplostim) / Amgen, Kyowa Kirin, Rituxan (rituximab) / Biogen, Zenyaku Kogyo, Roche
    "Between Scylla and Charybdis" - Severe Right Ventricular Dysfunction Due to Chronic Thromboembolic Pulmonary Hypertension (CTEPH) and Life Threatening Thrombocytopenia (Walter E. Washington Convention Center, Area J, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_8307;    
    After extensive multidisciplinary team discussions, he underwent Pulmonary thromboendarterectomy (PTE) and Inferior vena caval stent placement. Outcome- Followup Ventilation Perfusion (V/Q) scan showed no evidence of pulmonary embolism, platelet counts improved significantly,pulmonary pressures improved remarkably on RHC and 4 years later, he continues to do well.Discussion - Managing this patient with life threatening hemoptysis, RV failure, cardiogenic shock cardiorenal syndrome and multiorgan dysfunction in the setting of significant thrombotic burden,requiring intense parenteral anticoagulation, features suggestive of catastrophic Anti Phospholipid Syndrome( CAPS ),was extremely daunting and to complicate things further, there was worsening of thrombocytopenia with high risk of further bleeding.A focused, aggressive multidisciplinary approach with timely surgical intervention led to successful outcome.
  • ||||||||||  Tracleer (bosentan) / J&J, Roche, Tivicay (dolutegravir) / ViiV Healthcare
    Endothelin Receptor Antagonists (ERA) and Integrase Inhibitors: Competition or Cooperation? (Walter E. Washington Convention Center, Area B, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_6919;    
    All HIV-PAH patients who were using ERA (ambrisentan or bosentan) at our service from 2010 to 2021 were evaluated, a total of 12. Of these, data from 3 patients were excluded because they didn
  • ||||||||||  ambrisentan / Generic mfg., sildenafil / Generic mfg.
    A Case of Severe Pulmonary Arterial Hypertension and Cardiac Tamponade: A Clinical Dilemma (Walter E. Washington Convention Center, Area B, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_6836;    
    They were able to decrease diastolic pressures, but not enough cause right sided systolic failure. This may be an alternative management strategy to perhaps optimize outcomes in similar cases.
  • ||||||||||  ambrisentan / Generic mfg., sildenafil / Generic mfg.
    DAH in PAH: Vaping Associated Lung Injury in a PAH Patient (Walter E. Washington Convention Center, Area B, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_6834;    
    It is well known that many PAH patients are diagnosed as a relatively young age. Given the prevalence of vape use amongst this age group, it is increasingly important to understand the impact of EVALI on the PH population and highlights the importance of abstinence from vaping among this population.
  • ||||||||||  ambrisentan / Generic mfg., sildenafil / Generic mfg.
    Unilateral Pulmonary Artery Atresia - A Rare Case of Pulmonary Hypertension (Walter E. Washington Convention Center, Area B, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_6830;    
    Overall mortality is 7%, with PH and hemoptysis limiting preoperative survival1. Long term prognosis in those patients with persistent pulmonary hypertension following surgical management of UPAA is uncertain.
  • ||||||||||  Opsumit (macitentan) / Nippon Shinyaku, J&J, Tracleer (bosentan) / J&J, Roche
    Pulmonary Hypertension Associated With Bronchopulmonary Dysplasia in Adults (Walter E. Washington Convention Center, Area B, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_5288;    
    She was treated with Ambrisentan and Tadalafil; on surveillance RHC, PVR decreased to 3.7WU; functional class(FC) had also improved from FCIII to FCII.Case 2: 33-year-old male born 4 weeks premature with BPD, persistent fetal circulation and required tracheostomy for chronic respiratory failure until age 7...Bosentan was changed to Macitentan for convenience, but 15 years later, echocardiography and hemodynamics worsened...This case series is the first to use pulmonary vasodilators for treatment of BPD-PH. While initial results are promising, further studies are needed to fully evaluate treatment for adult BPD-PH patients.
  • ||||||||||  ambrisentan / Generic mfg.
    It's Complicated: Dyspnea and Hypoxemia in a Patient With Portal Hypertension (Walter E. Washington Convention Center, Area B, Hall C (Lower Level)) -  Mar 25, 2023 - Abstract #ATS2023ATS_5271;    
    Subsequent annual TTEs have showed sustained improvement in RVSP (most recently 32 mmHg) and grossly preserved RV function on monotherapy. Although HPS and POPH are frequently considered
  • ||||||||||  A RARE CASE OF TEMPI SYNDROME WITH PULMONARY HYPERTENSION (eAbstract Site) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_6402;    
    These findings highlight a new role of ETR/β-arr1 operating an inside-out integrin activation to modulate the metastatic process and suggest that in the new integrin-targeting programs might be considered that ETR/β-arr1 regulates Intα5β1 functional pathway. She was diagnosed with TEMPI syndrome a year ago (A history of dyspnea, edema and rash, elevated RBC 6.41
  • ||||||||||  ambrisentan / Generic mfg., sildenafil / Generic mfg.
    MANAGEMENT DILEMMA OF PULMONARY HYPERTENSION SECONDARY TO TOTAL PNEUMONECTOMY (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_6397;    
    She was diagnosed with TEMPI syndrome a year ago (A history of dyspnea, edema and rash, elevated RBC 6.41 The ideal management of PH following pneumonectomy is unknown, but treatment with Group 1 PH medications may be effective.
  • ||||||||||  VentaProst (epoprostenol inhalation) / Aerogen Pharma
    HYPOXIA, HEMOPTYSIS AND HEADACHE: A CASE OF EISENMENGER SYNDROME DUE TO A LARGE VSD IN A YOUNG ADULT (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_6333;    
    Despite this, she experienced worsening hypoxia (requiring 40L high-flow nasal cannula) so pulmonary vasodilator (PVD) therapy was escalated to IV epoprostenol and inhaled nitric oxide which did significantly improve oxygenation...Given the concern for PA thrombosis, anticoagulation (AC) with warfarin was initiated...The AC was reversed and inhaled tranexamic acid was administered...She was ultimately able to be transitioned to long-term PVD therapy of ambrisentan and sildenafil and discharged home on only 2L oxygen. ES is a life-threatening cause of PAH in adults and carries significant morbidity.
  • ||||||||||  nitroprusside / Generic mfg.
    DELAYED PULMONARY VASODILATOR RESPONSE TO SODIUM NITROPRUSSIDE (Poster Hall_Hall F) -  Jan 4, 2023 - Abstract #ACCWCC2023ACC_WCC_6332;    
    Background: The 2022 ESC pulmonary hypertension (PH) guidelines describe acute vasoreactivity as a drop of >10 mmHg in mean pulmonary artery pressure (PAP) to <40 mmHg with an increase or no change in cardiac output (CO) on right heart catheterization (RHC) in response to inhaled nitrous oxide, inhaled iloprost, or intravenous (IV) epoprostenol...For this reason, we describe a case of vasodilatory challenge (VDC) with SNP in which there was a delayed response in PAP.Case: 45-year-old male with a history of severe Group I pulmonary arterial hypertension on home Ambrisentan 5 mg daily, and Tadalafil 40 mg daily, presented with worsening shortness of breath... Prolonged exposure to vasodilator therapy may be required to diagnose delayed responders to vasodilator therapies and to identify an important patient population which may gain benefit from ALR in treatment of their mixed pre- and post-capillary PH.