- |||||||||| Opsumit (macitentan) / Nippon Shinyaku, J&J, tadalafil / Generic mfg.
[VIRTUAL] Initial Combination Therapy with Macitentan and Tadalafil in Pulmonary Arterial Hypertension (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_4701;
Conclusion In a real-world setting, macitentan and tadalafil as initial combination therapy for PAH was generally well tolerated and yielded significant clinical benefits. Nearly 40% of patients improved to a low risk profile, which has been associated with excellent 5-year transplant-free survival.
- |||||||||| Bosulif (bosutinib) / Pfizer
[VIRTUAL] Bosutinib-induced Pulmonary Arterial Hypertension in a Patient with Chronic Myelogenous Leukemia (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_4648;
Though not a standard therapy for PAH, imatinib has been noted to improve pulmonary hemodynamics and exercise capacity in small studies. Given the the growing number of cases of both dasatinib and bosutinib induced PAH, regular screening for pulmonary toxicity for patients taking certain TKIs should be considered as previous review articles have suggested.
- |||||||||| paroxetine / Generic mfg.
[VIRTUAL] Development of Pulmonary Arterial Hypertension After Neck Paraganglioma and Vagal Nerve Resection (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_4637;
The patient was diagnosed with idiopathic PAH and started on ambrisentan and sildenafil...Of note, she was on the SSRI paroxetine, which is known to cause mild elevations in serum catecholamines...Likewise, increasing parasympathetic activity with oral pyridostigmine has been shown to delayed progression to RV failure using the sugen-hypoxia model in rats. Further studies in humans are needed to elucidate the roles of the autonomic nervous system in PAH.
- |||||||||| ambrisentan / Generic mfg., sildenafil / Generic mfg., Thalomid (thalidomide) / Fujimoto, BMS
[VIRTUAL] A Case of Pulmonary Arteriovenous Malformation in the Setting of Pulmonary Arterial Hypertension (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_4632;
Thalidomide may be used to treat AVMs in HHT, however, it may not be useful in idiopathic microscopic PAVMs. Remaining treatment options include embolization or lobectomy - both of which can be life threatening due to sudden increase in pressures post therapy.
- |||||||||| [VIRTUAL] Using Cardiopulmonary Exercise Testing to Evaluate the Effects of Target Medicine for Pulmonary Artery Hypertension (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_3427;
6MWD was significantly related to peak VO 2 , peak VO 2 /kg, VE/VCO 2 slope, peak HR, peak systolic pressure (r =0.641, P =0.002; r =0.562, P =0.008; r =-0.600, P =0.011; r =0.451, P =0.040; r =0.499, P =0.021). CONCLUSION : CPET can objectively and quantitatively evaluate the effects of target medicine for pulmonary artery hypertension, and correlate with the traditional index.
- |||||||||| ambrisentan / Generic mfg., tadalafil / Generic mfg.
[VIRTUAL] Application of Machine Learning in Pulmonary Arterial Hypertension Risk Stratification (ATS 2020 Virtual) - Jul 6, 2020 - Abstract #ATSI2020ATS-I_349;
A BN model for PAH (PHORA) demonstrated an improvement in accuracy over the existing multi-variate model of the REVEAL 2.0 calculator that persisted when externally validated in two large PAH registries and a contemporary clinical trial. This improvement stems from the BN model's ability to understand both the dynamic influences of each risk factor on each other, as well as with the outcome itself.
- |||||||||| ambrisentan / Generic mfg., atorvastatin / Generic mfg.
[VIRTUAL] Synergic effect of a combined treatment with atorvastatin and ambrisentan in a rat model of NASH (Poster Area) - May 30, 2020 - Abstract #EASLILCI2020EASL-ILC-I-1459;
Further analysis of the short and long-term effects of this combination of therapies on RV remodeling is ongoing. In our NASH model rats, the combination of ambrisentan and atorvastatin exerts a potent inhibition of HSC contractility, and thus intrahepatic resistance, resulting in decreased portal pressure.
- |||||||||| ambrisentan / Generic mfg.
Clinical, PK/PD data, Journal: Pharmacokinetics of ambrisentan in a patient with Pulmonary Arterial Hypertension and a total gastrectomy. (Pubmed Central) - Apr 16, 2020
However, since it was not clear whether oral agents (specifically ambrisentan) would be absorbed and, if so, what the appropriate dose would be, we performed pharmacologic evaluation of ambrisentan absorption in this patient. These investigations identified an effective dose which led to successful transition from epoprostenol.
- |||||||||| Clinical, Journal, HEOR: Medication adherence, hospitalization, and healthcare resource utilization and costs in patients with pulmonary arterial hypertension treated with endothelin receptor antagonists or phosphodiesterase type-5 inhibitors. (Pubmed Central) - Apr 11, 2020
Rates and numbers of rehospitalizations within 30 days post-discharge were similar between groups. Mean total costs were higher with endothelin receptor antagonists than phosphodiesterase type-5 inhibitors in all patients ($91,328 versus $72,401, P = 0.0003) and in adherent patients ($88,867 versus $56,300, P < 0.0001), driven by higher drug costs.
- |||||||||| ambrisentan / Generic mfg.
Trial completion date: Long-term Ambrisentan Extension Study for Pediatric Patients Who Participated in AMB112529 (clinicaltrials.gov) - Apr 7, 2020
P2, N=66, Active, not recruiting,
Mean total costs were higher with endothelin receptor antagonists than phosphodiesterase type-5 inhibitors in all patients ($91,328 versus $72,401, P = 0.0003) and in adherent patients ($88,867 versus $56,300, P < 0.0001), driven by higher drug costs. Trial completion date: Jun 2024 --> Oct 2024
- |||||||||| Adempas (riociguat) / Bayer, Merck (MSD)
Trial completion date, Trial primary completion date, Combination therapy: Upfront Combination Pulmonary Arterial Hypertension Therapy (clinicaltrials.gov) - Mar 27, 2020
P4, N=20, Recruiting,
This method is now being used for study samples and clinical patient samples. Trial completion date: Jan 2020 --> Jan 2021 | Trial primary completion date: Dec 2019 --> Dec 2020
- |||||||||| Review, Journal: Endothelin Receptor Antagonists: Status Quo and Future Perspectives for Targeted Therapy. (Pubmed Central) - Mar 23, 2020
Treatment with approved endothelin receptor antagonists (ERAs), such as bosentan, ambrisentan, or macitentan, slow down PAH progression and relieves symptoms...Furthermore, in the SONAR (Study Of Diabetic Nephropathy With Atrasentan) trial, the selective ERA atrasentan reduced the risk of renal events in patients with diabetes and chronic kidney disease...Preclinical studies regarding heart failure, cancer, and fibrotic diseases have demonstrated promising effects, but clinical trials have not yet produced measurable results. Nevertheless, the potential benefits of ERAs may not be fully realized.
- |||||||||| ambrisentan / Generic mfg., imatinib / Generic mfg., sildenafil / Generic mfg.
A Unique Case of Pulmonary Tumor Thrombotic Microangiopathy (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area B) - Mar 15, 2020 - Abstract #ATS2020ATS_9967;
Additionally, sildenafil and ambrisentan have also shown some benefit. Due to the highly fatal disease, further studies aiming at improving our survival in PTTM should be undertaken.
- |||||||||| Remodulin (treprostinil diethanolamine oral) / United Therapeutics, ambrisentan / Generic mfg., tadalafil / Generic mfg.
Pulmonary Capillary Hemangiomatosis; Pathologic Findings of a Rare Cause of Pulmonary Hypertension (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area B) - Mar 15, 2020 - Abstract #ATS2020ATS_9939;
In figure 1, slides A through D show significant pulmonary edema in addition to diffuse nodular thickening and proliferation of the alveolar capillary loops, which is characteristic of pulmonary arteriopathy.DISCUSSIONPulmonary capillary hemangiomatosis is a rare, progressive disease and can ultimately only be confirmed with biopsy and pathological analysis. The vascular invasion characteristic of PCH often results in a secondary veno-occlusive phenomenon which can explain hypoxemia, vascular congestion and right-heart failure, and is almost always uniformly fatal.
- |||||||||| Benlysta (belimumab) / GSK, cyclophosphamide intravenous / Generic mfg.
Belimumab in the Treatment of Interstitial Lung Disease Associated with Systemic Lupus Erythematosus (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area H) - Mar 15, 2020 - Abstract #ATS2020ATS_9207;
Cyclophosphamide was switched to azathioprine in view of hemorrhagic cystitis...As demonstrated in our case, Belimumab also has the potential to delay ILD associated with CTDs progression and improve clinical outcomes. To our knowledge there has been no formal study to quantify the effects of Belimumab in patients with CTD associated ILD and is a potential venue to explore.
- |||||||||| Opsumit (macitentan) / Nippon Shinyaku, J&J, tadalafil / Generic mfg.
Initial Combination Therapy with Macitentan and Tadalafil in Pulmonary Arterial Hypertension (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7201;
Change in secondary endpoints of 6MWD and hemodynamic variables are summarized below (Table B).ConclusionIn a real-world setting, macitentan and tadalafil as initial combination therapy for PAH was generally well tolerated and yielded significant clinical benefits. Nearly 40% of patients improved to a low risk profile, which has been associated with excellent 5-year transplant-free survival.
- |||||||||| HIV-Related Pulmonary Hypertension: 10-Year Experience in a General Hospital in Chicago (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7149;
In our study we found similar 2-year mortality rates compared to published literature. HRPAH management with PAH therapy combined with ART seems to not only improve functional status and severity of the disease, but also survival rates.
- |||||||||| Bosulif (bosutinib) / Pfizer
Bosutinib-induced Pulmonary Arterial Hypertension in a Patient with Chronic Myelogenous Leukemia (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7142;
Though not a standard therapy for PAH, imatinib has been noted to improve pulmonary hemodynamics and exercise capacity in small studies. Given the the growing number of cases of both dasatinib and bosutinib induced PAH, regular screening for pulmonary toxicity for patients taking certain TKIs should be considered as previous review articles have suggested.
- |||||||||| paroxetine / Generic mfg.
Development of Pulmonary Arterial Hypertension After Neck Paraganglioma and Vagal Nerve Resection (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7131;
Additional workup included a CT chest without features of emphysema or interstitial lung disease, a normal V/Q scan and a negative rheumatologic workup.The patient was diagnosed with idiopathic PAH and started on ambrisentan and sildenafil...Of note, she was on the SSRI paroxetine, which is known to cause mild elevations in serum catecholamines.Autonomic dysfunction is an important feature of RV failure associated with PAH...Likewise, increasing parasympathetic activity with oral pyridostigmine has been shown to delayed progression to RV failure using the sugen-hypoxia model in rats. Further studies in humans are needed to elucidate the roles of the autonomic nervous system in PAH.
- |||||||||| ambrisentan / Generic mfg., sildenafil / Generic mfg., Thalomid (thalidomide) / Fujimoto, BMS
A Case of Pulmonary Arteriovenous Malformation in the Setting of Pulmonary Arterial Hypertension (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7126;
Thalidomide may be used to treat AVMs in HHT, however, it may not be useful in idiopathic microscopic PAVMs. Remaining treatment options include embolization or lobectomy - both of which can be life threatening due to sudden increase in pressures post therapy.
- |||||||||| Using Cardiopulmonary Exercise Testing to Evaluate the Effects of Target Medicine for Pulmonary Artery Hypertension (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area B) - Mar 15, 2020 - Abstract #ATS2020ATS_5809;
After the treatment of target medicines (sildenafil for 22 patients, tadalafil for 6 patients, ambrisentan for one patient, bosentan for one patient, iloprost for one patient, combined target medication for 2 patients),peak VO2, peak VO2/kg, peak VO2/heart rate (HR), peak workload, peak PetCO2, 6MWD were improved significantly (P<0.001, P<0.001, P=0.002, P<0.001, P<0.001, P=0.008), and rest VE/VCO2, VE/VCO2 slope, lowest VE/VCO2, lowest VE/VO2, NT-proBNP were significantly decreased (P=0.018, P=0.041, P=0.003, P=0.019, P<0.001). CPET can objectively and quantitatively evaluate the effects of target medicine for pulmonary artery hypertension, and correlate with the traditional index.
- |||||||||| ambrisentan / Generic mfg., tadalafil / Generic mfg.
Application of Machine Learning in Pulmonary Arterial Hypertension Risk Stratification (PHILADELPHIA MARRIOTT DOWNTOWN, Grand Ballroom Salon E (Level 5)) - Mar 15, 2020 - Abstract #ATS2020ATS_3293;
A BN model for PAH (PHORA) demonstrated an improvement in accuracy over the existing multi-variate model of the REVEAL 2.0 calculator that persisted when externally validated in two large PAH registries and a contemporary clinical trial. This improvement stems from the BN model’s ability to understand both the dynamic influences of each risk factor on each other, as well as with the outcome itself.
- |||||||||| ambrisentan / Generic mfg., Opsumit (macitentan) / Nippon Shinyaku, J&J, Tracleer (bosentan) / J&J, Roche
[VIRTUAL] COSTS OF TREATMENTS OF PULMONARY HYPERTENSION IN POOR POPULATION OF COLOMBIA. () - Mar 8, 2020 - Abstract #ISPOR2020ISPOR_1137;
With these data and with a prevalence of 52 cases per 1.000.000 habitants, we could say that the annual economic burden of PAH treatment would be about $5,505,328. These findings create awareness and provide valuable information for the development of strategies aimed at controlling costs and avoiding financial risks associated to it.
- |||||||||| ambrisentan / Generic mfg.
Clinical, Journal: Long-Term Response to Vasoactive Treatment in a Case of Kyphoscoliosis-Associated Pulmonary Hypertension. (Pubmed Central) - Feb 7, 2020
She was diagnosed with pulmonary hypertension in 2016 and had been on endothelin receptor antagonist (ambrisentan) and oxygen therapy ever since...CONCLUSIONS Managing the respiratory decline in kyphoscoliosis-related pulmonary hypertension can be challenging since these patients tend to deteriorate despite current treatment options. Our case reports on the use of vasoactive agents as a safe and effective treatment option in addition to established therapeutic regimen.
- |||||||||| ambrisentan / Generic mfg., tadalafil / Generic mfg.
Journal, Combination therapy: Risk-stratified outcomes with initial combination therapy in pulmonary arterial hypertension: Application of the REVEAL risk score. (Pubmed Central) - Jan 30, 2020
Our case reports on the use of vasoactive agents as a safe and effective treatment option in addition to established therapeutic regimen. This post-hoc analysis comparing outcomes by REVEAL risk group has shown that, at all levels of risk, patients enrolled in AMBITION receiving initial combination therapy have superior outcomes and, even in those assessed as low risk, initial combination therapy was clinically beneficial.
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