- |||||||||| Adempas (riociguat) / Bayer, Uptravi (selexipag) / J&J, Nippon Shinyaku, warfarin / Generic mfg.
A 100-Year-Old CTEPH Patient Treated with Combined Pulmonary Artery Vasodilators (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area B) - Mar 15, 2020 - Abstract #ATS2020ATS_10435; Additionally, our case highlights a successful combined regimen of riociguat and selexipag in treating CTEPH. Further studies are needed to evaluate this combination in CTEPH patients who are not surgical candidates.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku, Tyvaso (treprostinil) / United Therapeutics
Epoprostenol-Associated Ascites in Pulmonary Arterial Hypertension: Rare, Treatable and Persistently Inexplicable (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area B) - Mar 15, 2020 - Abstract #ATS2020ATS_9956; Furthermore, three of the four patients clearly did not exhibit a high-output state at the time ascites developed, leaving the mechanism of development unclear. Lastly, transitioning to alternative non-epoprostenol therapies, including other prostacyclin mimetics, such as intravenous treprostinil and oral selexipag, appears to be effective therapy.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
A Novel Selexipag Transition Protocol: Parenteral Therapy to Maximum Dose Selexipag in Four Days (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7187; We continue to recommend caution in Scleroderma-associated PAH. Patients with pulmonary hypertension on parenteral prostacyclin therapy can be safely transitioned to maximum dose selexipag in four days without short-term adverse effects.
- |||||||||| Tyvaso (treprostinil) / United Therapeutics
Real-World Transitions from Parenteral, Inhaled, and Oral Prostacyclin-Class Therapies to Oral Treprostinil: Interim Data from the ADAPT Registry (PENNSYLVANIA CONVENTION CENTER, Hall D-E (200 Level), Area D) - Mar 15, 2020 - Abstract #ATS2020ATS_7185; P=N/A 56% of patients transitioned from parenteral treprostinil, 22% from inhaled treprostinil, 19% from selexipag, and 4% from epoprostenol. In this real-world population, patients transitioning to ORE were most often on dual PAH Background therapy and achieved higher median ORE TDD across the first 6 months, with similar incidence of prostacyclin-related AEs, compared to non-transition patients.
- |||||||||| 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.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Real-World Experience Selecting Patients for a Selexipag-Based Triple Oral Combination Regimen to Treat Pulmonary Arterial Hypertension (PHILADELPHIA MARRIOTT DOWNTOWN, Grand Ballroom Salon B-D (Level 5)) - Mar 15, 2020 - Abstract #ATS2020ATS_3154; This regimen was chosen mostly as maintenance therapy for intermediate- and low-risk patients who had improved on parenteral prostacyclin therapy or for those who had not achieved treatment goals on double combination therapy. ST-COMBO was also commonly used as an alternative to inhaled prostacyclin therapies due to ease of use, and finally was utilized as a practical alternative in patients who could not be treated with parenteral therapy, regardless of risk status.
- |||||||||| Uptravi (selexipag) / J&J
Trial completion date, Trial primary completion date: A Clinical Study of to Confirm the Doses of Selexipag in Children With Pulmonary Arterial Hypertension (clinicaltrials.gov) - Jan 14, 2020 P2, N=55, Recruiting, Phase classification: P2/3 --> P2 | N=150 --> 74 | Trial completion date: Feb 2025 --> Jul 2023 | Initiation date: Sep 2019 --> Mar 2020 | Trial primary completion date: Jun 2022 --> Oct 2022 Trial completion date: Dec 2025 --> May 2026 | Trial primary completion date: Sep 2020 --> Feb 2026
- |||||||||| Uptravi (selexipag) / J&J
Enrollment change, Trial completion date: ACT-293987 in Pulmonary Arterial Hypertension (clinicaltrials.gov) - Dec 20, 2019 P3, N=1193, Active, not recruiting, These post-hoc analyses of GRIPHON provide valuable information about a large population of patients with corrected CHD-PAH, and suggest that selexipag may delay disease progression and was well-tolerated in patients with corrected CHD-PAH. N=670 --> 1193 | Trial completion date: Feb 2021 --> Oct 2023
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Clinical, Retrospective data, Journal: Baseline history of patients using selexipag for pulmonary arterial hypertension. (Pubmed Central) - Nov 21, 2019 The majority of patients with PAH remained on the same therapy in the 12 months prior to selexipag initiation despite high rates of healthcare utilization and increasing costs. Mean medical costs appeared to decrease after adding or switching to selexipag.
- |||||||||| Adempas (riociguat) / Bayer, University of Pittsburgh, Uptravi (selexipag) / J&J, Nippon Shinyaku, Opsumit (macitentan) / Nippon Shinyaku, J&J
Journal: New drugs, therapeutic strategies, and future direction for the treatment of Pulmonary Arterial Hypertension. (Pubmed Central) - Oct 30, 2019 In this review, we discuss the use of newly-approved drugs for PAH treatment with already known mechanisms of action (macitentan), innovative targets (riociguat and selexipag), and novel therapeutic approaches with initial up-front combination therapy. Secondly, we describe new potential signalling pathways and investigational drugs with promising role in the treatment of PAH.
- |||||||||| Review, Journal: Pharmacotherapy for pulmonary arterial hypertension. (Pubmed Central) - Oct 22, 2019
Earlier clinical trials involving PAH-specific therapies evaluated improvements in 6-minute walk time as a primary improvement whereas contemporary trials have been larger and focused on morbidity and mortality reductions. While there may be a role for monotherapy in disease management, most patients should be considered for dual or triple therapy.
- |||||||||| Tyvaso (treprostinil) / United Therapeutics
REAL-WORLD CHARACTERISTICS OF PATIENTS INITIATING ORAL TREPROSTINIL IN THE ADAPT REGISTRY: INTERIM BASELINE CHARACTERISTICS (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 5) - Sep 25, 2019 - Abstract #CHEST2019CHEST_2976; P=N/A At initiation, the majority of patients were on at least 1 background PAH therapy and those with available baseline clinical data appear to be at higher risk (FC III/IV, 6MWD <440 m). Patients transitioning from alternative PCY-class therapy had a higher median starting dose of oral treprostinil compared to PCY de novo starts.
- |||||||||| sildenafil / Generic Mfg., Uptravi (selexipag) / J&J, Nippon Shinyaku
HOLEY HEART: FISTULIZING STAPHYLOCOCCAL ENDOCARDITIS (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 4) - Sep 25, 2019 - Abstract #CHEST2019CHEST_2407; This case is an unusual presentation of a rare complication of infectious endocarditis. In a patient with bacteremia, acute decompensation of hemodynamics with conduction abnormalities or a widened pulse pressure should be thoroughly worked-up to evaluate for valvular regurgitation, aortic dissection, or fistula formation.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
PULMONARY ARTERIAL HYPERTENSION PATIENTS TRANSITIONED FROM PARENTERAL PROSTACYCLINS TO SELEXIPAG: HEMODYNAMIC AND LONG TERM OUTCOMES (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 3) - Sep 25, 2019 - Abstract #CHEST2019CHEST_1944; Background oral therapy consisted of an endothelin receptor antagonist and riociguat or tadalafil...In selected PAH patients, hemodynamics, RV function and clinical outcomes can be maintained long term following transition from parenteral prostacyclins to selexipag. While there was 1 failure may be due to mitigating circumstances of the discontinuation of antiretroviral treatment in a patient with HIV associated PAH.Clinical Implications:Selexipag appears to be a viable long term oral option for the transition of patients from parenteral prostacyclin therapy while on additinal background oral pulmonary vasodilators.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
TRANSITIONING FROM INHALED ILOPROST TO ORAL SELEXIPAG: A CASE REPORT (Ernest N. Morial Convention Center - Exhibit Hall - Poster Area 5) - Sep 25, 2019 - Abstract #CHEST2019CHEST_1891; While there was 1 failure may be due to mitigating circumstances of the discontinuation of antiretroviral treatment in a patient with HIV associated PAH.Clinical Implications:Selexipag appears to be a viable long term oral option for the transition of patients from parenteral prostacyclin therapy while on additinal background oral pulmonary vasodilators. The TRANSIT-1 study looked into transition from inhaled Treprostinil to oral Selexipag (SXP) over the course of 16 weeks[1]...We report the safe transition of a patient with connective tissue disease associated pulmonary arterial hypertension (CTD-PAH) from ILO over to SXP.Case Presentation:52 y/o woman with SLE-SSc overlap, Raynauds syndrome, CTD-PAH, & resolved past APLA-related PEs on indefinite warfarin, had been on Bosentan 125mg BID & ILO 5mcg 6 inhalations a day for many years...Our case report suggests that a relatively rapid & safe transition from ILO over to SXP is possible in clinically stable patients with CTD-PAH & relatively better preserved functional status.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Review, Journal: Prostacyclin for pulmonary arterial hypertension. (Pubmed Central) - Sep 12, 2019 Selexipag demonstrated less clinical worsening without discernable impact on survival, increased adverse events; and the effect on other outcomes is less certain. Real-world registry data may provide further information about clinical effect.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Journal: T cell Large Granular Lymphocytic Leukemia with Pulmonary Hypertension. (Pubmed Central) - Sep 11, 2019 He was treated for his leukemia with methotrexate and simultaneously treated for his pulmonary hypertension with selexipag and ambrisentan. As his leukemia improved, we also noticed an improvement in his pulmonary hypertension from a NYHA class IV to class I. Hence, we believe there is an etiopathological link between the T cell large granular leukemia and associated pulmonary hypertension.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Journal: Selexipag in the management of pulmonary arterial hypertension: an update. (Pubmed Central) - Sep 10, 2019 Efficacy in terms of improvement in effort tolerance, hemodynamic and mortality benefit is less than seen with IV therapy. This is the first prostanoid demonstrated in a clinical trial to have added benefit in those on background double combination therapy and the first non IV prostanoid to demonstrate outcome benefit in the connective tissue disease (CTD) population in a randomized controlled trial.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku
Role of Selexipag in COPD Patients with out of Proportion Pulmonary Hypertension () - Sep 9, 2019 - Abstract #HFSA2019HFSA_111; sIn this case series of COPD patients with out of proportion pulmonary hypertension, the use of selexipag was associated with an improvement in functional status at 6 months. Larger size prospective studies are needed to confirm the findings.
- |||||||||| sildenafil / Generic Mfg., Uptravi (selexipag) / J&J, Nippon Shinyaku
NTP42, an antagonist of the thromboxane receptor, attenuates experimentally-induced pulmonary arterial hypertension (7B) - Aug 26, 2019 - Abstract #ERS2019ERS_5252; A multiparameter score of key disease indices, including mPAP, RVSP, Fulton’s index, vessel remodelling, inflammation and fibrosis, shows that NTP42 has significant treatment benefits and superior to the SoCs tested. These findings suggest that NTP42 and antagonism of TP signalling may alleviate PAH pathophysiology, representing a novel therapeutic target with marked benefits over existing therapies.
- |||||||||| Tyvaso (treprostinil) / United Therapeutics
Journal: The prostacyclin analogue treprostinil in the treatment of pulmonary arterial hypertension. (Pubmed Central) - Aug 14, 2019 This MiniReview will assess the benefits and drawbacks of treprostinil in the treatment of PAH by examining its specific mechanism of action and pharmacological properties, such as pharmacokinetics, pharmacodynamics, adverse effects and interactions. In addition, we will analyse and discuss results from different clinical trials, comparing treprostinil's four different forms to each other as well as to other drugs targeting the prostacyclin pathway.
- |||||||||| Uptravi (selexipag) / J&J, Nippon Shinyaku, Opsumit (macitentan) / Nippon Shinyaku, J&J
Clinical, Journal, Combination therapy: Combination Therapy in Pulmonary Arterial Hypertension: Gleaning a Practical Approach from the Randomized Trials. (Pubmed Central) - Aug 7, 2019 Currently, Food and Drug Administration-approved agents are predominantly pulmonary vasodilators acting through different pathways, with minimal impact on progression of the proliferative pulmonary arteriopathy that is the key pathologic finding in PAH. It is to be hoped that treatment strategies that result in halting progression and substantial reversal of pulmonary arteriolar obstruction will soon be discovered and available.
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