Ambrisentan for treatment of inoperable chronic thromboembolic pulmonary hypertension (CTEPH)

Escribano, Pilar; Bendjenana, Hakim; Curtis, Paula; Lang, Irene; Vonk‐Noordegraaf, Anton

doi:10.1177/2045894019846433

Cited by 23 publications

(6 citation statements)

References 7 publications

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“…The AMBER 1 trial looked at ambrisentan compared to placebo in patients with inoperable CTEPH. Although the study was terminated early due to lack of enrollment, there were positive trends in 6MWD, PVR, and NT pro-BNP reported with ambrisentan [ 89 ]. Macitentan was investigated in inoperable CTEPH in a phase II RCT (MERIT-1); results showed a significant improvement in PVR at 16 weeks [ 90 ].…”

Section: Group 4 Ph: Ctephmentioning

confidence: 99%

Strategizing Drug Therapies in Pulmonary Hypertension for Improved Outcomes

et al. 2022

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Pulmonary hypertension (PH) is characterized by a resting mean pulmonary artery pressure (PAP) of 20 mmHg or more and is a disease of multiple etiologies. Of the various types of PH, pulmonary arterial hypertension (PAH) is characterized by elevated resistance in the pulmonary arterial tree. It is a rare but deadly disease characterized by vascular remodeling of the distal pulmonary arteries. This paper focuses on PAH diagnosis and management including current and future treatment options. Over the last 15 years, our understanding of this progressive disease has expanded from the concept of vasoconstrictive/vasodilatory mismatch in the pulmonary arterioles to now a better appreciation of the role of genetic determinants, numerous cell signaling pathways, cell proliferation and apoptosis, fibrosis, thrombosis, and metabolic abnormalities. While knowledge of its pathophysiology has expanded, the majority of the treatments available today still modulate the same three vasodilatory pathways that have been targeted for over 30 years (endothelin, nitric oxide, and prostacyclin). While modifying these pathways may help improve symptoms and quality of life, none of these directly modify the underlying disease pathogenesis. However, there are now studies ongoing with new drugs that can prevent or reverse these underlying causes of PAH. This review discusses the evidence base for the current treatment algorithms for PAH, as well as discusses novel therapies in development.

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Section: Group 4 Ph: Ctephmentioning

confidence: 99%

Strategizing Drug Therapies in Pulmonary Hypertension for Improved Outcomes

et al. 2022

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“…H.-A. Ghofrani et al gov: NCT01884675) [71]. Other potential treatments for CTEPH that are currently under investigation include the prostacyclin receptor agonist selexipag in persistent/recurrent and inoperable CTEPH (the Phase III SELECT study; NCT03689244) and the inhaled sGC activator BAY1237592 in patients with CTEPH or PAH (the Phase I ATMOS study; NCT03754660).…”

Section: Investigational Therapies For Ctephmentioning

confidence: 99%

Interventional and pharmacological management of chronic thromboembolic pulmonary hypertension

Ghofrani

D’Armini

Kim

et al. 2021

Respiratory Medicine

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Chronic thromboembolic pulmonary hypertension (CTEPH) is caused by obstruction of the pulmonary vasculature, leading to increased pulmonary vascular resistance and ultimately right ventricular failure, the leading cause of death in non-operated patients. This article reviews the current management of CTEPH. The standard of care in CTEPH is pulmonary endarterectomy (PEA). However, up to 40% of patients with CTEPH are ineligible for PEA, and up to 51% develop persistent/recurrent PH after PEA. Riociguat is currently the only medical therapy licensed for treatment of inoperable or persistent/recurrent CTEPH after PEA based on the results of the Phase III CHEST-1 study. Studies of balloon pulmonary angioplasty (BPA) have shown benefits in patients with inoperable or persistent/recurrent CTEPH after PEA; however, data are lacking from large, prospective, controlled studies. Studies of macitentan in patients with inoperable CTEPH and treprostinil in patients with inoperable or persistent/recurrent CTEPH showed positive results. Combination therapy is under evaluation in CTEPH, and long-term data are not available. In the future, CTEPH may be managed by PEA, medical therapy or BPAalone or in combination, according to individual patient needs. Patients should be referred to experienced centers capable of assessing and delivering all options.

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“…It also showed an improvement in 6WMD in the treatment arm (28 m vs 7 m), a marked decrease in NT-proBNP (−29.4% vs +14.1%), and a reduction in PVR. However, the trial was stopped early for futility of enrollment [124].…”

Section: Medical Managementmentioning

confidence: 99%