Bosentan or Macitentan Therapy in Chronic Thromboembolic Pulmonary Hypertension?

Thor, M.C.J. van; Klooster, Liesbeth ten; Snijder, Repke J.; Kelder, Johannes C.; Mager, Johannes J.; Post, Marco C.

doi:10.1007/s00408-019-00274-9

Cited by 7 publications

(5 citation statements)

References 37 publications

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“…A total of 64.5% of patients were under monotherapy treatment with Bosentan (60%) and sildenafil (25%) which are generally known for the management of patients with PAH. Prior to the publication of the CHEST-1 study, the use of Bosentan was supported by studies such as BENEFIT [9,12,18,19].…”

Section: Discussionmentioning

confidence: 99%

Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study)

Fajardo-Rivero

Mogollón

García-Bohórquez

et al. 2021

JoR

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Chronic thromboembolic disease (CTEPH) is one of the causes for developing pulmonary hypertension (PH). PH is characterized by an increase in pulmonary vascular pressure and resistance, ultimately leading to chronic overload. This study describes the clinical, functional, and hemodynamic characteristics as well as the established treatment strategy for a cohort of patients diagnosed with CTEPH in Bucaramanga, Colombia. In Colombia, PH is considered as an orphan disease with limited epidemiological data. We aim to provide useful information in order to help guide future clinical decisions for PH treatment and prevention. We conducted a cross-sectional study, obtaining clinical data from patients under follow-up, over 18 years of age, with hemodynamic confirmation of CTEPH in two pulmonary outpatient centers in Bucaramanga, Colombia between 2012 and 2018. 35 patients with diagnosis of CTEPH were included. Mean age was 52.3 ± 17.9 years. The mean time between the onset of symptoms to diagnosis was 14 months. 71% had a previous thrombotic event and 69% had functional class III and IV according to the world health organization (WHO) criteria. Most of the patients were classified as at high risk of mortality according to the European Society of Cardiology (ESC) and the European Respiratory Society (ERS/ESC) criteria and 60% were referred to undergo thromboendarterectomy. Most of the patients were under monotherapy treatment with Bosentan, the most prescribed medication in both monotherapy and dual therapy. This study identified a high number of patients in advanced stages of CETPH due to late diagnosis, related to health care limitations. This resulted in worse prognosis and quality of life. In addition, low adherence to non-pharmacological interventions was evidenced in patients who were not candidates for thromboendarterectomy despite the onset of pharmacological therapy.

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Section: Discussionmentioning

confidence: 99%

Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study)

Fajardo-Rivero

Mogollón

García-Bohórquez

et al. 2021

JoR

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“…Bedan et al indicated that macitentan has a similar mechanism of action as bosentan, however, it has a longer duration of action and exhibits higher antagonistic potency than bosentan and ambrisentan in pulmonary smooth muscle cells [24] . Considering that both bosentan and macitentan are nonselective ERAs it can be assumed that these drugs will have a similar impact not only on patients with PH, as it was demonstrated by Thor et al, but also on patients with ARDS [23] , [24] . However, this hypothesis needs a clinical confirmation.…”

Section: Endothelin Receptor Antagonistsmentioning

confidence: 91%

“…There are two more endothelin receptor antagonists used in treatment of pulmonary hypertension, which are ambrisentan, a selective ETA antagonist and macitentan, a nonselective ETA/ETB antagonist [23] , [24] . Although these drugs are effective in treatment of PH, there are no studies investigating their impact in treatment of ARDS.…”

Section: Endothelin Receptor Antagonistsmentioning

confidence: 99%

“…Although these drugs are effective in treatment of PH, there are no studies investigating their impact in treatment of ARDS. Thor et al investigated 112 patients with PAH treated with either bosentan or macitentan and discovered no significant difference in therapy outcome and two-year survival rate [23] . Bedan et al indicated that macitentan has a similar mechanism of action as bosentan, however, it has a longer duration of action and exhibits higher antagonistic potency than bosentan and ambrisentan in pulmonary smooth muscle cells [24] .…”

Section: Endothelin Receptor Antagonistsmentioning

confidence: 99%

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Pulmonary artery targeted therapy in treatment of COVID-19 related ARDS. Literature review

Puk

Nowacka

Smulewicz

et al. 2022

Biomedicine & Pharmacotherapy

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Introduction The most grievous complication of the COVID-19 is the acute respiratory distress syndrome. A specific, rescue treatment for rapidly deteriorating patients should emerge to improve respiratory function and help patients to survive the most challenging period. Drugs used in targeted therapy of pulmonary arterial hypertension (PAH) appears to be suitable for this task and this article describes their potential for treatment of severe cases of COVID-19. Methods The authors reviewed the following databases for randomized controlled trials, reviews and meta-analyses published up to July 2020: Pubmed, Scopus, Google Scholar, Cochrane Database and ClinicalKey. The authors included every study contributory to the assessment of the potential of drugs used in targeted PAH therapy in treatment of COVID-19. Results Endothelin receptor antagonists, phosphodiesterase 5 inhibitors, riociguat and prostacyclin have proven ani-inflammatory effect and reduce pulmonary artery blood pressure, lung oedema and remodelling. Bosentan shows antiviral properties and sildenafil, as well as epoprostenol, inhibits apoptosis of lung epithelial cells. Among patients with lung lesions the decrease of pulmonary blood pressure can lead to increase of ventilation/perfusion mismatch and decrease of blood oxygenation. Conclusions Among all assessed drugs bosentan, sildenafil and epoprostenol appear to be most promising and a combination of these drugs should be considered due to synergism. The targeted PAH therapy in treatment of COVID-19 associated ARDS could be a useful tool saving lives of patients with severe SARS-CoV-2 infection, however, its introduction should be investigated and monitored very carefully as it can lead to transient deterioration of patient condition.

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“…47,48 Some units consider the off-label use of pulmonary vasodilators for high-risk patients as a bridging therapy for PEA surgery. [49][50][51] Further evidence is required to justify their routine use preoperatively. 46,52 All candidates for PEA are on life-long anticoagulation treatment as recommended for CTEPH patients.…”

Section: Perioperative Optimizationmentioning

confidence: 99%

Perioperative Management in Pulmonary Endarterectomy

Jenkins

Martínez

Salaunkey

et al. 2023

Semin Respir Crit Care Med

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Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thromboembolic pulmonary hypertension (PH), provided lesions are proximal enough in the pulmonary vasculature to be surgically accessible and the patient is well enough to benefit from the operation in the longer term. It is a major cardiothoracic operation, requiring specialized techniques and instruments developed over several decades to access and dissect out the intra-arterial fibrotic material. While in-hospital operative mortality is low (<5%), particularly in high-volume centers, careful perioperative management in the operating theater and intensive care is mandatory to balance ventricular performance, fluid balance, ventilation, and coagulation to avoid or treat complications. Reperfusion pulmonary edema, airway hemorrhage, and right ventricular failure are the most problematic complications, often requiring the use of extracorporeal membrane oxygenation to bridge to recovery. Successful PEA has been shown to improve both morbidity and mortality in large registries, with survival >70% at 10 years. For patients not suitable for PEA or with residual PH after PEA, balloon pulmonary angioplasty and/or PH medical therapy may prove beneficial. Here, we describe the indications for PEA, specific surgical and perioperative strategies, postoperative monitoring and management, and approaches for managing residual PH in the long term.

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Bosentan or Macitentan Therapy in Chronic Thromboembolic Pulmonary Hypertension?

Cited by 7 publications

References 37 publications

Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study)

Characterization of a Cohort of Patients with Chronic Thromboembolic Pulmonary Hypertension from Northeastern Colombia (REHINO Study)

Pulmonary artery targeted therapy in treatment of COVID-19 related ARDS. Literature review

Perioperative Management in Pulmonary Endarterectomy

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