Long-term Effects on Gas Exchange of Nonselective Endothelin Receptor Antagonist (ETRA) Bosentan in Patients With Severe Pulmonary Hypertension Associated With COPD or Idiopathic Chronic Interstitial Pneumonia

CHEST Journal ◽  
2010 ◽  
Vol 138 (4) ◽  
pp. 365A
Author(s):  
Anna A. Stanziola ◽  
Michele D'Alto ◽  
Emanuela Carpentieri ◽  
Paola Argiento ◽  
Christian Russo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Gas Exchange ◽  
Receptor Antagonist ◽  
Interstitial Pneumonia ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Severe Pulmonary Hypertension ◽  
Long Term Effects

scholarly journals Multidimensional fatigue in pulmonary hypertension: prevalence, severity and predictors

2018 ◽  
Vol 4 (1) ◽  
pp. 00079-2017 ◽  
Author(s):  
Todd M. Tartavoulle ◽  
Aryn C. Karpinski ◽  
Andrew Aubin ◽  
Benzi M. Kluger ◽  
Oliver Distler ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Pulmonary Hypertension ◽  
Receptor Antagonist ◽  
Body Mass ◽  
Phosphodiesterase Inhibitor ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Physical Fatigue ◽  
General Fatigue ◽  
Reduced Activity

Pulmonary hypertension is a potentially fatal disease. Despite pharmacological advances in pulmonary hypertension, fatigue remains common in patients with pulmonary hypertension.A convenience sample of 120 participants at an international patient conference completed the Multidimensional Fatigue Inventory (MFI)-20 scale. Data on New York Heart Association Functional Class, body mass index, oxygen use and medication type/use were also collected.There was a high prevalence of “severe” to “very severe” fatigue for each dimension: General Fatigue (60%), Physical Fatigue (55.8%), Reduced Activity (41.7%), Reduced Motivation (32.5%) and Mental Fatigue (27.5%). The mean±sd overall MFI-20 score was 58±5.1. Dimensions with the highest averaged levels were General Fatigue (13.40±3.61), Physical Fatigue (13.23±3.67) and Reduced Activity (11.33±4.16). Body mass index correlated with higher fatigue scores. Phosphodiesterase inhibitor plus endothelin receptor antagonist combination negatively predicted General Fatigue, Physical Fatigue, Reduced Motivation and Reduced Activity. Triple therapy was a significant predictor of General Fatigue, Physical Fatigue and Reduced Activity. There were no significant predictors of Mental Fatigue.Multidimensional fatigue is common and severe in patients with pulmonary hypertension. Phosphodiesterase inhibitor plus endothelin receptor antagonist combination resulted in lower scores in most fatigue dimensions. Comprehensive assessment of fatigue should be considered in the clinical care of patients with pulmonary hypertension and clinical research to develop formal interventions that target this disabling symptom.

scholarly journals Efficacy and safety of riociguat in combination therapy for patients with pulmonary arterial hypertension (PATENT studies)

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402094212
Author(s):  
Hossein-Ardeschir Ghofrani ◽  
Ekkehard Grünig ◽  
Pavel Jansa ◽  
David Langleben ◽  
Stephan Rosenkranz ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Combination Therapy ◽  
Receptor Antagonist ◽  
Endothelin Receptor Antagonist ◽  
Walking Distance ◽  
Endothelin Receptor ◽  
Pretreated Patients ◽  
Sequential Combination ◽  
Pulmonary Arterial

Many patients with pulmonary arterial hypertension do not achieve treatment goals with monotherapy, and therefore combination therapy is becoming the standard of care. The soluble guanylate cyclase stimulator riociguat is licensed for the treatment of pulmonary arterial hypertension; here we present findings from patients who were receiving combined riociguat plus endothelin receptor antagonists or non-intravenous prostanoids in the randomized, placebo-controlled PATENT-1 study and its open-label extension (PATENT-2). Moreover, we include new data from patients receiving early sequential combination therapy (three to six months of endothelin receptor antagonist treatment) or long-term background endothelin receptor antagonist therapy (>6 months). Patients were randomized to riociguat 2.5 mg–maximum ( N = 131 pretreated patients) and placebo ( N = 60 pretreated patients). Riociguat improved 6-min walking distance (PATENT-1 primary endpoint), functional capacity, and hemodynamics after 12 weeks in pretreated patients. The placebo-corrected changes in 6-min walking distance were +24 m in endothelin receptor antagonist-pretreated patients and +106 m in the small group of prostanoid-pretreated patients. In the early sequential combination and long-term background endothelin receptor antagonist groups, the placebo-corrected changes in 6-min walking distance were +65 m (95% CI: 17 to 113 m) and +13 m (95% CI: –8 to 33 m), respectively. In conclusion, these data suggest that early sequential combination of an endothelin receptor antagonist plus riociguat is a feasible treatment option. Both early sequential therapy and long-term background endothelin receptor antagonist plus riociguat were well tolerated in the PATENT studies.

scholarly journals Safety and efficacy of the endothelin receptor antagonist macitentan in pediatric pulmonary hypertension

2020 ◽  
Vol 10 (5) ◽  
pp. 1675-1685
Author(s):  
Sabrina Schweintzger ◽  
Martin Koestenberger ◽  
Axel Schlagenhauf ◽  
Gernot Grangl ◽  
Ante Burmas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Receptor Antagonist ◽  
Endothelin Receptor Antagonist ◽  
Endothelin Receptor ◽  
Safety And Efficacy
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