Trial reportBritish journal of haematology2010
Exercise capacity and haemodynamics in patients with sickle cell disease with pulmonary hypertension treated with bosentan: results of the ASSET studies.
Trial report in British journal of haematology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 4 registered trials, which are not on this map. Cited by 65 papers, 10 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Randomized, Placebo-controlled, Double-blind, Multicenter, Parallel Group Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Symptomatic Pulmonary Arterial Hypertension Associated With Sickle Cell Disease
Randomized, Placebo-controlled, Double-blind, Multicenter, Parallel Group Study to Assess the Efficacy, Safety and Tolerability of Bosentan in Patients With Symptomatic Pulmonary Hypertension Associated With Sickle Cell Disease
Long-Term, Open-Label, Multicenter, Extension Study of Bosentan in Patients With Pulmonary Hypertension Associated With Sickle Cell Disease Completing a Double-Blind ASSET Study (AC-052-368 or AC 052-369)
The Safety and Efficacy of Macitentan for Treatment of Pulmonary Hypertension in Sickle Cell Disease
Who cites it
65 citing papers in PubMed, 10 syntheses or guidelines pooled it, 128 citations in OpenAlex.
- Endothelin receptor antagonists for pulmonary arterial hypertension.The Cochrane database of systematic reviews · 2021Pooled it
- Combined Methods (Formal Adjusted Indirect Comparison, Meta-Analysis and Principal Component Analysis) Comparisons of the Safety and Efficacy of Ambrisentan, Bosentan, and Sildenafil in the Patients With Pulmonary Arterial Hypertension.Frontiers in pharmacology · 2020Pooled it
- American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease.Blood advances · 2019Guideline
- Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease.The Cochrane database of systematic reviews · 2019Pooled it
- Clinical Adverse Effects of Endothelin Receptor Antagonists: Insights From the Meta-Analysis of 4894 Patients From 24 Randomized Double-Blind Placebo-Controlled Clinical Trials.Journal of the American Heart Association · 2016Pooled it
- Pooled it
- Regular long-term red blood cell transfusions for managing chronic chest complications in sickle cell disease.The Cochrane database of systematic reviews · 2016Pooled it
- Systematic review of interventional sickle cell trials registered in ClinicalTrials.gov.Clinical trials (London, England) · 2015Pooled it
- An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease.American journal of respiratory and critical care medicine · 2014Guideline
- Endothelin receptor antagonists for pulmonary arterial hypertension.The Cochrane database of systematic reviews · 2013Pooled it
- Blockade of the mineralocorticoid receptor improves markers of human endothelial cell dysfunction and hematological indices in a mouse model of sickle cell disease.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2023Trial
- Sickle cell disease-associated pulmonary hypertension: an integrated framework linking pathologies, mechanisms, and clinical phenotypes.EBioMedicine · 2026Review
- Endothelin receptor antagonism in sickle cell disease: Is the time now?Canadian journal of physiology and pharmacology · 2026Review
- Cardiopulmonary Hemodynamic Determinants of Long-Term Survival in Sickle Cell Disease: Insights from the ETENDARD Study.American journal of respiratory and critical care medicine · 2025Article
- Article
- Macitentan Administration for Pulmonary Hypertension Due to β-thalassemia with Multiple Organ Failure.Internal medicine (Tokyo, Japan) · 2024Article
- Pulmonary Hypertension in Sickle Cell Disease: Novel Findings of Gene Polymorphisms Related to Pathophysiology.International journal of molecular sciences · 2024Review
- The Cardiopulmonary Complications of Sickle Cell Disease.Hematology/oncology clinics of North America · 2022Review
- Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.Journal of thrombosis and thrombolysis · 2022Article
- Treatment dilemmas: strategies for priapism, chronic leg ulcer disease, and pulmonary hypertension in sickle cell disease.Hematology. American Society of Hematology. Education Program · 2021Review
5 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
12 authors at 11 institutions in 2 countries.
Funding
Abstract
Doppler-defined pulmonary hypertension (PH) in sickle cell disease (SCD) is associated with 40% mortality at 40 months. To assess the effect of bosentan in SCD-PH, two randomized, double-blind, placebo-controlled, 16-week studies were initiated. Safety concerns are particularly relevant in SCD due to comorbid conditions. ASSET-1 and -2 enrolled patients with pulmonary arterial hypertension (PAH) and pulmonary venous hypertension (PH), respectively. Haemodynamics and 6-min walk distance (6MWD) were obtained at baseline and week 16. The studies were terminated due to slow site initiation and patient enrolment (n = 26). Bosentan appeared to be well tolerated. Although sample sizes were limited, in ASSET-1 at baseline, 6MWD correlated with cardiac output (CO; P = 0.006) with non-significant inverse correlations between 6MWD and pulmonary vascular resistance (PVR; P = 0.07) and between 6MWD and right atrial pressure (P = 0.08). In ASSET-2 at baseline, there was a non-significant correlation between 6MWD and CO (P = 0.06). Due to limited sample sizes, efficacy endpoints were not analysed. However, in both studies, non-significant increases in CO were observed with bosentan compared to placebo. Similarly, non-significant decreases in PVR were observed with bosentan. Limited data in SCD-PH suggest that a low 6MWD predicts a low CO. Standard-dose bosentan appears to be well tolerated. Further investigation is warranted. Clinicaltrials.gov registration numbers NCT00310830, NCT00313196, NCT00360087.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.