Trial reportPloS one2014
Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom.
Trial report in PloS one, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00492531 (Treatment of Pulmonary Hypertension and Sickle Cell Disease With Sildenafil Therapy), which is not on this map. Cited by 84 papers, 4 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.
Treatment of Pulmonary Hypertension and Sickle Cell Disease With Sildenafil Therapy
Who cites it
84 citing papers in PubMed, 4 syntheses or guidelines pooled it, 126 citations in OpenAlex.
- Mortality Associated with Intensive Care Unit Admission and Mechanical Ventilation in Adults with Acute Chest Syndrome: A Systematic Review.Medicina (Kaunas, Lithuania) · 2026Pooled it
- Pooled it
- American Society of Hematology 2019 guidelines for sickle cell disease: cardiopulmonary and kidney disease.Blood advances · 2019Guideline
- Risk factors for mortality in adult patients with sickle cell disease: a meta-analysis of studies in North America and Europe.Haematologica · 2017Pooled it
- Clinical Study to Evaluate the Possible Efficacy and Safety of L-Arginine in Children with Sickle Cell Disease and Increased Tricuspid Regurgitant Jet Velocity: a Randomized Controlled Trial.Paediatric drugs · 2025Trial
- Effect of voxelotor on cerebral perfusion and cerebral oxygen metabolism and cardiac stress in adult patients with sickle cell disease.American journal of hematology · 2025Trial
- Arginine Therapy and Cardiopulmonary Hemodynamics in Hospitalized Children with Sickle Cell Anemia: A Prospective, Double-blinded, Randomized Placebo-controlled Clinical Trial.American journal of respiratory and critical care medicine · 2022Trial
- Exercise-induced changes of vital signs in adults with sickle cell disease.American journal of hematology · 2021Trial
- Tricuspid regurgitant jet velocity and myocardial tissue Doppler parameters predict mortality in a cohort of patients with sickle cell disease spanning from pediatric to adult age groups - revisiting this controversial concept after 16 years of additional evidence.American journal of hematology · 2021Trial
- Tricuspid regurgitation velocity and other biomarkers of mortality in children, adolescents and young adults with sickle cell disease in the United States: The PUSH study.American journal of hematology · 2020Trial
- Potential Contribution of Pulmonary Thromboembolic Disease in Pulmonary Hypertension in Sickle Cell Disease.Annals of the American Thoracic Society · 2020Trial
- Thrombospondin-1 gene polymorphism is associated with estimated pulmonary artery pressure in patients with sickle cell anemia.American journal of hematology · 2017Trial
- B-type natriuretic peptide and plasma hemoglobin levels following transfusion of shorter-storage versus longer-storage red blood cells: Results from the TOTAL randomized trial.American heart journal · 2017Trial
- Article
- Sickle Cell Disease: From Ancient Origins to Modern Breakthroughs in Gene Therapy.Biomedicines · 2026Review
- Retinal imaging and supervised learning predict hospitalizations and kidney and heart-lung damage in sickle cell disease.Blood advances · 2026Observational
- Circulating erythropoietin concentration associates with thromboembolism in sickle cell disease.British journal of haematology · 2026Article
- Article
- Health-related quality of life among patients with sickle cell disease in the hereditary blood disease center in Saudi Arabia.Medicine · 2026Article
- Prevalence, patterns and factors associated with spirometry abnormalities among children and adolescents with sickle cell disease in northern and eastern Uganda: a cross-sectional study.Frontiers in pediatrics · 2026Article
24 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
23 authors at 11 institutions in 2 countries.
Funding
Abstract
backgroundThe role of pulmonary hypertension as a cause of mortality in sickle cell disease (SCD) is controversial. METHODS AND
resultsWe evaluated the relationship between an elevated estimated pulmonary artery systolic pressure and mortality in patients with SCD. We followed patients from the walk-PHaSST screening cohort for a median of 29 months. A tricuspid regurgitation velocity (TRV)≥ 3.0 m/s cuttof, which has a 67-75% positive predictive value for mean pulmonary artery pressure ≥ 25 mm Hg was used. Among 572 subjects, 11.2% had TRV ≥ 3.0 m/sec. Among 582 with a measured NT-proBNP, 24.1% had values ≥ 160 pg/mL. Of 22 deaths during follow-up, 50% had a TRV ≥ 3.0 m/sec. At 24 months the cumulative survival was 83% with TRV ≥ 3.0 m/sec and 98% with TRV < 3.0 m/sec (p < 0.0001). The hazard ratios for death were 11.1 (95% CI 4.1-30.1; p < 0.0001) for TRV ≥ 3.0 m/sec, 4.6 (1.8-11.3; p = 0.001) for NT-proBNP ≥ 160 pg/mL, and 14.9 (5.5-39.9; p < 0.0001) for both TRV ≥ 3.0 m/sec and NT-proBNP ≥ 160 pg/mL. Age > 47 years, male gender, chronic transfusions, WHO class III-IV, increased hemolytic markers, ferritin and creatinine were also associated with increased risk of death.
conclusionsA TRV ≥ 3.0 m/sec occurs in approximately 10% of individuals and has the highest risk for death of any measured variable. The study is registered in ClinicalTrials.gov with identifier: NCT00492531.
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.