ArticleAmerican journal of respiratory and critical care medicine2013
Hemodynamic predictors of mortality in adults with sickle cell disease.
Article in American journal of respiratory and critical care medicine, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 67 papers, 3 of them syntheses that pooled it.
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Who cites it
67 citing papers in PubMed, 3 syntheses or guidelines 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
- 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
- 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
- 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
- Elevated transpulmonary gradient and cardiac magnetic resonance-derived right ventricular remodeling predict poor outcomes in sickle cell disease.Haematologica · 2016Trial
- Risk factors for death in 632 patients with sickle cell disease in the United States and United Kingdom.PloS one · 2014Trial
- The Mirage of Individualized Therapy in the United States: Barriers in Pulmonary Hypertension Due to Sickle Cell Disease and Sarcoidosis.American journal of respiratory and critical care medicine · 2025Article
- Ten-Year Longitudinal Study Clarifies Impact of Hemolysis and Pulmonary Hypertension on Patients with Sickle Cell Anemia.American journal of respiratory and critical care medicine · 2025Article
- 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
- Pathophysiological Associations and Measurement Techniques of Red Blood Cell Deformability.Biosensors · 2025Review
- Correlation of Pulmonary Arterial Hypertension with Laboratory Parameters in Sickle Cell Disease- A Retrospective Study.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2025Article
- Different cardiac phenotypes of sickle cell disease revealed using cardiac magnetic resonance imaging.The British journal of cardiology · 2025Article
- Mortality in adults with sickle cell disease: Results from the sickle cell disease implementation consortium (SCDIC) registry.American journal of hematology · 2024Article
- Pulmonary Hypertension in Sickle Cell Disease: Novel Findings of Gene Polymorphisms Related to Pathophysiology.International journal of molecular sciences · 2024Review
- Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study.Frontiers in pediatrics · 2023Article
- The Cardiopulmonary Complications of Sickle Cell Disease.Hematology/oncology clinics of North America · 2022Review
- Long-Term Health Effects of Curative Therapies on Heart, Lungs, and Kidneys for Individuals with Sickle Cell Disease Compared to Those with Hematologic Malignancies.Journal of clinical medicine · 2022Review
- Sickle cell disease related chronic thromboembolic pulmonary hypertension: challenging clinical scenario.Journal of thrombosis and thrombolysis · 2022Article
7 more citing papers are in PubMed but not listed here.
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Authors and funding
15 authors.
Funding
Abstract
backgroundPulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH.
objectivesTo identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization.
methodsNine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression. MEASUREMENTS AND MAIN
resultsSpecific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10 mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19; 95% CI, 1.23-3.89 per 10 mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14-2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality.
conclusionsMortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.
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