Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease
Open accessAbstract
Objectives The goal of this study was to characterize left ventricular diastolic function in the sickle cell disease (SCD) population and to relate echocardiographic measures of dysfunction with pulmonary hypertension and mortality.Background Pulmonary hypertension has been identified as a predictor of death in the adult SCD population. Although diastolic dysfunction is also observed in this population, its prevalence, association with high pulmonary artery systolic pressure, and attributable mortality remain unknown.Methods Diastolic function assessment using tissue Doppler imaging was performed in a group of 141 SCD patients. Conventional echocardiographic parameters of diastolic function were performed in a total of 235 SCD patients.Results Diastolic dysfunction was present in 18% of patients. A combination of diastolic dysfunction and pulmonary hypertension was present in 11% of patients, and diastolic dysfunction accounted for only 10% to 20% of the variability in tricuspid regurgitation (TR) jet velocity. Diastolic dysfunction, as reflected by a low E/A ratio, was associated with mortality with a risk ratio of 3.5 (95% confidence interval 1.5 to 8.4, p < 0.001), even after adjustment for tricuspid regurgitation (TR) jet velocity. The presence of both diastolic dysfunction and pulmonary hypertension conferred a risk ratio for death of 12.0 (95% confidence interval 3.8 to 38.1, p < 0.001).Conclusions Diastolic dysfunction and pulmonary hypertension each contribute independently to prospective mortality in patients with SCD. Patients with both risk factors have an extremely poor prognosis. These data support the implementation of echocardiographic screening of adult patients with SCD to identify high-risk individuals for further evaluation.
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Development of pulmonary hypertension is related to pulmonary vascular disease in the setting of intravascular hemolysis and thrombotic events, but can also occur secondary to diastolic left heart disease.
“While the development of pulmonary hypertension is related to pulmonary vascular disease in the setting of intravascular hemolysis and thrombotic events, it can also occur secondary to diastolic left heart disease (77, 82).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [82]
The introductory passage (page not provided) links pulmonary hypertension in sickle cell disease to vasculopathy arising in the setting of hemolytic anemia and recurrent vaso-occlusion. The Conclusions (page not provided) say diastolic dysfunction directly contributes to elevated pulmonary pressures in a small fraction of patients. The paper does not specifically identify thrombotic events as a cause, and it limits the diastolic contribution rather than presenting it as a general mechanism. (LLM, conf=0.84)
Experimental — accuracy not yet measuredMeasures of diastolic dysfunction obtained by cardiac echocardiography are associated with excess mortality, even after adjustment for tricuspid regurgitant jet velocity, with a risk ratio of 3.5.
“Measures of diastolic dysfunction obtained by cardiac echocardiography are associated with excess mortality, even after adjustment for tricuspid regurgitant jet velocity, with a risk ratio of 3.5 (82).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [82]
The presence of both diastolic dysfunction and an elevated tricuspid regurgitant jet velocity is associated with a risk ratio for death of 12.0.
“the presence of both diastolic dysfunction and an elevated tricuspid regurgitant jet velocity is associated with a risk ratio for death of 12.0 (82).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [82]
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- Authors
- Sachdev, V., Machado, R. F., Shizukuda, Y., Rao, Y. N., Sidenko, S., Ernst, I., St. Peter, M., Coles, W. A., Rosing, D. R., Blackwelder, W. C., Castro, O., Kato, G. J., & Gladwin, M. T.
- Affiliations
- National Institutes of Health (US)
- National Heart, Lung, and Blood Institute (US)
- Howard University (US)
- Corresponding author
- Sachdev, Vandana
- ORCID
- Venue
- Journal of the American College of Cardiology
- Publisher
- Elsevier BV
- Published
- 2007-01
- Volume
- 49
- Issue
- 4
- Pages
- 472-479
- ISSN
- 0735-1097 (ISSN-L)
- PMC
- PMC2082057
- OpenAlex
- W2047558252
- Cited by
- 323
- References
- 43
- Citations by year
- 2026: 15 · 2025: 14 · 2024: 15 · 2023: 11 · 2022: 19 · 2021: 21 · 2020: 15 · 2019: 15 · 2018: 11 · 2017: 14 · 2016: 28 · 2015: 14 · 2014: 18 · 2013: 12 · 2012: 16
- FWCI
- 11.24
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Journal of the American College of Cardiology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Digital Howard (Howard University) (submittedVersion)
- Funders
- National Institutes of Health (Z99 HL999999)
- National Heart, Lung, and Blood Institute (ZIAHL006012)
- Topics
- Health Sciences › Medicine › Genetics › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiovascular Function and Risk Factors
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- sickle cell disease, diastolic dysfunction, pulmonary hypertension, mortality, left ventricular diastolic function, tricuspid regurgitation jet velocity, E/A ratio, tissue Doppler imaging, echocardiographic screening, cardiovascular risk factors
- MeSH terms
- Adolescent, Adult, Aged, Anemia, Sickle Cell, Diastole, Female, Heart Ventricles, Humans, Hypertension, Pulmonary, Male, Middle Aged, Risk Factors, Echocardiography, Doppler, Ventricular Function, Left
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