CLINICAL RESEARCH: DIASTOLIC DYSFUNCTION
Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease
Vandana Sachdev, MD*,*,
Roberto F. Machado, MD ,
Yukitaka Shizukuda, MD, PhD*,
Yesoda N. Rao, MD*,
Stanislav Sidenko*,
Inez Ernst, RN, RDCS*,
Marilyn St. Peter, RDCS*,
Wynona A. Coles, RRT,
Douglas R. Rosing, MD*,
William C. Blackwelder, PhD ,
Oswaldo Castro, MD ,
Gregory J. Kato, MD and
Mark T. Gladwin, MD,,1
* Cardiovascular Branch, Echocardiography Laboratory, National Heart, Lung, and Blood Institute, Bethesda, Maryland
Vascular Medicine Branch, Echocardiography Laboratory, National Heart, Lung, and Blood Institute, Bethesda, Maryland
Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, Maryland
Center for Sickle Cell Disease, Howard University College of Medicine, Washington, DC.
Manuscript received May 8, 2006;
revised manuscript received September 12, 2006,
accepted September 28, 2006.
* Reprint requests and correspondence: Dr. Vandana Sachdev, Echocardiography Laboratory, Cardiovascular Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, 10 Center Drive, CRC, 5-1436, Bethesda, Maryland 20892-1454. (Email: sachdevv{at}nhlbi.nih.gov).
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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Abbreviations and Acronyms
| | BP = blood pressure | | LV = left ventricle/ventricular | | LVH = left ventricular hypertrophy | | PH = pulmonary hypertension | | RV = right ventricle/ventricular | | RWT = relative wall thickness | | SCD = sickle cell disease | | TR = tricuspid regurgitant/regurgitation |
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|
|
|
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|
|
|
|
|
|
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|
|
|
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|
|
|
|
|
|
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|
|
|
|
|
|
|
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|
|
|
|
|
|
|
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June 1, 2010;
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|
|
|
|
|
|
|
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|
|
|
|
|
|
|
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300(22):
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|
|
|
|
|
|
|
P. P. Landburg, T. Teerlink, E. J. van Beers, F. A.J. Muskiet, M. C. Kappers-Klunne, J. W.J. van Esser, M. R. Mac Gillavry, B. J. Biemond, D. P.M. Brandjes, A. J. Duits, et al.
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93(9):
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|
|
|
|
|
|
|
C. F. Barnett, P. Y. Hsue, and R. F. Machado
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January 23, 2008;
299(3):
324 - 331.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. R. Morris, J. H. Suh, W. Hagar, S. Larkin, D. A. Bland, M. H. Steinberg, E. P. Vichinsky, M. Shigenaga, B. Ames, F. A. Kuypers, et al.
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January 1, 2008;
111(1):
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|
|
|
|
|
|
|
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50(4):
378 - 378.
[Full Text]
[PDF]
|
|
|
|
|
|
|
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Reply
J. Am. Coll. Cardiol.,
July 24, 2007;
50(4):
378 - 379.
[Full Text]
[PDF]
|
|
|
|
|
|
|
A. Anthi, R. F. Machado, M. L. Jison, A. M. Taveira-DaSilva, L. J. Rubin, L. Hunter, C. J. Hunter, W. Coles, J. Nichols, N. A. Avila, et al.
Hemodynamic and Functional Assessment of Patients with Sickle Cell Disease and Pulmonary Hypertension
Am. J. Respir. Crit. Care Med.,
June 15, 2007;
175(12):
1272 - 1279.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Voskaridou, G. Tsetsos, A. Tsoutsias, E. Spyropoulou, D. Christoulas, and E. Terpos
Pulmonary hypertension in patients with sickle cell/{beta} thalassemia: incidence and correlation with serum N-terminal pro-brain natriuretic peptide concentrations
Haematologica,
June 1, 2007;
92(6):
738 - 743.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
M. J. Telen
Role of Adhesion Molecules and Vascular Endothelium in the Pathogenesis of Sickle Cell Disease
Hematology,
January 1, 2007;
2007(1):
84 - 90.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
