Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease

Vandana Sachdev, Roberto F. Machado, Yukitaka Shizukuda, Yesoda N. Rao, Stanislav Sidenko, Inez Ernst, Marilyn St. Peter, Wynona A. Coles, Douglas R. Rosing, William C. Blackwelder, Oswaldo Castro, Gregory J. Kato, Mark T. Gladwin

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)472-479
Number of pages8
JournalJournal of the American College of Cardiology
Volume49
Issue number4
DOIs
StatePublished - Jan 30 2007
Externally publishedYes

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Sickle Cell Anemia
Pulmonary Hypertension
Tricuspid Valve Insufficiency
Mortality
Odds Ratio
Confidence Intervals
Population
Left Ventricular Function
Pulmonary Artery
Blood Pressure

ASJC Scopus subject areas

  • Nursing(all)

Cite this

Sachdev, V., Machado, R. F., Shizukuda, Y., Rao, Y. N., Sidenko, S., Ernst, I., ... Gladwin, M. T. (2007). Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease. Journal of the American College of Cardiology, 49(4), 472-479. https://doi.org/10.1016/j.jacc.2006.09.038

Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease. / Sachdev, Vandana; Machado, Roberto F.; Shizukuda, Yukitaka; Rao, Yesoda N.; Sidenko, Stanislav; Ernst, Inez; St. Peter, Marilyn; Coles, Wynona A.; Rosing, Douglas R.; Blackwelder, William C.; Castro, Oswaldo; Kato, Gregory J.; Gladwin, Mark T.

In: Journal of the American College of Cardiology, Vol. 49, No. 4, 30.01.2007, p. 472-479.

Research output: Contribution to journalArticle

Sachdev, V, Machado, RF, Shizukuda, Y, Rao, YN, Sidenko, S, Ernst, I, St. Peter, M, Coles, WA, Rosing, DR, Blackwelder, WC, Castro, O, Kato, GJ & Gladwin, MT 2007, 'Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease', Journal of the American College of Cardiology, vol. 49, no. 4, pp. 472-479. https://doi.org/10.1016/j.jacc.2006.09.038
Sachdev, Vandana ; Machado, Roberto F. ; Shizukuda, Yukitaka ; Rao, Yesoda N. ; Sidenko, Stanislav ; Ernst, Inez ; St. Peter, Marilyn ; Coles, Wynona A. ; Rosing, Douglas R. ; Blackwelder, William C. ; Castro, Oswaldo ; Kato, Gregory J. ; Gladwin, Mark T. / Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease. In: Journal of the American College of Cardiology. 2007 ; Vol. 49, No. 4. pp. 472-479.
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abstract = "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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AU - Sachdev, Vandana

AU - Machado, Roberto F.

AU - Shizukuda, Yukitaka

AU - Rao, Yesoda N.

AU - Sidenko, Stanislav

AU - Ernst, Inez

AU - St. Peter, Marilyn

AU - Coles, Wynona A.

AU - Rosing, Douglas R.

AU - Blackwelder, William C.

AU - Castro, Oswaldo

AU - Kato, Gregory J.

AU - Gladwin, Mark T.

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N2 - 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.

AB - 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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