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

Authors

Vandana Sachdev, National Heart, Lung, and Blood Institute (NHLBI)
Roberto F. Machado, National Heart, Lung, and Blood Institute (NHLBI)
Yukitaka Shizukuda, National Heart, Lung, and Blood Institute (NHLBI)
Yesoda N. Rao, National Heart, Lung, and Blood Institute (NHLBI)
Stanislav Sidenko, National Heart, Lung, and Blood Institute (NHLBI)
Inez Ernst, National Heart, Lung, and Blood Institute (NHLBI)
Marilyn St. Peter, National Heart, Lung, and Blood Institute (NHLBI)
Wynona A. Coles, National Heart, Lung, and Blood Institute (NHLBI)
Douglas R. Rosing, National Heart, Lung, and Blood Institute (NHLBI)
William C. Blackwelder, National Institutes of Health (NIH)
Oswaldo Castro, The Center For Sickle Cell Disease
Gregory J. Kato, National Heart, Lung, and Blood Institute (NHLBI)
Mark T. Gladwin, National Heart, Lung, and Blood Institute (NHLBI)

Document Type

Article

Publication Date

1-30-2007

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. © 2007 American College of Cardiology Foundation.

Recommended Citation

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.; and Gladwin, Mark T., "Diastolic Dysfunction Is an Independent Risk Factor for Death in Patients With Sickle Cell Disease" (2007). The Center For Sickle Cell Disease Faculty Publications. 165.
https://dh.howard.edu/sicklecell_fac/165