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.
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Figure 1 Prevalence of Diastolic Dysfunction in Sickle Cell Disease
(A) Distribution of patients with pulmonary arterial hypertension (PH) and diastolic dysfunction (DD). Venn diagram indicating the number of patients without PH (tricuspid regurgitation [TR] <2.5 m/s), with PH (TR  2.5) and tissue Doppler results indicating DD. (B) Detailed distribution by degree of PH and DD. The number of patients without or with mild, moderate, or severe DD are shown in groups based on their TR jet velocity (TR <2.5 m/s, TR 2.5 to 2.9 m/s, and TR 3 m/s). In the 2 high TR velocity groups, there were no patients with severe DD.
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Figure 2 Kaplan-Meier Survival Curve According to Both TR Jet Velocity and E/A Ratio
Patients were classified as low risk if they had a tricuspid regurgitation (TR) jet velocity of <2.5 m/s and an E/A ratio of 1.0. The high-risk group of patients had either a TR velocity of 2.5 m/s or an E/A ratio of <1.0 or both. Mortality was significantly increased in the group having one or both risk factors (p < 0.0001).
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