Diastolic dysfunction is associated with exercise impairment in patients with sickle cell anemia

Pediatric Blood & Cancer
Tarek AlsaiedMichael D Taylor

Abstract

Left ventricular diastolic dysfunction (DD) is an independent risk factor for mortality in sickle cell anemia (SCA) and is associated with increased extracellular volume (ECV) on cardiac MRI (CMR). Exercise impairment is common in SCA, but its causes and prognostic value are not well understood. To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA. As part of a prospective study to characterize the cardiomyopathy of SCA (NCT02410811), 20 children and adults with SCA underwent CMR, echocardiography, and cycle ergometer CPET (age range 8-43 years). Maximum exercise was reached in 18 patients and 17 (94%) had reduced exercise capacity (%predicted VO2 less than 80%). Six patients had DD and none had systolic dysfunction. Patients with DD had lower exercise capacity compared to patients with normal diastolic function (%predicted VO2 48.2 ± 9.1% vs. 61.2 ± 11.7%; P = 0.01). The z-score of left ventricular lateral E/e' ratio, which is a marker of DD, was negatively associated with %predicted VO2 (r = -0.61, P = 0.01). All patients with moderate-to-severe exercise impairment (%predicted VO2  < 60%) had lateral E/e' z-score > 2. In a multivariate analysis, lateral E/e' z-score was independently...Continue Reading

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Related Concepts

Teens
Anemia, Sickle Cell
Diastole
Oxygen Consumption
Exercise, Isometric
Ventricular Dysfunction, Left
Biological Markers
Echocardiography
Extracellular Fluid
Left Ventricular Function

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