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

ABSTRACT: BACKGROUND: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. OBJECTIVE:To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA. METHODS AND RESULTS: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 associated with %predicted VO2 (P = 0.02). All participants had elevated ECV but the degree of elevation was not associated with exercise parameters. CONCLUSION:Left ventricular DD is associated with decreased exercise capacity in SCA. Interventions to prevent or delay DD could improve exercise capacity, quality of life, and long-term outcomes in SCA.

SUBMITTER: Alsaied T

PROVIDER: S-EPMC6019177 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature

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

Alsaied Tarek T Niss Omar O Powell Adam W AW Fleck Robert J RJ Cnota James F JF Chin Clifford C Malik Punam P Malik Punam P Quinn Charles T CT Taylor Michael D MD

Pediatric blood & cancer 20180521 8

<h4>Background</h4>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.<h4>Objective</h4>To study the effects of DD and ECV on cardiopulmonary exercise test (CPET) in patients with SCA.<h4>Methods and results</h4>As part of a prospective study to character ...[more]

PMID: 29781568

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Project description:BACKGROUND:Noninvasively assessed pulmonary pressure elevations and left ventricular (LV) diastolic dysfunction are associated with increased mortality in adults with sickle cell disease, but their relationship to exercise intolerance has not been evaluated prospectively. METHODS AND RESULTS:Echocardiography, 6-minute walk distance, hemolytic rate, and serum concentrations of ferritin and erythropoietin were evaluated in a cohort of 483 subjects with homozygous hemoglobin S in the U.S. and U.K. Walk-Treatment of Pulmonary Hypertension and Sickle Cell Disease with Sildenafil Therapy (Walk-PHaSST) study. Tricuspid regurgitation velocity, which reflects systolic pulmonary artery pressure, was 2.7 to <3.0 m/s (mean±SD, 2.8±0.1) in 26% of the subjects and ?3.0 m/s (mean±SD, 3.4±0.4) in 11%. The LV lateral E/e' ratio, which has been shown to reflect LV filling pressure in other conditions but has not been studied in sickle cell disease, was significantly higher in the groups with tricuspid regurgitation velocity ?2.7 m/s. Increased hemolysis (P<0.0001), LV lateral E/e' ratio (P=0.0001), blood urea nitrogen (P=0.0002), and erythropoietin (P=0.002) were independently associated with an increased tricuspid regurgitation velocity. Furthermore, female sex (P<0.0001), older age (P<0.0001), LV lateral E/e' ratio (P=0.014), and tricuspid regurgitation velocity (P=0.019) were independent predictors of a shorter 6-minute walk distance. CONCLUSIONS:Echocardiography-estimated elevated pulmonary artery systolic pressure and LV lateral E/e' ratio were independently associated with poor exercise capacity in a large cohort of patients with sickle cell anemia. Controlled trials investigating whether strategies to prevent or delay pulmonary hypertension and/or LV diastolic dysfunction will improve exercise capacity and long-term outcomes in sickle cell anemia should be considered. Clinical Trial Registration- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00492531.

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

Publications

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

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