Cardiac Output and Oxygen Delivery during Exercise in Sickle Cell Anemia

Pianosi, Paul; D'souza, S. J. A.; Charge, T. D.; Béland, Marie J.; Esseltine, D. W.; Coates, Allan L.

doi:10.1164/ajrccm/143.2.231

Cited by 18 publications

(14 citation statements)

References 4 publications

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“…Most striking in our present analysis is the overwhelming proportion of individuals with demonstrable exercise limitation, as reflected by low maximum achievable work rate and peak VO 2 on cardiopulmonary exercise testing. Others have reported poor exercise capacity in children with SCD [10][11][12][13] although prior studies have inconsistently examined the relationship between ECHO and PFT data and exercise capacity. Breathing reserve was reduced in half of our subjects tested, which in the setting of low peak VO 2 , suggests that exercise limitation in these individuals may in part be explained by pulmonary vascular disease or specifically, intrinsic lung disease with impaired peripheral oxygenation.…”

Section: Discussionmentioning

confidence: 98%

Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease

et al. 2009

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Although cardiopulmonary disease is associated with decreased functional capacity among adults with sickle cell disease (SCD), its impact on functional capacity in children with SCD is unknown. We evaluated 6-min walk (6MW) distance in 77 children and young adults with SCD undergoing screening for cardiopulmonary disease. Of 30 subjects who also underwent cardiopulmonary exercise testing, we found evidence for decreased exercise capacity in a significant proportion. Exercise capacity was related to baseline degree of anemia and was significantly lower in subjects with a history of recurrent acute chest syndrome. We found that 6MW distance adjusted for weight and body surface area was shorter in subjects with restrictive lung disease but that only 6MW adjusted for weight remained significantly shorter when we controlled for baseline hemoglobin. Exercise capacity was not significantly different in subjects with and without cardiopulmonary disease. We conclude that restrictive lung disease is associated with shorter 6MW distances in children and young adults with SCD, but that variables associated with decreased exercise capacity, other than anemia, remain unclear. Our study underscores the importance of further delineating the direct pathophysiologic processes that contribute to decreased exercise capacity observed among individuals with SCD and cardiopulmonary disease. Am. J. Hematol. 84:645-649, 2009. V

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Section: Discussionmentioning

confidence: 98%

Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease

et al. 2009

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“…Anemia has been consistently suggested as a likely contributor to decreased VO 2peak among adults and children with SCA [4,5,7,34,35]. In prior evaluations of maximal aerobic capacity, children with SCA had increased pulmonary blood flow and decreased maximal arteriovenous oxygen difference compared to healthy children, both of which were attributed to anemia [4,36]. In several cross-sectional studies, anemia has been associated with decreased VO 2peak as well as weight-adjusted 6 min walk time in children with SCA undergoing cardiopulmonary testing [5,7].…”

Section: Discussionmentioning

confidence: 99%

Longitudinal differences in aerobic capacity between children with sickle cell anemia and matched controls

Watson

Liem

et al. 2014

Pediatr Blood Cancer

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Background The purpose of this study was to compare longitudinal trajectories of maximal aerobic capacity in children with sickle cell anemia (SCA) and matched healthy controls, and explore whether these trajectories were associated with selected physiologic variables. Procedures Children with SCA (n=33) and healthy controls (n=30) matched at baseline for race, sex, Tanner stage, height, and weight completed three consecutive annual fitness assessments (VO2peak). Data were compared between the groups at each time point and within groups over time. Change in VO2peak between the two groups over time was assessed using a linear mixed model with age, sex, fat-free mass (FFM), Tanner stage, and hemoglobin (Hgb) concentration as covariates. Results At baseline, children with SCA had significantly lower Hgb concentration (8.9 vs. 13.7 g/dL, p<0.001) and relative VO2peak (24.2 vs. 27.9 ml/kg/min, p=0.006) than healthy controls. Over time, children with SCA had smaller increases than healthy controls in VO2peak (−0.1 and +4.9 ml/kg/min, p<0.001), Tanner stage at year 2 (15% and 66% Tanner 4, p<0.001), and FFM (+4.0 and +6.8 kg, p=0.02). Changes in Hgb concentration did not differ between groups (+0.03 and +0.09 g/dL, p=1.0). After adjusting for age, sex, Tanner stage, FFM, and Hgb concentration the differences in change in VO2peak over time remained significant (p<0.001). Conclusion Children with SCA demonstrate lower relative VO2peak compared to healthy children and the difference increases over time. The difference in VO2peak trajectories between the two groups during puberty remains significant after adjusting for age, sex, FFM, Tanner stage, and Hgb concentration.

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“…Cardiomegaly is related to a high output secondary to anemia, hyperemia, and hypervolemia51 and is more common in stroke patients 52, 53. Relative hypertension is associated with an increased risk of stroke and mortality 54, 55…”

Section: Risk Factors For Strokementioning

confidence: 99%

Sickle cell disease: The neurological complications

Prengler

Pavlakis

Prohovnik

et al. 2002

Annals of Neurology

155

112

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The genetic cause of sickle cell disease has been known for decades, yet the reasons for its clinical variability are not fully understood. The neurological complications result from one point mutation that causes vasculopathy of both large and small vessels. Anemia and the resultant cerebral hyperemia produce conditions of hemodynamic insufficiency. Sickled cells adhere to the endothelium, contributing to a cascade of activated inflammatory cells and clotting factors, which result in a nidus for thrombus formation. Because the cerebrovascular reserve becomes exhausted, the capacity for compensatory cerebral mechanisms is severely limited. There is evidence of small-vessel sludging, and a relative deficiency of nitric oxide in these vessels further reduces compensatory vasodilatation. Both clinical strokes and silent infarcts occur, affecting motor and cognitive function. New data suggest that, in addition to sickle cell disease, other factors, both environmental (eg, hypoxia and inflammation) and genetic (eg, mutations resulting in thrombogenesis), may contribute to a patient's stroke risk. The stroke risk is polygenic, and sickle cell disease can be considered a model for all cerebrovascular disease. This complex disease underscores the potential intellectual and practical distance between the determination of molecular genetics and effective clinical application and therapeutics.

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Cardiac Output and Oxygen Delivery during Exercise in Sickle Cell Anemia

Cited by 18 publications

References 4 publications

Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease

Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease

Longitudinal differences in aerobic capacity between children with sickle cell anemia and matched controls

Sickle cell disease: The neurological complications

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