Cardiac function in sickle cell anemia

Denenberg, Barry; Criner, Gerard J.; Jones, Richard A.; Spann, James F.

doi:10.1016/0002-9149(83)90208-4

Cited by 84 publications

(48 citation statements)

References 26 publications

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“…Traditional indexes of systolic and diastolic function are not adequate to analyse ventricular performance in these patients (Denenberg et al, 1983). It has been demonstrated that TDI can unmask both systolic and diastolic dysfunction earlier than other echocardiographic modalities in a variety of diseases (Mori et al, 2000;Border et al, 2003;McMahon et al, 2004).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in sickle cell disease children under 10 years of age

et al. 2010

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SummaryDespite the finding of elevated Tricuspid Regurgitant Velocity (TRV) in children below 5 years of age, the prevalence and evolution of Pulmonary Hypertension (PH) in young children with sickle cell disease (SCD) are unclear. In order to identify predictive factors of precocious PH development, SCD children ‡3 years old, at steady state, underwent annual echocardiography and Tissue Doppler Imaging (TDI). Patients receiving chronic transfusion were excluded. Thirty-seven of seventy-five patients were ‡3 years, with measurable TRV. In our young population (mean age 6AE2 years) of mainly African, HbS/HbS patients, 8/37 (21AE6%) had TRV ‡2AE5 m/s, 8% being only 3 years old. Significant correlation was found between precocious TRV elevation and high platelet and reticulocyte counts and frequent acute chest syndromes (ACS). In multivariate analysis, ACS was the only variable predicting TRV ‡2AE5 m/s. TDI of the 37 patients showed signs of diastolic dysfunction of the left ventricle. At follow-up all eight patients with high TRV displayed further increase and seven more developed TRV ‡2AE5 m/s. PH seems to begin in children earlier than expected. Factors involved in its early onset might be different from the ones causing its development in older children or adults. African children might benefit from early screening and re-assessment once a year.

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Section: Discussionmentioning

confidence: 99%

Pulmonary hypertension in sickle cell disease children under 10 years of age

et al. 2010

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“…Although no comparable microcirculatory measurements exist for sickle cell disease patients, several studies have demonstrated that low blood pressure in these patients is associated with almost 50% reduction in the peripheral resistance (12,39). The reduced peripheral resistance in sickle cell disease patients is also associated with a significant increase in Q in large arteries (16,20).…”

Section: Figurementioning

confidence: 99%

Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice

Kaul¹,

Liu²,

Chang³

et al. 2004

J. Clin. Invest.

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In sickle cell disease, intravascular sickling and attendant flow abnormalities underlie the chronic inflammation and vascular endothelial abnormalities. However, the relationship between sickling and vascular tone is not well understood. We hypothesized that sickling-induced vaso-occlusive events and attendant oxidative stress will affect microvascular regulatory mechanisms. In the present studies, we have examined whether microvascular abnormalities expressed in sickle transgenic-knockout Berkeley (BERK) mice (which express exclusively human α-and β S -globins with <1% γ-globin levels) are amenable to correction with increased levels of antisickling fetal hemoglobin (HbF). In BERK mice, sickling, increased oxidative stress, and hemolytic anemia are accompanied by vasodilation, compensatory increases in eNOS and COX-2, and attenuated vascular responses to NO-mediated vasoactive stimuli and norepinephrine. The hypotension and vasodilation (required for adequate oxygen delivery in the face of chronic anemia) are mediated by non-NO vasodilators (i.e., prostacyclin) as evidenced by induction of COX-2. In BERK mice, the resistance to NO-mediated vasodilators is associated with increased oxidative stress and hemolytic rate, and in BERK + γ mice (expressing 20% HbF), an improved response to these stimuli is associated with reduced oxidative stress and hemolytic rate. Furthermore, BERK + γ mice show normalization of vessel diameters, and eNOS and COX-2 expression. These results demonstrate a strong relationship between sickling and microvascular function in sickle cell disease.

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“…The method validated by Denenberg et al 17 was used to assess the left ventricular function, so as to minimize the influence of the loading conditions imposed to the heart, which are altered in SCA; this method consists in the ratio between left ventricular end-systolic wall stress (LVESS) and the left ventricular end-systolic volume index (iESV). LVESS was estimated by a noninvasive method, according to Reichek et al's formula 18 .…”

Section: Introductionmentioning

confidence: 99%