Increased blood viscosity in patients with cyanotic congenital heart disease and iron deficiency

Linderkamp, Otwin; Klose, Hans; Betke, Klaus; Brodherr-Heberlein, Silke; Bühlmeyer, K; Kelson, Shraga; Sengespeik, Christoph

doi:10.1016/s0022-3476(79)80770-2

Cited by 71 publications

(37 citation statements)

References 7 publications

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“…Importantly, hydroxyurea therapy without diligent correction of iron deficiency may not be efficient. However, in contrast to the previously mentioned reports, [3,9] a recent study did not show an association between iron deficiency and hyperviscosity [34]. The methods to assess viscosity used in each study were different and may contribute to the different results.…”

Section: Discussionmentioning

confidence: 59%

“…Relative rigidity of iron-deficient red cells has been recognized [9] and seen as a clinically significant danger of excessive phlebotomy in this clinical setting [17,18,32]. Even at a hematocrit below 65%, iron-deficient red cells may increase the viscosity dramatically and lead to serious vascular events.…”

Section: Discussionmentioning

confidence: 99%

“…However, this approach can have serious consequences including inadequate oxygen delivery and symptomatic hypoxemia, as well as effects from acute changes in blood volume and viscosity, which may paradoxically increase the risk of stroke or other end organ damage independent of the hematocrit [5,6]. Repeated phlebotomy may result in iron deficiency leading to increased rigidity within red cells, which further increases the risk of symptomatic hyperviscosity [7][8][9][10][11].…”

Section: Introductionmentioning

confidence: 99%

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Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease

et al. 2007

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Secondary erythrocytosis in cyanotic congenital heart disease (CCHD) causes substantial morbidity because of complications of hyperviscosity, including stroke and chronic end organ damage. Phlebotomy provides temporary improvement but leads to iron deficiency and can actually increase blood viscosity. We describe the successful use of hydroxyurea (hydroxycarbamide) in four patients with uncorrected CCHD and symptomatic secondary erythrocytosis. In all patients, hydroxyurea improved symptoms of hyperviscosity. Substantial decreases in the red blood cell (RBC) count were observed, along with increases in the mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH), leading to only modest declines in the circulating hemoglobin concentration. Two patients experienced transient mild myelosuppression, which promptly resolved with dose reduction of hydroxyurea. Hydroyxurea provides a novel and useful therapeutic approach to reduce hyperviscosity from secondary erythrocytosis in patients with CCHD, while preserving oxygen carrying capacity and avoiding iron depletion by phlebotomy. Am. J. Hematol. 82:740-743, 2007. V

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Section: Discussionmentioning

confidence: 59%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease

et al. 2007

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“…Adult patients with CCHD and secondary erythrocytosis who undergo repeated phlebotomies are at risk for developing iron deficiency and microcytic circulating erythrocytes. Such microcytic erythrocytes have been shown to be rigid and resist deformation in high shear rates in the microcirculation, 23 which, in turn, increases the risk of hyperviscosity symptoms and CVAs in adults and children with CCHD and secondary erythrocytosis. 16,17 Therefore, the short-term potential benefit of phlebotomy in lowering hematocrit in patients with CCHD and secondary erythrocytosis may be offset by development of chronic iron deficiency with increased risk of hyperviscosity symptoms and cerebrovascular events.…”

Section: Discussionmentioning

confidence: 99%

Cyanotic Congenital Heart Disease (CCHD) with Symptomatic Erythrocytosis

et al. 2007

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“…Mean hemoglobin was 20.7 g/dl (SD 1.9) (range: 17.8 -23.9 g/dl). All were studied in an iron-replete state, because deficiency results in microspherocytes that increase blood viscosity (22,34).…”

Section: Study Populationmentioning

confidence: 99%

Myocardial perfusion reserve in adults with cyanotic congenital heart disease

Brunken

Perloff

Czernin

et al. 2005

American Journal of Physiology-Heart and Circulatory Physiology

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In patients with cyanotic congenital heart disease (CCHD), a right-to-left shunt results in systemic hypoxemia. Systemic hypoxemia incites a compensatory erythrocytosis, which increases whole blood viscosity. We considered that these changes might adversely influence myocardial perfusion in CCHD patients. Basal [LV, 55 (SD 25) vs. 48 mmHg ⅐ ml Ϫ1 ⅐ g ⅐ min (SD 16); septum, 67 (SD 35) vs. 50 mmHg ⅐ ml Ϫ1 ⅐ g ⅐ min (SD 21); RV, 59 (SD 26) vs. 61 mmHg ⅐ ml Ϫ1 ⅐ g ⅐ min (SD 27), P ϭ not significant]. These findings suggest that adult CCHD patients have remodeling of the coronary circulation to compensate for the rheologic changes attending chronic hypoxemia. heart defects congenital; myocardial perfusion; positron emission tomography; myocardial perfusion reserve IN PATIENTS with cyanotic congenital heart disease (CCHD), infusion of poorly oxygenated blood into the systemic circulation via a right-to-left shunt results in sustained arterial hypoxemia. Arterial hypoxemia accelerates the release of erythropoetin from specialized sensor cells in the renal cortex, which in turn increases the number of circulating red blood cells (erythrocytosis) and the arterial hemoglobin concentration (17,25). This adaptive response helps to sustain tissue oxygen delivery, with equilibrium conditions typically being reached at elevated hematocrit levels (42). Oxygen delivery might also be augmented in the peripheral tissues of CCHD patients by an increase in oxygen extraction (3). However, this mechanism appears to be of limited utility for the coronary microcirculation, because resting oxygen extraction in the healthy human myocardium is already high and approaches 70% under basal conditions (23). This suggests that increases in myocardial oxygen demand need to be accompanied by nearly parallel increases in perfusion to sustain aerobic metabolism. Measurements of myocardial perfusion have previously been made in a small number of CCHD patients during cardiac catheterization, using clearance rates of inert gases or iodine-131 iodoantipyrine (51, 53). These early studies were limited by the methodology used to measure the tracer clearance rates and were further confounded by the need to normalize blood flows to derived estimates of ventricular mass. Moreover, these studies provided information only about global, and not regional, myocardial perfusion.Erythrocytosis increases whole blood viscosity in a nonlinear fashion (30,46). In vivo measurements made with intravital microscopy, for example, indicate that a change in hematocrit from 45% to 65% increases whole blood viscosity by about 60% and nearly doubles microvascular resistance (46). Because prior clinical reports suggest that marked increases in red blood cell mass might impair myocardial perfusion (27,61), it has been proposed that the erythrocytosis in CCHD may be more detrimental than beneficial for some patients. On the other hand, more recent studies indicate that increases in perfusate viscosity are associated with increases in shear stress, which could serve to stimula...

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Increased blood viscosity in patients with cyanotic congenital heart disease and iron deficiency

Cited by 71 publications

References 7 publications

Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease

Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease

Cyanotic Congenital Heart Disease (CCHD) with Symptomatic Erythrocytosis

Myocardial perfusion reserve in adults with cyanotic congenital heart disease

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