Hyperviscosity in congenital heart disease

Kontras, Stella B.; Bodenberder, Joann G.; Craenen, Jo; Hosier, Don M.

doi:10.1016/s0022-3476(70)80165-2

Cited by 48 publications

(23 citation statements)

References 9 publications

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“…First, the myelosuppressive effect of hydroxyurea can be titrated to sufficiently decrease the red cell count and thus lower the hematocrit, while avoiding toxicity such as significant neutropenia or thrombocytopenia. A lower hematocrit directly reduces the blood viscosity and improves perfusion and oxygen delivery to the tissues [1,3,5,6]. Second, hydroxyurea also induces macrocytosis with increased MCV and MCH while preserving the MCHC.…”

Section: Discussionmentioning

confidence: 99%

“…In response, a physiological increase of erythropoietin causes an increase in erythocyte mass to improve oxygen delivery. The hematocrit can rise to 60-75% or higher, which increases the oxygen carrying capacity because of the higher hemoglobin concentration, but eventually leads to signs and symptoms of hyperviscosity such as headaches, bone pain, respiratory distress, transient ischemic attacks, stroke, and chronic end organ damage [1][2][3]. In addition, hyperviscosity can decrease cardiac output and limit oxygen delivery, which further stimulates erythrocytosis and establishes a vicious cycle.…”

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: 99%

Section: Introductionmentioning

confidence: 99%

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

et al. 2007

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“…Depending on the level of hypoxemia, patients with PAH-CHD develop compensatory erythrocytosis, which when severe can lead to hyperviscosity syndrome, the manifestations of which include headaches, blurry vision, dizziness, paresthesias, and myalgias. [70][71][72][73][74] Patients with Eisenmenger syndrome are generally iron deficient, and iron supplementation (oral or intravenous) is often necessary.…”

Section: Hematologic Manifestationsmentioning

confidence: 99%

Congenital Heart Disease and Pulmonary Hypertension

Gupta

Tonelli

Krasuski

2012

Heart Failure Clinics

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“…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).…”

mentioning

confidence: 99%

“…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).…”

mentioning

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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Hyperviscosity in congenital heart disease

Cited by 48 publications

References 9 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

Congenital Heart Disease and Pulmonary Hypertension

Myocardial perfusion reserve in adults with cyanotic congenital heart disease

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