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Journal ArticleDOI

Acute Hemodynamic Effects of Red Cell Volume Reduction in Polycythemia of Cyanotic Congenital Heart Disease

01 Aug 1970-Circulation (Lippincott Williams & Wilkins)-Vol. 42, Iss: 2, pp 297-308
TL;DR: The replacement of whole blood with plasma or 5% albumin is shown to result in an increased systemic blood flow and oxygen delivery and Hypervolemia in hypoxic polycythemia should be maintained in order to sustain an adequate SBF.
Abstract: Acute reduction in red cell volume (RCV) without significant alterations of blood volume in 22 patients with severe polycythemia secondary to cyanotic congenital heart disease resulted in a decrease in peripheral vascular resistance and an increase in stroke volume, systemic blood flow (SBF), and systemic oxygen transport. These changes are probably related to the decreased blood viscosity and yield shear stress associated with lower red cell concentrations. Hypervolemia in hypoxic polycythemia should be maintained in order to sustain an adequate SBF. In contrast to acute phlebotomy which may be expected to decrease blood oxygen content and SBF, the replacement of whole blood with plasma or 5% albumin is shown to result in an increased systemic blood flow and oxygen delivery.
Citations
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Journal ArticleDOI
TL;DR: Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis.
Abstract: Aims To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome. Methods The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33·02±12·8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and transplantation, death from all causes and sudden death. Results Eighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Patients with complex heart disease and Eisenmenger reaction had earlier clinical deterioration (18·6±11·3 vs 26·7±12·2) and shorter survival (25·8±7·9 vs 32·5±14·6 years). Thirty-eight (20·2%) patients had at least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13·2%) had pulmonary thromboembolism at a mean age of 35·2±13·4 years. Fifteen (7·9%) had a stroke and 7 (3·7%) a cerebral abscess at a mean age of 31·4±15·7 and 24·1±4·9 years, respectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2·04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23·5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35·8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29·5%), heart failure (22·9%) and from haemoptysis (11·4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis. Conclusions patients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks

502 citations

Journal ArticleDOI
15 Dec 2002-Blood
TL;DR: Polycythemia vera is a clonal disorder arising in a multipotent hematopoietic progenitor cell that causes the accumulation of morphologically normal red cells, white cells, platelets, and their progenitors in the absence of a definable stimulus.

421 citations

Journal ArticleDOI
TL;DR: Since only the red cell lineage is involved, the term erythrocytosis has more validity and will be used throughout this article, and polycythaemia will be retained in relation to the clonal disorder, polycytaemia vera (PV), in which three cell lineages are involved.
Abstract: Traditionally, polycythaemia has been used to identify a group of varied disorders with an increase in circulating red cells that are typified by a persistently raised haematocrit (Hct). Since only the red cell lineage is involved, the term erythrocytosis has more validity and will be used throughout this article. Polycythaemia will be retained in relation to the clonal disorder, polycythaemia vera (PV), in which three cell lineages are involved.

308 citations

Journal ArticleDOI
TL;DR: Patients with the Eisenmenger syndrome often have a history of transient pulmonary congestion in infancy as a result of a substantial pulmonary blood flow caused by a large left-to-right intracardiac shunt, and seek medical attention because of progressive fatigue, dyspnea, or cyanosis in adulthood.
Abstract: For this article, the literature on the pathophysiology, clinical features, natural history, prognosis, and management of the Eisenmenger syndrome in adults was reviewed. English-language articles from 1966 to the present were identified through a search of the MEDLINE database by using the terms Eisenmenger, congenital heart disease, and pulmonary hypertension. Selected cross-referenced articles were also included. Articles on the pathophysiology, clinical presentation, evaluation, natural history, complications, and treatment of the Eisenmenger syndrome in adults were selected, and descriptive and analytical data relevant to the practicing physician were manually extracted. The Eisenmenger syndrome is characterized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic-to-pulmonary circulation connection. Most patients with the syndrome survive for 20 to 30 years. The hemostatic changes associated with the syndrome may lead to thromboembolic events, cerebrovascular complications, or the hyperviscosity syndrome. Erythrocytosis is present in most patients, but excessive phlebotomy may cause microcytosis and exacerbate the symptoms of hyperviscosity. Other complications associated with the Eisenmenger syndrome include hemoptysis, gout, cholelithiasis, hypertrophic osteoarthropathy, and decreased renal function. Pregnancy or noncardiac surgery is associated with a high mortality rate in patients with the Eisenmenger syndrome. Because most pediatric patients with the Eisenmenger syndrome survive to adulthood, primary care physicians should have a thorough understanding of the syndrome; its associated complications; and medical and surgical management, especially with regard to the appropriate timing of phlebotomy and lung or heart-lung transplantation. In addition, patients with the syndrome should undergo routine follow-up at a tertiary care center that has physicians and nurses with special expertise in congenital heart disease. In patients with the Eisenmenger syndrome who are pregnant or require noncardiac surgery, a multidisciplinary approach should be used to reduce the excessive mortality associated with these conditions.

278 citations


Cites background from "Acute Hemodynamic Effects of Red Ce..."

  • ...However, isovolumetric reduction of the hematocrit increases cardiac output and systemic oxygen transport, decreases systemic vascular resistance, and improves symptoms at rest and during exercise (36, 37)....

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Journal ArticleDOI
TL;DR: Observations on 124 cyanotic adults for general physicians, cardiologists, and hematologists who care for these patients focused on regulation of erythrocyte mass, symptoms of deficient tissue oxygen transport, hyperviscosity and iron deficiency, the potential relation between elevated hematocrit levels and brain injury, hemostasis, urate metabolism, and renal function.
Abstract: Hematologic management of adults with cyanotic congenital heart disease has received little recent attention. The lack of practical therapeutic guidelines prompted us to consolidate our observations on 124 cyanotic adults for general physicians, cardiologists, and hematologists who care for these patients. Specific attention focused on regulation of erythrocyte mass and concepts of compensated and decompensated erythrocytosis, symptoms of deficient tissue oxygen transport, hyperviscosity and iron deficiency, the potential relation between elevated hematocrit levels and brain injury, hemostasis, urate metabolism, and renal function. Cerebral infarction was not seen in any patient. Phlebotomy is best reserved for treatment of symptomatic hyperviscosity. Iron therapy is indicated for symptomatic iron deficient erythropoiesis. Abnormal hemostatic mechanisms are the rule. Antithrombotic medications have little or no role in treatment. Hyperuricemia is the result of abnormal renal uric acid excretion not urate overproduction, and serves as a marker of abnormal renal function. Drugs that promote urate excretion are the preferred maintenance treatment in symptomatic hyperuricemic patients.

162 citations

References
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Journal ArticleDOI
TL;DR: The dynamic rheological properties in the creeping flow range are such that the relative viscosity of blood to water is almost independent of temperature, including red cell aggregation promoted by elements in the plasma.

229 citations

Journal ArticleDOI
TL;DR: There was a significant decrease in total peripheral resistance in anemia and a marked rise in polycythemia, and the maximum number of red cells present for oxygen transport to the tissues was near the mean normal hematocrit of 40.
Abstract: Normovolemic anemia and polycythemia were studied in 14 dogs. Cardiac outputs increased with anemia and fell with rises in hematocrit. Although many factors—such as chemical changes—may play an important role in these variations in cardiac output, there was an indication that viscosity alone may have a major effect. There was no significant association between changes in cardiac output and the various pressures—mean arterial, mean right atrial, mean pulmonary and mean circulatory. Although the pressures did not change significantly, there was a significant decrease in total peripheral resistance in anemia and a marked rise in polycythemia. It was also found that the maximum number of red cells present for oxygen transport to the tissues was near the mean normal hematocrit of 40.

222 citations