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Query: UMLS:C0018801 (heart failure)
 72,216 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Premenopausal women in affluent societies are protected from heart diseases which kill large numbers of men. The basis for this sex difference and the loss of protection with menopause is unknown. The hypothesis offered is that the greater incidence of heart diseases in men and postmenopausal women compared with the incidence in premenopausal women is due to higher levels of stored iron in these two groups. The hypothesis is supported by observations of (1) myocardial failure in iron strong disease, (2) accumulation of stored iron with age in men, and (3) accumulation of stored iron after menopause to levels found in men. In addition, the heart diseases of affluence are rare among impoverished peoples who are often iron deficient. The depletion of iron stores by regular phlebotomy could be the experimental system for testing this hypothesis, and a preventive therapy if the hypothesis is confirmed.
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PMID:Iron and the sex difference in heart disease risk. 2535 74

Severe congestive cardiac failure developed in a few weeks in a 44 year old man who had undergone porto-caval anastamosis for post-hepatitis cirrhosis one year previously and then treated for anaemia by repeated blood transfusion and chronic daily oral iron therapy. Infiltrative, congestive and restrictive cardiomyopathy was diagnosed in the presence of global cardiomegaly, electrocardiographic changes (microvoltage, diffuse ST-T wave changes), echocardiographic appearances (dilatation of the left ventricle, with hypertrophic and hypokinetic walls), and hemodynamic signs of adiastole with equalisation of filling pressures at 15 mmHg and a cardiac index of 1,88 l/min/m2. Cardiac haemochromatosis was confirmed by the laboratory (serum iron: 35 mumol/l; siderophilin saturation: 100 p. 100; serum ferritin: 1854 ng/ml; induced siderouria: 51 mg/24 hours) and histological findings (endomyocardial biopsy showing pigment overload). The absence of a family history, of homozygote A3 antigen, of diabetes, of iron overload on hepatic biopsy one year previously, excluded the diagnosis of familial idiopathic haemochromatosis. A secondary form of the disease was diagnosed on a possible genetic predisposition (heterozygote A3 antigen) and on environmental factors (blood transfusions, iron therapy, cirrhosis, alcoholism and perhaps the porto-caval anastamosis. Cardiac haemochromatosis was cured in this case by iron chelating therapy comprising daily subcutaneous infusions of 2 g of desferrioxamine for 2 months. The cure was confirmed by regression of the signs of clinical cardiac failure and of cardiomegaly, the increase in QRS voltages and the near normalisation of the hemodynamic and laboratory findings.
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PMID:[Adiastole caused by a secondary cardiac hemochromatosis. Successful treatment with an iron chelating agent]. 641 3

Up until recently in clinical practice suspected hemochromatosis with a pathological iron-screening test (plasma iron, percentage transferrin saturation, serum ferritin, desferrioxamine-induced urinary iron excretion) made a liver biopsy necessary. Today, as a first step, the density of the liver parenchyma can be measured by means of computed tomography. Normal findings obviate the need for laparoscopy. Since the late forties weekly or twice weekly phlebotomy has been the sole form of treatment for manifest idiopathic hemochromatosis. In the mid-sixties the hopes placed in chelating substances (desferrioxamine) were not fulfilled, because the plasma half-life (only 7-10 minutes) of this drug was too short. Even with several daily injections only a small amount of iron was removed from the body tissue (10-25 mg daily urinary iron excretion). The introduction of portable infusion pumps in the late seventies offered us a new possibility of administering desferrioxamine by subcutaneous injection (Propper et al., 1976). Until that time such treatment was successfully used only in the field of pediatrics to treat secondary transfusion hemochromatosis in thalassemia. In one case of idiopathic hemochromatosis with severe organic involvement (right heart failure, repeated esophageal hemorrhage and bronzed diabetes) we had to achieve rapid iron elimination, and for this purpose we used continuous long-term desferrioxamine administration by means of a portable infusion pump (Autosyringe) in addition to phlebotomy. Since, particularly in the critical initial phase of treatment when heart failure was always threatening, great care had to be exercised in the use of phlebotomy, iron removal was achieved largely by desferrioxamine administration (daily up to 240 mg iron elimination in urine and stools).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:New diagnostic and therapeutic possibilities in manifest idiopathic hemochromatosis. 651 41

The excessive storage of iron in idiopathic haemochromatosis leads to severe organic lesion up to life-threatening conditions (cardiac insufficiency, portal decompensation). The symptoms melanodermia , diabetes mellitus and other endocrine failures, liver cirrhosis, cardiac insufficiency and arthropathy appear together or in various combinations. The diagnosis is ascertained by the proof of iron storage, the multiple organic affection and by familial accumulation of the various laboratory diagnostic possibilities are particularly to be emphasized the serum iron value together with the percetal transferrin saturation (as search test), serum ferritin, the desferrioxamine test, simple ferrokinetic investigations and the quantitative determination of iron in the liver in the bioptate . For family examinations, apart from the search test, a HLA typisation is reasonable, in order to estimate the risk of the disease (particularly of brothers and sisters). The therapy of choice are blood- lettings (0.5 l once to twice a week) up to obtaining a permanent easy iron deficiency anaemia. The maintenance therapy should be performed with monthly to quarterly blood- lettings . Only in cases exception a desferal treatment is indicated. Endocrine failures and cardiac disturbances need a particular therapy.
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PMID:[Idiopathic hemochromatosis--diagnosis and therapy]. 673 May 91

Serious defects in the living conditions of the vast majority of people in the tropics, rather than racial factors, are the underlying reasons why anaemia is common, why malaria is rampant and why the complications of sickle cell disease are so serious. Mass illiteracy, poor environmental hygiene and widespread poverty with all their implications explain why malaria eradication programmes have so far failed in tropical Africa and why basic health-care schemes have been difficult to establish. Pregnant women are very vulnerable to the effects of anaemia, malaria and sickle cell disease. However, appropriate use of folic acid and iron supplements as well as malarial chemosuppression succeeds in maintaining haemoglobin concentrations at reasonable levels during pregnancy. If, for whatever reason, the haemoglobin level falls to under 4.4 g/dl or the haematocrit value is 0.14 or less, anaemia becomes an obstetric emergency. Both maternal and fetal mortality rise sharply, maternal death being due to anaemic heart failure, fulminating bacterial infection and shock from even small loss at delivery or abortion. With the haemoglobin concentration as low as 4.4 g/dl, blood transfusion greatly improves maternal but not necessarily fetal prognosis. Additional cause of morbidity in sickle cell disease is painful crises, the control of which remains largely unsatisfactory. Now that sickle cell disease can be diagnosed early in intrauterine life the idea of aborting the affected fetuses as a means of controlling or reducing sickle cell disease is well within the means of developed countries, but it is a line of approach which developing countries cannot afford at present.
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PMID:Anaemia, malaria and sickle cell disease. 675 46

A review of nutritional anaemia in Africa is presented above. It has been noted that nutritional anaemia, including iron-deficiency anaemia, megaloblastic anaemia due to folate deficiency or vitamin B12 deficiency, or both, and protein deficiency-anaemia, is widespread throughout Africa. It is particularly common in growing children, women of child-bearing age, pregnant women and lactating mothers. The anaemia is also especially common during the second half of the dry season and the first half of the wet season, when food supplies are limited. In all cases the anaemia is caused either by limited dietary intake, excessive loss of nutrients or excessive utilization. The anaemia is associated with a number of sequelae including both structural changes, like mitochondrial swelling and mucosal atrophy, and functional abnormalities, such as cardiac failure, decreased work output, increased pregnancy risks and increased susceptibility to infections. The evidence in favour of increased susceptibility to infections in megaloblastic anaemia and protein-deficiency anaemia is overwhelming, but in iron-deficiency anaemia the available information argues in favour of reduced susceptibility to infections, except after initiation of iron therapy. The treatment of nutritional anaemia includes replacement of the deficient nutrients (and blood transfusion in severe cases), prevention of further nutrient losses and treatment of associated complications.
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PMID:Nutritional anaemias. Part 1: Tropical Africa. 703 May 54

Alcohol, adriamycin, viruses and excess deposition of iron in the heart are known to induce cardiomyopathy. Altered immune response in the form of defective T cell function, overactive anti-body-producing B lymphocytes and circulating anti-heart antibodies; increased fibrous tissue in the heart, with a tendency to develop mural thrombi, arrhythmias and cardiac failure are some of the important features observed in these cases. Prostaglandins can modify immune response, regulate fibrous tissue and collagen biosynthesis, are believed to be involved in the pathogenesis of cardiac arrhythmias and failure. Ethanol, adriamycin, viruses and iron are known to alter prostaglandin(PG) synthesis. The altered PG function may play a major role in cardiomyopathy.
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PMID:Possible role of prostaglandins in the pathogenesis of cardiomyopathies. 719 84

A new feature has been encountered in review of a large species of autopsy materials of beta-thalassaemia/Hb E disease. Among 43 patients pulmonary arterial obstructive lesions were found in 19 (44%), of which 17 were splenectomised cases. The pulmonary arterial thromboembolism may have been due to circulating platelet aggregates. This newly discovered pathology may be an additional factor contributing toward dyspnoea and heart failure in thalassaemia besides anaemia and cardiac iron deposition. If it is proven that this pulmonary arterial thromboembolism is indeed due to circulating platelet aggregates, preventive measure by administration of drugs reducing platelet aggregation such as aspirin and Persantin may be indicated, especially after splenectomy.
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PMID:Pulmonary artery obstruction in thalassaemia. 722 95

We describe a 31 year old male patient who presented with severe cardiomyopathy caused by primary hemochromatosis. After a stormy course, complicated by heart failure and severe ventricular arrythmias, improvement in clinical status and myocardial function occurred. Depletion of myocardial iron was documented by the technique of serial endomyocardial biopsy. Myocardial iron stores were not yet depleted when hypoferremia and iron deficiency anemia occurred. This is the first reported study of myocardial morphology in a successfully treated patient with hemochromatotic cardiomyopathy.
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PMID:Myocardial involvement in idiopathic hemochromatosis. Morphologic and clinical improvement following venesection. 723 94

Although high blood transfusion regimens have improved the life expectancy of the patient with Thalassemia Major, cardiac failure and arrhythmias remain a cause of early death. It is not certain whether the massive myocardial iron deposition found in such patients is preventable by intensive chelation therapy. This study evaluates endomyocardial biopsy as a method of assessing myocardial iron deposition. Of four patients with clinical and biochemical evidence of severe haemochromatosis, only one had a myocardial iron content comparable to that found in severe haemochromatotic myocardium. The one patient with cardiac failure had an endomyocardial iron content within the normal range. Studies of the iron distribution in haemochromatotic myocardium demonstrate that the subendocardial myocardium contains only half the iron content of the subepicardial layer, and there is a large sampling variation. It is concluded that catheter endomyocardial biopsy is an insensitive method of determining early myocardial deposition because of the location of iron and the variability of the sampling. Studies of the nature of the myocardial iron protein with CM32 cation exchange resin chromatography show that there is a large increase in the haemosiderin: ferritin ratio (5:1) in iron overload myocardium as compared with the normal heart (2:1). Similar results have been observed in the liver with iron overload, where the increase in hepatic haemosiderin was associated with greater lysosomal fragility. It is possible that myocardial cell damage may also occur by the rupture of iron engorged lysosomes.
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PMID:Cardiac involvement in secondary haemochromatosis: a catheter biopsy study and analysis of myocardium. 726 Sep 65


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