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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Red cell volume reduction corrected the platelet aggregation abnormality and bleeding tendency in four boys, aged 5 to 16 years, with severe cyanotic congenital heart disease and polycythemia. Red cell volume was reduced by replacing 15 to 20 ml/kg body weight of the patient's blood with plasma in 50 ml increments over a 1 to 2 hour period. Within 3 days, platelet aggregation was restored essentially to normal, and previous bleeding symptoms disappeared. Platelet aggregation remained normal during 3 weeks of follow-up study and hematocrit values remained at palliative levels. The procedure was safe and simple, and it had beneficial effects not only on bleeding but also on other symptoms related to polycythemia. These preliminary observations suggest that red cell volume reduction may be useful preoperatively in patients with cyanotic heart disease and defects in platelet function to lessen the risk of serious bleeding during the early postoperative period. Palliation in severely cyanotic children whose condition is inoperable is another possible indication for this procedure.
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PMID:Correction of platelet dysfunction and bleeding in cyanotic congenital heart disease by simple red cell volume reduction. 4 35

The results of treatment of iron deficiency anemia in 217 children and infants (aged 8 months to 12 years) with ferrosulfate serin complex are demonstrated: 208 children with simple iron deficiency anemia with hemoglobin 9.1 g/100 ml before treatment had in the first 4 weeks a daily increase of 0.082 g/10000 ml hemoglobin; in 3 children with severe anemia of chronic blood loss the daily increase was 0.21 mg/100 ml and in 6 children with hypoxic polycythemia with congenital heart disease and so called latent iron deficiency anemia the increse was 0.16 g/100 ml. Through the better absorption of this serin-ferrosulfate complex the required dosis is only 3 mg/kg/d. The benefit of this small dosis is the low rate of side effects.
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PMID:[Treatment of iron deficiency anemia with ferrosulfate serin komplex (author's transl)]. 62 90

Gout is rarely noted as a clinical problem in secondary polycythemia-- even if profound polycythemia exists, as in cyanotic congenital heart disease. A retrospective study of 81 patients with congenital heart disease was done to assess the incidence of hyperuricemia. Twenty of 46 patients with cyanotic congenital heart disease had serum levels of uric acid greater than 8 mg/dl. Thirteen of 16 (81%) cyanotic male patients more than 15 years old had serum levels greater than 8 mg/dl. For cyanotic patients, serum levels of uric acid were related directly to the degree of polycythemia (r = .44; P less than .02). Impaired renal function or drug therapy did not seem to account for the hyperuricemia. Because levels of uric acid greater than 10 mg/dl probably are nephropathic, many of these patients may be incurring subclinical uric acid nephropathy.
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PMID:Hyperuricemia in congenital heart disease. 68 9

The isolated left lower lobes of 15 dogs' lungs were perfused by means of a roller pump with blood at hematocrit values ranging from 31 to 80 per cent. Pressure-flow curves were constructed at blood flow rates from one half to three times the normal flow for the left lower lobe at each hematocrit level. The perfusion pressure was normalized with reference to the normal hematocrit(38 to 48 per cent) and normal blood flow for the left lower lobe (20 ml. per kilogram per minute). From these normalized pressure-flow curves, normalized resistance-flow curves were constructed at different mean hematocrit levels. Regression lines were drawn relating normalized pulmonary vascular resistance to hematocrit at different rates of pulmonary blood flow which might be found in patients with congenital heart disease. It was found that pulmonary vascular resistance rose in an exponential fashion as the hematocrit was increased, and that the blood viscosity determined both the shape of the resistance-flow curve and magnitude of the increase in resistance to pulmonary blood flow, especially when the pulmonary blood flow was less than normal and the hematocrit was greater than 54 per cent. The family of regression lines relating pulmonary vascular resistance to hematocrit at different flow rates may be used clinically in patients with congenital heart disease and polycythemia to determine if an elevated pulmonary vascular resistance is due to increased blood viscosity or obstructive pulmonary vascular disease. It is concluded that an increased blood viscosity due to polycythemia significantly alters the pulmonary hemodynamics of patients with congenital heart disease with either increased or decreased pulmonary blood flow. Increased blood viscosity may play an important part in the early initiation and development of pulmonary arteriosclerosis in patients with transposition of the great arteries.
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PMID:The effects of increased blood viscosity on pulmonary vascular resistance. 96 77

Brain abscess is a serious complication of congenital cyanotic heart disease. We retrospectively evaluated the risk factors for brain abscess in 21 such patients treated between 1975 and 1990 in comparison with a control group. The mean arterial oxygen saturation, arterial partial pressure of O2, arterial blood oxygen content, and base excess in patients with brain abscess were significantly lower than in the control patients. The mean arterial partial pressure of CO2, pH, hematocrit, hemoglobin, and red blood cell content in patients with brain abscess were not significantly different. Patients with congenital cyanotic heart disease may develop minute encephalomalacia due to severe hypoxemia and increased blood viscosity resulting from compensatory polycythemia. The increased blood viscosity and reduced blood flow in the microcirculation may induce cerebral thrombosis or exaggerate minute encephalomalacia during dehydration or cardiac dysfunction, and shunted blood containing infectious organisms at such sites may be followed by focal cerebritis.
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PMID:Risk factors for brain abscess in patients with congenital cyanotic heart disease. 138 54

A 62-year-old woman, long suspected of having heart disease, was admitted to our hospital for thorough examination. Her hemoglobin level was 17.7 g/dl and her 2.3-DPG level was 8.90 microM/ml RBC. The patient proved to have polycythemia, hemoglobin Kansas, and diabetes mellitus. To our knowledge, this is the third case of hemoglobin Kansas in the world.
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PMID:Hemoglobin Kansas found in a patient with polycythemia. 145 81

Clinical observation suggested an association between an increased cardiothoracic ratio (CT) and growth retardation (IUGR) in the premature neonate. To investigate this hypothesis, a case-control study was performed. Study subjects included 23 cases (IUGR) and 55 control (appropriate for gestational age [AGA]) premature infants with birthweights 2000 gm or less; Apgar scores greater than 5 at 5 minutes; no congenital heart disease; no polycythemia; no toxoplasmosis, rubella, cytomegalovirus, or herpes infection. In random order, the first chest radiograph of each infant (less than 24 hours) was reviewed by a single radiologist, unaware of the infant's growth status. The CT ratio was computed after measuring the widest internal width of the bony thorax and the cardiac diameter. Mean birthweight (+/- 1 SD) of the IUGR infants was 1161 +/- 289 g and of AGA infants was 1401 +/- 401 g (p less than 0.002); the mean gestational ages (+/- 1 SD) were 33.2 +/- 2.8 and 30.8 +/- 2.5 weeks (p less than 0.001). Mean CT for IUGR infants was 0.57 +/- 0.07 (+/- 1 SD) versus AGA infants, 0.51 +/- 0.04 (+/- 1 SD), p less than 0.001. When the infants were stratified by growth status and CT ratio, 11 of 23 IUGR and 1 of 55 AGA infants had an increased CT ratio p less than 0.0001. When birthweight and gestational age were covaried, growth status remained the best predictor of CT, p = 0.005. There is a strong association of increased CT and growth retardation in premature infants with birthweights 2000 gm or less.
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PMID:Association of increased cardiothoracic ratio and intrauterine growth retardation. 198 63

Cyanosis is frequently encountered in the neonate or infant. Most often it is due to congenital heart disease or primary lung disease. Pulmonary arteriovenous malformation is an unusual cause of cyanosis. Polycythemia and clubbing are associated findings. The treatment is excision or embolization if the disease is not too extensive.
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PMID:Cyanosis due to pulmonary arteriovenous malformation. 305 75

Serum erythropoietin levels were measured by radioimmunoassay in 146 children and young adults with congenital heart disease to assess the relationship between erythropoietin and clinical factors (heart failure, anemia, cyanosis) and hemodynamic variables affecting oxygen delivery and utilization. Erythropoietin values were in the normal range (10 to 30 microU/mL) in 73% (58 of 80) of the patients with and 82% (54 of 66) of those without cyanosis. Elevated erythropoietin values in cyanotic patients were associated with lower mixed venous oxygen saturation and tension than in cyanotic patients with normal erythropoietin levels, even though the degree of polycythemia was similar. In contrast, most of the acyanotic patients who had elevated erythropoietin levels were anemic. Of the blood oxygen measurements, mixed venous oxygen saturation and tension had the closest inverse correlation with erythropoietin values. The normal erythropoietin values in most patients are in accord with other observations that show that an elevation in erythropoietin level in response to hypoxia will be transient if it results in a rise in hemoglobin concentration "appropriate" to the degree of hypoxia. Persistent elevation of erythropoietin in patients with congenital heart disease may indicate harmful impairment of hemoglobin production that is potentially correctable.
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PMID:Serum erythropoietin levels in patients with congenital heart disease. 355 1

A Blalock-Taussig-anastomosis was performed at the age of 2 years in a boy with transposition of the great arteries, ventricular septal defect, and pulmonary atresia. Nine years later he developed a transient aphasia. Cranial computed tomography (CT-scan) revealed a structure compatible with brain abscess. The boy was conscious and no neurological deficit was found. Initial therapy consisted of Ampicillin, Tobramycin, and Metronidazole, 12 days later an acute hemiplegia developed. 30 ml of pus were aspirated from the brain abscess, and the boy's condition dramatically improved. Streptococcus milleri was found bacteriologically and antibiotic therapy was continued over six weeks. Serial CT-scans during and after therapy demonstrated disappearance of the brain abscess. The presented case shows that after a shunt procedure in cyanotic heart disease right-to-left-shunting and therefore chronic oxygen desaturation and polycythemia are still present as facilitating factors for focal encephalomalacia, cerebritis, and brain abscess. In case of short duration of neurological symptoms and a size of abscess less than 4 cm in diameter antibiotic therapy without total excision may eliminate the infection.
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PMID:[Successful treatment of a brain abscess with antibiotics and drainage puncture in an 11-year-old boy with a complex cyanotic heart defect]. 358 39


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