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Acute effects of phlebotomy on right ventricular size and performance in polycythemic patients with chronic obstructive pulmonary disease.

To assess the acute effects of phlebotomy on right ventricular size and performance, gated equilibrium radionuclide angiography was used to evaluate 10 patients with severe chronic obstructive pulmonary disease and erythrocytosis (hematocrit greater than 55%). The mean right ventricular ejection fraction was abnormal before (0.38) but not after (0.43) phlebotomy, and the increase was highly significant (p less than 0.001). Similarly, the right ventricular end-diastolic area decreased significantly from 588 to 505 pixels (p less than 0.02). No significant changes occurred on the left side. Cardiac output was unchanged or slightly increased. Thus, assuming that afterload was unchanged, as reported by other investigators, the data herein are consistent with the hypothesis that a normalization in preload shifts the Starling curve, resulting in improved right ventricular performance with preservation of the cardiac output.

Adult↗

Cardiac hemochromatosis: beneficial effects of iron removal therapy. An echocardiographic study.

The echocardiographic (echo) features of idiopathic hemochromatosis (IH) were studied in 22 patients. Results were compared with a control group of 22 patients without heart disease. Statistically significant increases in left ventricular (LV) mass, end-diastolic and end-systolic diameters of the left ventricle and in left atrial dimension were observed in patients with IH; significant changes of systolic function indexes (decrease in fractional shortening and ejection fraction and increase in distance of the E point to the septum) were seen as well. These echo abnormalities were mainly seen in patients with abnormal electrocardiograms. In 11 patients with IH, iron removal therapy was carried out by means of periodic phlebotomies. In patients with impaired LV function at the beginning of therapy, comparison between measurements of the initial echo and posttreatment echo showed significant improvement in LV diameters, fractional shortening, ejection fraction, distance from the E point to the septum, LV mass and left atrial dimension.

Adult↗

Primary hemochromatosis: anatomic and physiologic characteristics of the cardiac ventricles and their response to phlebotomy.

M-mode and 2-dimensional echocardiography and gated equilibrium blood pool imaging (rest and exercise) were used in 10 patients with primary hemochromatosis to characterize the spectrum of pathophysiologic abnormalities of the cardiac ventricles and to determine the response to chronic therapeutic phlebotomy. Dilated and restrictive cardiomyopathic patterns were identified in 1 patient each, but our data do not permit conclusions on when in the natural history a given pattern becomes overt. On entry into study, 3 patients had normal ventricles and 7 had ventricular abnormalities on echocardiography and blood pool angiography. In 2 of the latter patients, biventricular dysfunction and increased left ventricular (LV) mass normalized after phlebotomy; 1 patient achieved a normal LV response to exercise. Of the 4 patients with isolated abnormal LV ejection fraction responses to exercise, the EF normalized in 2 after phlebotomy. In 1 patient, isolated right ventricular enlargement and dysfunction (echocardiographic and radionuclide imaging) normalized after phlebotomy. Thus, primary hemochromatosis can effect LV and RV size and function; clinically occult cardiac involvement can be identified by echocardiography and equilibrium blood pool imaging; therapeutic phlebotomy can ameliorate or reverse the deleterious effects of excess cardiac iron deposition which appears to exert its harm, at least in part, by a mechanism other than irreversible connective tissue replacement.

Adult↗

Hemodynamic effects of staged hematocrit reduction in patients with stable cor pulmonale and severely elevated hematocrit levels.

Patients with cor pulmonale and high hematocrit levels are often subjected to phlebotomy in the belief that the adverse effects of high viscosity may outweigh the benefit of increased oxygen carrying capacity. To evaluate this, 12 patients with stable cor pulmonale and hematocrit values greater than 55 per cent were studied before and after a series of venesections. Right heart and aortic pressures, cardiac output and blood gases were measured at three mean hematocrit levels, 61 per cent (stage I), 50 per cent (stage II) and 44 per cent (stage III), with blood volume unchanged. From stages I to II, there were significant decreases in both man pulmonary artery pressure and total pulmonary resistance. Oxygen transport fell but not oxygen consumption. Right ventricular end-diastolic pressure and cardiac output did not change. Right ventricular work either fell or was maintained by increased output. Frank-Starling performance (supine exercise) improved. No significant changes occurred with further reduction in hematocrit to normal levels (stage III). The findings of this study support the concept of overcompensating erythrocytosis in cor pulmonale, and the effects of moderate hematocrit reduction should not be overlooked in these severely ill patients.

Adult↗

Arthritis of hemochromatosis. Clinical spectrum, relation to histocompatibility antigens, and effectiveness of early phlebotomy.

Five patients who presented with arthritis as the sole manifestation of hereditary hemochromatosis and 51 family members were studied. Studies included clinical evaluation for the presence of arthritis and hemochromatosis, roentgenography of hands, knees, and pelvis, serum iron and serum ferritin measurements, complete HLA typing for 50 of the A and B loci, and, when indicated, liver biopsy. Arthritis occurred in 45 percent of persons with hemochromatosis. Although typical involvement of second and third metacarpophalangeal joints was observed in all five patients and some family members, two with typical arthritis did not have characteristic radiographic changes, two had constitutional symptoms without arthropathy, and one had unilateral hand changes. A specific HLA haplotype (A2/B17 in Family 1 and A29/B15 in Family 2) correlated with hereditary hemochromatosis but not with the arthropathy. Phlebotomy alleviated the early constitutional symptoms but did not help advanced arthritis. Anti-inflammatory drugs, intraarticular injections of glucocorticoids, and resection osteotomies of metacarpal heads were other treatment modalities.

Anti-Inflammatory Agents↗

Improved exercise tolerance of the polycythemic lung patient following phlebotomy.

The present study evaluated the effects of therapeutic phlebotomy on the exercise tolerance and the maximal carbon dioxide output of polycythemic patients with chronic obstructive pulmonary disease. Fifteen maximal exercise studies were performed before and after phlebotomy in patients with moderate to severe chronic obstructive pulmonary disease (mean forced expiratory volume in one second [FEV1]= 970 ml). After phlebotomy there were no significant differences in pulmonary function, blood gases, oxygen consumption, or carbon dioxide production at rest. However, after phlebotomy there was a significant increase in the exercise tolerance of the patients. The mean workload, the duration of exercise, the maximal oxygen consumption, the maximal carbon dioxide production, and the ventilation at maximal exercise all increased significantly. The improved exercise tolerance after phlebotomy appeared due to an increased cardiac output generated mainly through an increased stroke volume. We hypothesize that the increased stroke volume was due to a higher ejection fraction of the right ventricle secondary to a lower pulmonary artery pressure. This study provides further evidence that patients with chronic obstructive pulmonary disease who have polycythemia benefit by therapeutic interventions that maintain their hematocrits below 55 percent.

Aged↗

Evidence for heterogeneity in hereditary hemochromatosis. Evaluation of 174 persons in nine families.

Hereditary hemochromatosis is an autosomal recessive disease in which the gene is linked to the HLA system. Investigation of nine unrelated probands and their family members has revealed distinct groups based on biochemical and clinical manifestations of the disease. Four different types of disease expression were identified: Group I--classic hereditary hemochromatosis with elevated transferrin saturation, serum ferritin levels, and liver iron content; Group II--severe iron overload, accelerated disease manifesting at an early age; Group III--elevated total body iron stores, normal transferrin saturation and serum ferritin levels; Group IV--markedly elevated findings on serum biochemical tests, e.g., transferrin saturation, serum ferritin levels, with minimal elevation in total body iron stores. This evidence for several clearly distinguishable modes of expression in different families suggests that more than one genetic lesion in iron metabolism may be responsible for iron overload in hereditary hemochromatosis. This genetic heterogeneity may be helpful in delineating the fundamental abnormalities in iron metabolism in this group of disorders.

Absorption↗

Intrapartum fetal surveillance: the role of fetal scalp blood sampling.

Continuous electronic fetal heart rate monitoring and fetal scalp blood sampling have traditionally played a complementary role in intrapartum fetal surveillance. Nevertheless, biochemical assessment of fetal blood pH, with the use of scalp or umbilical cord blood, is often viewed as the "gold standard" against which biophysical indicators of fetal distress must be judged. In actual clinical practice, however, fetal scalp blood sampling is only rarely used. In addition, there is a growing body of evidence to suggest that, when properly interpreted, fetal heart rate assessment may be equal or superior to measurement of fetal blood pH in the prediction of both good and bad fetal outcomes. Under certain circumstances, fetal scalp blood sampling remains a valuable clinical tool; however, we recommend a deemphasis of fetal scalp blood sampling in general clinical practice. Both theoretical and practical considerations suggest that the properly trained clinician may pursue an approach for the detection of fetal distress that does not include scalp blood sampling without either compromising his ability to detect fetal distress or significantly increasing the cesarean section rate.

Apgar Score↗

Donation of blood by the pregnant patient for autologous transfusion.

A study was conducted to determine the safety and utility of autologous blood donation in third trimester pregnancy. Thirty-seven obstetric patients, 32 with an obstetric risk factor, donated an average of 485 ml of blood. Twenty-one of the 37 patients were expected to undergo cesarean section. Nonstress testing was performed before and after phlebotomy. Continuous fetal heart rate monitoring was maintained throughout the donation, which lasted an average of 9 minutes. All nonstress test results were normal before and after the phlebotomy except in one case. All fetal heart rates remained stable during phlebotomy and premature labor was not precipitated. All fetal outcomes were normal. One patient delivered on the day of phlebotomy, 6 hours after the procedure. Only one of the autologous units was used, in a patient who had a pelvic infection and moderate anemia. The incidence of primary cesarean section was 35%. Phlebotomy of the mother appears to be safe for the fetus at term. Further investigation is needed to determine the safety of removal of more than 1 unit of blood and blood donation at earlier gestational ages.

Adult↗