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

Erythrocytosis, thrombocytopenia, platelets function defects, coagulation factors deficiencies are the main haematologic disorders in patients with cyanotic congenital heart disease. They are responsible for increased bleeding tendency and contraindication of anticoagulation. Phlebotomy in this group of patients should be recommended when erythrocytosis is accompanied by clinical symptoms. Excessive phlebotomy can lead to iron deficiency and cause hyperviscosity symptoms. The iron supplementation should be closely monitored.
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PMID:[Hematologic disorders in patients with cyanotic congenital heart disease]. 1052 25

In general, most authors advocate nonintervention in Eisenmenger's syndrome, but an awareness of potential palliative measures to avoid destabilizing a delicately balanced physiology is needed as well. This approach has failed to alter long-term outcome, however. Survival for patients with Eisenmenger's syndrome has not improved substantially in the past several decades. Quality of life is universally altered by the presence of cyanosis, exercise intolerance, and the comorbid conditions associated with erythrocytosis. We therefore believe that the use of novel alternatives, as they become available, is warranted and that these alternatives are likely to be best evaluated in multicenter collaborative trials. The approach to the patient with pulmonary vascular obstructive disease (PVOD) should begin with maximization of palliative therapy and should, as compliance and teaching are ensured, proceed to the use of therapies designed to reverse the underlying proliferative changes in the pulmonary vasculature. Frequent checking of potential supplemental oxygen responsiveness and use of inhaled oxygen as needed to maximize systemic arterial saturation should be considered, although evidence of the value of home oxygen use is currently lacking. We favor systemic anticoagulation to a target international normalized ratio of 2.0 to 2.5. There are currently no published data supporting this practice in patients with PVOD, but we believe that as in patients with primary pulmonary hypertension, benefit is likely to outweigh risk. In the setting of a meticulous outpatient anticoagulation service, we have witnessed acceptably low bleeding event rates. A controlled clinical trial is warranted. Selective pulmonary vasodilators and antiproliferative agents hold significant promise in altering the natural history of PVOD associated with intracardiac shunting. The risk of paradoxic embolism and the theoretical worsening of right- to-left shunting compound the already high risk of systemically administered therapies; neither, to date, has been limiting in our patients. Studies of infused or newer subcutaneous and inhaled formulations are under way, and preliminary experience suggests real benefit--improved hemodynamics, improved exercise tolerance, and increased systemic arterial saturation--in this group of patients. Lung transplantation still trades a disease for another set of problems associated with long-term immunosuppression and chronic graft rejection in patients with previous sternotomy and thoracotomy and with a high acute surgical risk. Population studies of mortality and morbidity in patients with PVOD associated with congenital heart disease who receive transplants do not seem to suggest significant improvement with this therapy. In the future, the management of Eisenmenger's syndrome will probably include a multipharmacologic approach that targets several factors in the inflammatory cascade leading to vascular proliferation, perhaps offered in concert with novel surgical or transcatheter strategies designed to limit intracardiac shunting and, if desired, provide complete repair of intracardiac defects.
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PMID:Eisenmenger's Syndrome. 1109 2

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 and hyperemic (intravenous dipyridamole) perfusion measurements were obtained with [13N]ammonia positron emission tomographic imaging in left (LV) and right (RV) ventricular and septal myocardium in 14 adults with CCHD [age: 34.1 yr (SD 6.5)]; hematocrit: 62.2% (SD 4.8)] and 10 healthy controls [age: 34.1 yr (SD 6.5)]. In patients, basal perfusion measurements were higher in LV [0.77 (SD 0.24) vs. 0.55 ml x min(-1) x g(-1) (SD 0.09), P < 0.02], septum [0.71 (SD 0.16) vs. 0.49 ml x min(-1) x g(-1) (SD 0.09), P < 0.001], and RV [0.77 (SD 0.30) vs. 0.38 ml x min(-1) x g(-1) (SD 0.09), P < 0.001]. However, basal measurements normalized for the rate-pressure product were similar to those of controls. Calculated oxygen delivery relative to rate-pressure product was higher in the patients [2.2 (SD 0.8) vs. 1.6 (SD 0.4) x 10(-5) ml O2 x min(-1) x g tissue(-1) x (beats x mmHg)(-1) in the LV, P < 0.05, and 2.0 (SD 0.7) vs. 1.4 (SD 0.3) x 10(-5) ml O2 x min(-1) x g tissue(-1) x (beats x mmHg)(-1) in the septum, P < 0.01]. Hyperemic perfusion measurements in CCHD patients did not differ from controls [LV, 1.67 (SD 0.60) vs. 1.95 ml x min(-1) x g(-1) (SD 0.46); septum, 1.44 (SD 0.56) vs. 1.98 ml x min(-1) x g(-1) (SD 0.69); RV, 1.56 (SD 0.56) vs. 1.65 ml x min(-1) x g(-1) (SD 0.64), P = not significant], and coronary vascular resistances were comparable [LV, 55 (SD 25) vs. 48 mmHg x ml(-1) x g x min (SD 16); septum, 67 (SD 35) vs. 50 mmHg x ml(-1) x g x min (SD 21); RV, 59 (SD 26) vs. 61 mmHg x ml(-1) x g x min (SD 27), P = not significant]. These findings suggest that adult CCHD patients have remodeling of the coronary circulation to compensate for the rheologic changes attending chronic hypoxemia.
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PMID:Myocardial perfusion reserve in adults with cyanotic congenital heart disease. 1600 39

Erythrocytosis is an adaptive response to improve oxygen transport in cyanotic congenital heart disease (CCHD). However, at highly increased hematocrit levels patients may experience hyperviscosity symptoms. Iron deficiency in CCHD patients is often overlooked due to elevated hemoglobin concentrations. A 29-year-old male with CCHD was readmitted to our outpatient clinic. Red blood cells (11.65*10(12)/L), hemoglobin (25.7 g/dL), and hematocrit (80%) were extremely elevated. Measurements of iron supply showed a constellation typical for iron deficiency with low ferritin (13.2 microg/L), and high sTfR (20 mg/L). We present a case of extremely high red blood cell counts with concomitant iron deficiency. For appropriate management and to avoid misinterpretation of the iron status, ferritin and sTfR should always accomplish laboratory examination of CCHD patients.
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PMID:Iron deficiency in a patient with extreme erythrocytosis due to cyanotic congenital heart disease. 1709 63

Blood hyperviscosity due to secondary erythrocytosis is a common pathologic feature of cyanotic congenital heart disease (CCHD). In CCHD, it is possible that hematological parameters other than red blood cells influence blood rheology. We measured blood passage time to evaluate the blood rheology in patients with CCHD (n=18, age: 15.3+/-11.9 years, mean +/- SD) and age-matched control subjects (n=27) using the microchannel array flow analyzer (MC-FAN), and the results [several hematological parameters, including hematocrit (Hct)] were compared. Blood passage time in the CCHD group was prolonged, compared with the control group (67.6+/-27.2 s vs. 44.6+/-6.7 s). For the CCHD group, blood passage time correlated significantly with red blood cell (RBC) count, hemoglobin (Hb) concentration, Hct, mean corpuscular hemoglobin concentration (MCHC), platelet (Plt) count, high-density lipoprotein cholesterol (HDL-C) level, and triglycerides (TG) level (RBC, r=0.77; Hb, r=0.69; Hct, r=0.73; MCHC, r=-0.64; Plt, r=-0.49; TG, r=0.53; HDL-C, r=-0.49, p<0.05 for each variable). For all 45 subjects, blood passage time correlated significantly with HbA1c level (r=0.45, p<0.01) and tissue-type plasminogen activator (t-PA) antigen level (r=0.46, p<0.01). Our results indicated that blood rheology is reduced in patients with CCHD as expressed by prolonged blood passage time, and it may be defined by several blood parameters in addition to erythrocytosis.
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PMID:Evaluation of blood rheology in patients with cyanotic congenital heart disease using a microchannel array flow analyzer. 1714 49

Complications of chronic hypoxia, including erythrocytosis, hyperviscosity, abnormalities of hemostasis, cerebral abscesses, stroke, and endocarditis, are among the most common consequences of cyanotic heart disease in adults. The compensatory erythrocytosis of cyanotic heart disease can become pathologic by causing an increase in blood viscosity, thereby decreasing perfusion and resulting in decreased total oxygen delivery and increased risk of venoocclusive/hyperviscosity syndrome. Treatment of hyperviscosity secondary to erythrocytosis in cyanotic heart disease is controversial. Data is limited but suggest that phlebotomy has the potential to increase exercise capacity, reduce the symptoms of hyperviscosity, and reduce the potential risk of vasoocclusive disease in selected patients with polycythemia secondary to cyanotic heart disease. Unfortunately, repeated phlebotomy can quickly lead to iron deficiency, resulting in microcytic erythrocytes that induce higher viscosity than normocytic erythrocytes, which may increase the risk for venoocclusive events. There are limited data on the use of hydroxyurea to suppress erythrocytosis in this patient population. The authors conclude that until newer approaches to decreasing hematocrit without inducing iron deficiency are shown to be safe and efficacious, phlebotomy should only be used for the acute resolution of hyperviscosity symptoms. In addition, the use of hydroxyurea should be limited to patients with recurrent symptoms.
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PMID:Blood is thicker than water: the management of hyperviscosity in adults with cyanotic heart disease. 1717 81

During the past three decades, interventional cardiology and cardiac surgery have found solutions even for the most complex congenital heart malformations with an overall low operative mortality. A careful clinical and instrumental follow-up of postoperative congenital heart disease patients is fundamental not only to prevent complications and/or to treat eventual residua and sequelae, but also to modify future surgical strategies on the basis of long-term results. To be able to give a correct prognostic meaning to the data collected during the follow-up, the cardiologist should have an excellent knowledge of the native defect, the surgical technique and the post-surgical anatomy and physiology. Major cardiological concerns during a follow-up after corrective surgery are: arrhythmias; heart failure; cyanosis and erythrocytosis; and infective endocarditis. Psychosocial needs, such as employment, contraception, pregnancy and physical exercise, are very important to enable a 'normal' life, complying with the postoperative hemodynamic situation of the patients.
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PMID:Follow-up and physical activity in postoperative congenital heart disease. 1725 23

Endothelial-derived nitric oxide (NO) diffuses abluminally to regulate blood flow by activating soluble guanylate cyclase in medial smooth muscle. However, a significant fraction of NO diffuses luminally, where the extremely high reaction rate with red blood cell hemoglobin (Hb) effectively reduces luminal concentration to zero. The erythrocytosis of cyanotic congenital heart disease has potentially opposing effects, namely, a reduction in medial smooth muscle NO bioavailability because of the increase in luminal consumption of the molecule and, conversely, an increase in the elaboration of NO in response to the high endothelial shear stress of the erythrocytotic perfusate. NO metabolism in cyanotic congenital heart disease is unknown. Accordingly, this study aimed to establish the metabolic fate of NO and to determine the degree to which its levels are altered. Blood samples from 25 nonfasting patients with cyanotic congenital heart disease and 25 nonfasting normal controls were collected in Vacutainer tubes containing citrate dextrose and in separate Vacutainer tubes containing a solution that specifically preserves S-nitrosated Hb. Total NO species, plasma S-nitrosated proteins, iron nitrosyl Hb, and S-nitrosated Hb were quantified using chemiluminescence. In conclusion, a significant increase in plasma concentrations of NO metabolites and a modest increase in iron nitrosyl Hb levels were found, suggesting increased luminal consumption caused by erythrocytosis and further suggesting that hypoxemia might activate nonoxidative NO metabolic pathways and enhance tissue oxygen delivery.
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PMID:Nitric oxide metabolism in adults with cyanotic congenital heart disease. 1731 73

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.
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PMID:Hydroxyurea therapy for management of secondary erythrocytosis in cyanotic congenital heart disease. 1750 64

Secondary erythrocytosis of cyanotic congenital heart disease (CCHD) is pathologically different from primary erythrocytosis of polycythemia vera (PV). An association between elevated hematocrit and thrombosis has been established in PV patients, and treatment guidelines recommend maintaining hematocrit <45%. Although an association between elevated hematocrit and thrombosis has not been established in CCHD and secondary erythrocytosis, the current clinical practice is to phlebotomize these patients to hematocrit <65%. We report a 21-year-old woman with CCHD who presented with symptomatic erythrocytosis with numbness and tingling with hemoglobin 25.2 g/dl and hematocrit 75.8%. Her symptoms resolved with IV hydration. Other factors, including dehydration and iron deficiency, may precipitate hyperviscosity symptoms. The treatment is volume replacement and low-dose iron therapy, not phlebotomy. Repeated phlebotomy causes iron deficiency with microcytic erythrocytes, which increases the whole blood viscosity and, therefore, can potentially accentuate rather than decrease the risk for a cerebrovascular accident.
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PMID:Cyanotic congenital heart disease (CCHD) with symptomatic erythrocytosis. 1791 83


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