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Query: UMLS:C0039730 (thalassemia)
 10,305 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Autopsy protocols and heart slides of 47 patients with beta thalassaemia/haemoglobin E disease were reviewed. All but 1 patient had cardiac hypertrophy, accompanied by dilatation in 17; 5 of 9 patients (56%) with right ventricular and 11 of 22 patients (50%) with biventricular hypertrophy had chronic pulmonary thromboembolism. Cardiac iron deposition while present in 15 patients (32%) was very slight, in contrast to the amount of iron in their liver and pancreas. Four patients had fibrinous pericarditis, 2 with rheumatic heart disease. Twelve patients had chronic pericardial changes, 7 with adhesive pericarditis. The effects of cardiac pathology on the morbidity and mortality of patients with beta thalassaemia/haemoglobin E disease were discussed.
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PMID:Cardiac pathology in 47 patients with beta thalassaemia/haemoglobin E. 624 76

A case of pericardial effusion due to Campylobacter fetus in a patient with thalassemia is presented. The patient failed to respond to ceftriaxone and clarithromycin despite in vitro susceptibility, but improved after pericardiectomy and ampicillin. Pericarditis due to C. fetus has rarely been reported. A high index of suspicion is essential to recognise this organism, because of its special microbiological characteristics.
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PMID:Campylobacter fetus pericarditis in a patient with beta-thalassemia: case report and review of the literature. 1167 37

We present a 57-year old male patient with thalassemia intermedia and right heart failure. He had a 30-year history of anemia and short-term iron therapy without blood transfusion. Hemoglobin level was 7.1 g/dl and hematocrit was 22.7%. White blood-cell and platelet counts, and serum ferritin level were normal. Electrocardiography showed irregular narrow QRS bradyarrhythmia, suggesting slow atrial fibrillation at a mean rate of 35 beats/min. Echocardiographic examination revealed dilatation of the right atrium and ventricle, depressed systolic right ventricular function, advanced tricuspid regurgitation, and mild pericardial effusion. In the electrophysiologic study, no electrical activity was recorded in the right atrium. It was inexcitable at multiple sites and no retrograde conduction to the right atrium could be elicited by ventricular pacing. His bundle (HB) recording showed fixed retrograde HB activation with ventricular rhythm originating from different foci. Retrograde V-H conduction time during ventricular rhythm was 95 msec and did not change. There was no retrograde nodal conduction. A VVIR pacemaker was implanted. During a six-month follow-up, he felt well, his functional capacity was NYHA class II, and his basic rhythm was widened QRS arrhythmia with a rate of 20 beats/min. To the best of our knowledge, atrial electrical inactivity together with right-heart failure and pericarditis confined to the right heart chambers has hitherto not been reported in thalassemic disorders.
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PMID:Persistent atrial standstill and idioventricular rhythm in a patient with thalassemia intermedia. 1971 59

Beta-thalassaemia major is a genetic blood disorder caused by the reduced synthesis of beta globin chain. The consequences of the resulting chronic anaemia are also common and include growth retardation, bone marrow expansion, extramedular hematopoiesis, splenomegaly, increased intestinal iron absorption, susceptibility to infections, and hypercoagulability. Transfusional iron overload can affect heart function by directly damaging tissue through iron deposition or via iron-mediated effects at other sites. Cardiac dysfunction is common in patients with thalassaemia and is the leading cause of mortality. The main cardiac abnormalities reported in patients with thalassaemia major (TM) and iron overload are left ventricular systolic and diastolic dysfunction, pulmonary hypertension, valvulopathies, arrhythmias and pericarditis. These cardiac abnormalities are a consequence of the general co-morbid conditions in thalassaemia but are closely related to concomitant endocrine deficiencies, hypercoagulability state and inflammatory milieu. Iron's toxicity within cells arises from its capacity to catalyse the production of reactive oxygen species that cause lipid peroxidation and organelle damage, which lead ultimately to cell death and fibrosis. With the introduction of new technologies such as cardiac magnetic resonance T2* , the early detection of cardiac iron overload and associated cardiac dysfunction is now possible, allowing time for reversal through iron chelation therapy.
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PMID:Cardiovascular aspect of Beta-thalassaemia. 2223 92

Hereditary hemoglobin disorders affecting the globin chain synthesis namely thalassemia syndromes and sickle cell disease (SCD) are the most common genetic disorders in human. Around 7% of the world population carries genes for these disorders, mainly the Mediterranean Basin, Middle and Far East, and Sub-Saharan Africa. An estimated 30 million people worldwide are living with sickle cell disease, while 60-80 million carry beta thalassemia trait. About 400,000 children are born with severe hemoglobinopathies each year. Cardiovascular complications of hemoglobinopathies include left and right ventricular (RV) dysfunction, arrhythmias, pericarditis, myocarditis, valvular heart disease, myocardial ischemia, and notably pulmonary hypertension (PH). Because of a unique pathophysiology, pulmonary hypertension associated with hemolytic disorders was moved from WHO group I to group V PH diseases. Treatment strategies are also unique and include blood transfusion, iron chelation, hydroxyurea, and oxygen therapy. The role of PH-specific agents has not been established.
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PMID:Saudi Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Pulmonary hypertension associated with hemolytic anemia. 2507