Cardiac pathology in 47 patients with beta thalassaemia/haemoglobin E.
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.
D000293:Adolescent; D000328:Adult; D000368:Aged; D006332:Cardiomegaly / Q000150:complications / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006331:Heart Diseases / Q000150:complications / Q000473:pathology*; D006349:Heart Valve Diseases / Q000150:complications / Q000473:pathology; D006446:Hemoglobin E; D006455:Hemoglobins, Abnormal; D006801:Humans; D007223:Infant; D007501:Iron / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000378:metabolism / Q000473:pathology; D010493:Pericarditis / Q000150:complications / Q000473:pathology; D013789:Thalassemia / Q000097:blood / Q000150:complications* / Q000378:metabolism
D006455:Hemoglobins, Abnormal; D006446:Hemoglobin E; D007501:Iron