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.