Cardiac pathology in 76 thalassemic patients

Heart disease is a major cause of death in thal patients after the first decade of life. This study was carried out on autopsy material from 76 patients, six with beta-thal major, 58 with beta-thal/Hb E, and 12 with Hb H disease. Of the 58 patients with beta-thal/Hb E, which form the main group, all but one had cardiac hypertrophy, accompanied by dilatation in 17, five of ten patients with right ventricular and 14 of 25 patients with biventricular hypertrophy had chronic pulmonary thromboembolism. Iron deposition, while present in 18 patients, was very slight. Four patients had fibrinous pericarditis, two with diagnostic rheumatic heart disease; 15 patients had chronic pericarditis, with extensive fibrosis in half the cases. In comparison, the six patients with beta-thal major showed more severe cardiac changes, including more iron deposition. Of the 12 Hb H patients, two died of rheumatic heart disease, one had frank bilateral pulmonary embolism and striking right ventricular hypertrophy, while the remaining nine showed little cardiac pathology.