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Cardiac pathology in 76 thalassemic patients
D Sonakul1, K Thakerngpol, P Pacharee
1Department of Pathology, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Insights
Heart disease is a significant cause of death in thalassemia patients. This autopsy study found cardiac hypertrophy and pulmonary issues were common in beta-thalassemia/Hb E and Hb H disease.
Area of Science:
- Cardiology
- Hematology
- Pathology
Background:
- Heart disease is a leading cause of mortality in thalassemia patients post-childhood.
- Different types of thalassemia, including beta-thalassemia major, beta-thalassemia/Hb E, and Hb H disease, present varied clinical challenges.
Purpose of the Study:
- To investigate the cardiac pathology in patients with different types of thalassemia.
- To determine the prevalence of cardiac complications such as hypertrophy, pericarditis, and thromboembolism.
Main Methods:
- Autopsy analysis of 76 patients with various forms of thalassemia.
- Detailed examination of cardiac tissues for hypertrophy, dilatation, iron deposition, and pericarditis.
- Correlation of cardiac findings with specific thalassemia types and clinical history.
Main Results:
- Beta-thalassemia/Hb E patients (58) frequently exhibited cardiac hypertrophy (57/58) and pulmonary thromboembolism (19/35).
- Iron deposition was minimal across most cases, contrasting with more severe cardiac changes in beta-thalassemia major (6 patients).
- Hb H disease patients (12) showed less cardiac pathology, though two had rheumatic heart disease and one experienced pulmonary embolism.
Conclusions:
- Cardiac hypertrophy and pulmonary thromboembolism are prevalent in beta-thalassemia/Hb E and Hb H disease.
- While iron overload is not a primary factor, other cardiac pathologies significantly impact survival in these thalassemia forms.
- Further research into managing cardiac complications in thalassemia is warranted.
Abstract:
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.