CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT
Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia. The two main factors that dete...
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PAGEPress Publications
2009-07-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
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| Online Access: | http://www.mjhid.org/index.php/mjhid/article/view/122 |
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| author | Athanasios Aessopos Vasilios Berdoukas |
| author_facet | Athanasios Aessopos Vasilios Berdoukas |
| author_sort | Athanasios Aessopos |
| collection | DOAJ |
| description | Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia. The two main factors that determine cardiac disease in homozygous β thalassemia are the high output state that results from chronic tissue hypoxia, hypoxia-induced compensatory reactions and iron overload. The high output state playing a major role in thalassaemia intermedia and the iron load being more significant in the major form. Arrhythmias, vascular involvement that leads to an increased pulmonary vascular resistance and an increased systemic vascular stiffness and valvular abnormalities also contribute to the cardiac dysfunction in varying degrees according to the severity of the phenotype. Endocrine abnormalities, infections, renal function and medications can also play a role in the overall cardiac function. For thalassaemia major, regular and adequate blood transfusions and iron chelation therapy are the mainstays of management. The approach to thalassaemia intermedia, today, is aimed at monitoring for complications and initiating, timely, regular transfusions and/or iron chelation therapy. Once the patients are on transfusions, then they should be managed in the same way as the thalassaemia major patients. If cardiac manifestations of dysfunction are present in either form of thalassaemia, high pre transfusion Hb levels need to be maintained in order to reduce cardiac output and appropriate intensive chelation therapy needs to be instituted. In general recommendations on chelation, today, are usually made according to the Cardiac Magnetic Resonance findings, if available. With the advances in the latter technology and the ability to tailor chelation therapy according to the MRI findings as well as the availability of three iron chelators, together with increasing the transfusions as need, it is hoped that the incidence of cardiac dysfunction in these syndromes will be markedly reduced. This of course depends very much on the attention to detail with the monitoring and the cooperation of the patient with both the recommended investigations and the prescribed chelation. |
| format | Article |
| id | doaj-art-064adb1b75004898b10f3f8e5fec2c66 |
| institution | Kabale University |
| issn | 2035-3006 |
| language | English |
| publishDate | 2009-07-01 |
| publisher | PAGEPress Publications |
| record_format | Article |
| series | Mediterranean Journal of Hematology and Infectious Diseases |
| spelling | doaj-art-064adb1b75004898b10f3f8e5fec2c662024-12-02T00:01:34ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062009-07-0111e2009002e200900210.4084/mjhid.2009.00216CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENTAthanasios Aessopos0Vasilios Berdoukas1First Dept. of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, GreeceFirst Dept. of Internal Medicine, University of Athens Medical School, Laiko Hospital, Athens, GreeceHeart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia. The two main factors that determine cardiac disease in homozygous β thalassemia are the high output state that results from chronic tissue hypoxia, hypoxia-induced compensatory reactions and iron overload. The high output state playing a major role in thalassaemia intermedia and the iron load being more significant in the major form. Arrhythmias, vascular involvement that leads to an increased pulmonary vascular resistance and an increased systemic vascular stiffness and valvular abnormalities also contribute to the cardiac dysfunction in varying degrees according to the severity of the phenotype. Endocrine abnormalities, infections, renal function and medications can also play a role in the overall cardiac function. For thalassaemia major, regular and adequate blood transfusions and iron chelation therapy are the mainstays of management. The approach to thalassaemia intermedia, today, is aimed at monitoring for complications and initiating, timely, regular transfusions and/or iron chelation therapy. Once the patients are on transfusions, then they should be managed in the same way as the thalassaemia major patients. If cardiac manifestations of dysfunction are present in either form of thalassaemia, high pre transfusion Hb levels need to be maintained in order to reduce cardiac output and appropriate intensive chelation therapy needs to be instituted. In general recommendations on chelation, today, are usually made according to the Cardiac Magnetic Resonance findings, if available. With the advances in the latter technology and the ability to tailor chelation therapy according to the MRI findings as well as the availability of three iron chelators, together with increasing the transfusions as need, it is hoped that the incidence of cardiac dysfunction in these syndromes will be markedly reduced. This of course depends very much on the attention to detail with the monitoring and the cooperation of the patient with both the recommended investigations and the prescribed chelation.http://www.mjhid.org/index.php/mjhid/article/view/122β-thalassemia, thalassemia major, thalassemia intermedia, heart disease, pulmonary hypertension, high output state. |
| spellingShingle | Athanasios Aessopos Vasilios Berdoukas CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT Mediterranean Journal of Hematology and Infectious Diseases β-thalassemia, thalassemia major, thalassemia intermedia, heart disease, pulmonary hypertension, high output state. |
| title | CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT |
| title_full | CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT |
| title_fullStr | CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT |
| title_full_unstemmed | CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT |
| title_short | CARDIAC FUNCTION AND IRON CHELATION IN THALASSEMIA MAJOR AND INTERMEDIA: A REVIEW OF THE UNDERLYING PATHOPHYSIOLOGY AND APPROACH TO CHELATION MANAGEMENT |
| title_sort | cardiac function and iron chelation in thalassemia major and intermedia a review of the underlying pathophysiology and approach to chelation management |
| topic | β-thalassemia, thalassemia major, thalassemia intermedia, heart disease, pulmonary hypertension, high output state. |
| url | http://www.mjhid.org/index.php/mjhid/article/view/122 |
| work_keys_str_mv | AT athanasiosaessopos cardiacfunctionandironchelationinthalassemiamajorandintermediaareviewoftheunderlyingpathophysiologyandapproachtochelationmanagement AT vasiliosberdoukas cardiacfunctionandironchelationinthalassemiamajorandintermediaareviewoftheunderlyingpathophysiologyandapproachtochelationmanagement |