DIAGNOSIS AND MANAGEMENT OF THALASEMIC CARDIOMYOPATHY

AGUNG RIZKA PRATAMA

Abstract


Thalassemia is a autosomal recessive inherited conditions characterized by decreased or absent polypeptide chain synthesis resulting in reduced hemoglobin in red blood cells and anemia. Synthesis disorders are characterized by ineffective formation of erythropoiesis which causes the need for lifelong transfusions, this will lead to increased intestinal iron absorption and leading to iron accumulation in vital organs, accumulation of iron in the heart can cause myocardial dysfunction including serious complications such as cardiomipopathy of the heart, where thalassemic cardiomyopathy is a the main cause of morbidity was 63.6% and the main cause of death was 71% of thalassemia patients. It is necessary to early detection for thalassemia cardiomyopathy, such as periodic electrocardiography and echocardiography examinations to assess diastolic and systolic functions so that they do not develop into cardiomyopathy. The proper management of heart failure due to thalassemia cardiomyopathy must be optimally given to patients including chelation therapy which can be used as cardioprotective in thalassemic patients.


Keywords


thalassemia; cardiomyopathy; chelation

References


Peters M., Heijboer H., Smiers F. and Giordano P. C. Diagnosis and management of thalassaemia. BMJ. 2012;344:e228.

Riset Kesehatan Dasar. Badan Penelitian dan Pengembangan Kesehatan Departemen Kesehatan. 2007.

Koohi F., Kazemi T. and Miri-Moghaddam E. Cardiac complications and iron overload in beta thalassemia major patients-a systematic review and meta-analysis. Ann Hematol. 2019;98:1323-1331.

Koonrungsesomboon N., Tantiworawit A., Phrommintikul A., Saekho S., Srichairattanakool S. and Chattipakorn N. Heart Rate Variability for Early Detection of Iron Overload Cardiomyopathy in beta-Thalassemia Patients. Hemoglobin. 2015;39:281-6.

Gujja P., Rosing D. R., Tripodi D. J. and Shizukuda Y. Iron overload cardiomyopathy: better understanding of an increasing disorder. J Am Coll Cardiol. 2010;56:1001-12.

Kremastinos D. T., Farmakis D., Aessopos A., Hahalis G., Hamodraka E., Tsiapras D., et al. Beta-thalassemia cardiomyopathy: history, present considerations, and future perspectives. Circ Heart Fail. 2010;3:451-8.

Kremastinos D. T., Toutouzas P. K., Vyssoulis G. P., Venetis C. A., Vretou H. P. and Avgoustakis D. G. Global and segmental left ventricular function in beta-thalassemia. Cardiology. 1985;72:129-39.

Murphy C. J. and Oudit G. Y. Iron-overload cardiomyopathy: pathophysiology, diagnosis, and treatment. J Card Fail. 2010;16:888-900.

Walker J. M. Thalassaemia major and the heart: a toxic cardiomyopathy tamed? Heart. 2013;99:827-34.

Kremastinos D. T., Tsetsos G. A., Tsiapras D. P., Karavolias G. K., Ladis V. A. and Kattamis C. A. Heart failure in beta thalassemia: a 5-year follow-up study. Am J Med. 2001;111:349-54.

Kremastinos D. T. and Farmakis D. Iron overload cardiomyopathy in clinical practice. Circulation. 2011;124:2253-63.

Ponikowski P., Voors A. A., Anker S. D., Bueno H., Cleland J. G. F., Coats A. J. S., et al. 2016 ESC Guidelines for the Diagnosis and Treatment of Acute and Chronic Heart Failure. Eur Heart J. 2016;69:1167.

Auger D. and Pennell D. J. Cardiac complications in thalassemia major. Ann N Y Acad Sci. 2016;1368:56-64.

Gammella E., Recalcati S., Rybinska I., Buratti P. and Cairo G. Iron-induced damage in cardiomyopathy: oxidative-dependent and independent mechanisms. Oxid Med Cell Longev. 2015;2015:230182.

Pennell D. J., Udelson J. E., Arai A. E., Bozkurt B., Cohen A. R., Galanello R., et al. Cardiovascular function and treatment in beta-thalassemia major: a consensus statement from the American Heart Association. Circulation. 2013;128:281-308.


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DOI: 10.24815/jks.v21i3.21063

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