Arq. Bras. Cardiol. 2019; 113(3): 438-443
Immobile Tricuspid Valve: Incidental Finding in a Case of Terminal Cardiomyopathy Due to Thalassemia Major
DOI: 10.5935/abc.20190195
Introduction
Thalassemia Major is an inherited disorder caused by impaired synthesis of the B globin chain and characterized by ineffective erythropoiesis that requires regular, lifelong transfusion therapy, which creates a state of iron overload. Once reticuloendothelial stores saturate, iron deposition increases in myocardium such as other parenchymal tissues. Cardiac complications due to this deposition are the leading cause of death. After a silent first decade, iron deposits in the cardiac tissue lead to arrhythmias, systolic and diastolic dysfunction, and congestive heart failure in the second or third decade. In this case report, we present an adolescent girl who did not receive regular iron chelation therapy and had cardiomyopathy, arrhythmia and immobile tricuspid valve secondary to thalassemia major.
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