ABC | Volume 113, Nº3, September 2019

Case Report Cilsal Single-ventricle atrioventricular connection in elderly individual Arq Bras Cardiol. 2019; 113(3):438-443 Figure 3 – Transthoracic echocardiography from the parasternal view by tilting transducer inferomedially exploring the right atrium (RA) and right ventricle (RV) inflow tract; immobile leaflets of the tricuspid valve (TV) leading to severe insufficiency. of implantable cardioverter defibrillator was decided after the patient was taken to control ventricular arrhythmias with amiodarone, she died due to ventricular tachycardia on the 22nd day of hospitalization. Discussion In thalassemia, cardiovascular system involvement is pivotal in the prognosis and quality of life. Iron overload cardiomyopathy is the leading cause of mortality accounts up to 67% and 71% in thalassemia. 4 As iron overload, multiple factors such as chronic anemia, hypersplenism, non-progressive restrictive lung disease also lead to cardiac complications in Thalassemia Major. 5 Iron is mainly stored in myocytes and other cells in the form of free iron, also ferritin and hemosiderin. Free iron, which is referred to as labile cellular iron, is the most toxic form of iron and also the most accessible form for chelation. The goal of iron chelation therapy is to reduce the iron deposition especially in plasma and other tissues. In some cases, these heart complications were reported as reversible with early detection of iron overload and response to regular iron chelation therapy. 6 Cardiac magnetic resonance imaging (MRI) is the gold standard for detecting myocardial iron deposition. In our case, cardiac MRI was not performed due to lack of experienced staff in our hospital. Progressive increase of brain natriuretic Video 1 – Transthoracic echocardiography from the right ventricular inflow view in systole showing immobile leaflets of tricuspid valve in a fixed open position, causing mal-coaptation and severe regurgitation. To view the video click on the link: https://bit.ly/2lTc6iX 440