Abstract

Introduction: Thalassemia is the most common genetic disorder worldwide. Regular transfusion therapy, while improving patient quality of life, creates a state of iron overload. Once reticuloendothelial stores saturate, iron deposition increases in parenchymal tissues such as endocrine glands, hepatocytes, and myocardium. Cardiac iron deposition produces arrhythmias, systolic and diastolic dysfunction, and congestive heart failure in the second or third life decade. Aims and Objective: The present study was planned to find the prevalence of thalassemia cardiomyopathy and to study the spectrum of cardiac disease in thalassemia patients. Methods: All consecutive patients of thalassemia more than 12-year-old were included in this study. Screening included medical history assessment, focusing on cardiovascular symptomatology, transfusion and chelation history, physical examination, and transthoracic resting echocardiography. Clinical parameters included age, sex, address, height weight, body mass index (BMI), systolic blood pressure (BP), diastolic BP, mean hemoglobin, and mean serum ferritin. Results: A total of 56 patients of thalassemia were included in the study. Fifty-one patients were of thalassemia major and 5 patients were of thalassemia intermedia. Mean age of patients included in our study was 15.9 ± 4.6 years. Mean duration of thalassemia in our patients was 12.9 ± 3.05 years. Mean hemoglobin of the patients included in our study was 8.7 ± 1.1 g%. The level of mean serum ferritin in our patients was 666.69 ± 325.46 ng/ml. In our study, we had 8 (14.2%) patients out of 56 having systolic dysfunction. Furthermore, our patients with left ventricular systolic dysfunction had younger mean age as compared to those patients who had a normal left ventricular function. We also found a higher ferritin value in patients with left ventricular dysfunction as compared to patients with normal left ventricular dysfunction studies. Conclusion: In our study, we found out that patients with left ventricular dysfunction had a younger age of onset of disease, higher ferritin levels, lower blood pressure, and paradoxically higher BMI as compared to patients with normal left ventricular dysfunction.

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