Prevalence of Cardiac Complications in Transfusion dependent Thalassemia Patients in Oman A National Cohort Study

Beta-thalassemia is an autosomal recessive hemoglobin disorder and one of humankind's most common genetic diseases. Homozygous Beta-thalassemia affects the synthesis of ?-globin chains, leading to chronic anemia, ineffective erythropoiesis, and the need for regular blood transfusions early in infancy. Regular blood transfusions every 3?4 weeks are the standard therapy for transfusion-dependent Beta-thalassemia, also known as thalassemia major and thalassemia intermedia. Nearly 5% of the global population carries the globin gene variants. It is prevalent in Oman, as local studies have shown an estimated carrier incidence of 2.2%-4%. The management of thalassemia major and thalassemia intermedia has evolved over the last few decades through blood transfusion, chelation therapy, and hematopoietic stem cell transplantation. Despite the advances in the management of transfusion-dependent thalassemia, heart disease remains the leading cause of morbidity and mortality in these patients, accounting for almost three-quarters of deaths secondary to iron overload. Cardiac involvement in thalassemia encompasses a spectrum of disorders, including myocardial dysfunction, atrial and ventricular arrhythmias, pulmonary hypertension, and peripheral vascular disease. This study aims to determine cardiac as well as systemic complications in thalassemia major and thalassemia intermedia patients in Oman and markers of fibrosis.