Assessment of systolic, diastolic function and development of pulmonary hypertension in children with ?-Thalassemia Intermedia and Sickle Cell Disease (SCD) using conventional and tissue Doppler echocardiography

Sickle Cell Disease and andbeta;-Thalassemia Intermedia are major genetic blood disorders in Oman. Both are Hemoglobinopathies characterized by non-transfusion dependence and chronic hemolytic process. This chronic heomolytic process depletes the Nitric Oxide resulting in Pulmonary Vaso- Constriction and in long term a pulmonary hypertension. Another pathophysiologic mechanism involves a hypercoagulable state with increased incidence of thrombo-embolic events. Previous results have shown that pulmonary hypertension can be reversible by regular blood transfusion. In this study, we intend to assess the cardiac functions using conventional and tissue Doppler echocardiography in a cohort of children with B-Thalassemia intermedia and sickle cell disease.