Pulmonary hypertension in adolescents with sickle cell disease

Sickle cell disease consists of a group of disorders that have a similar mutation in at least one of the beta-globin chains of hemoglobin. This results in a change of the hemoglobin to sickle shaped cells when in the deoxygenated state. It is these sickled cells that lead to the symptoms and complications characteristic of sickle cell disease, including vaso-occlusion. The recurrence of sickling and polymerization in the blood vessels throughout the body ultimately results in tissue and organ damage that increases the mortality of patients. Pulmonary hypertension is the increase in blood pressure in the pulmonary vasculature causing less blood to reach the lungs. The right side of the heart has to pump harder to compensate for this, which can ultimately lead to right heart failure. With vaso-occlusion being the major complication in sickle cell disease and affecting many tissues and organs like the lungs, pulmonary hypertension has become a major risk factor in these patients, especially in children. Most research has been with adults so the seriousness of this condition is still being discovered in children. This review will cover the important aspects of both sickle cell disease and pulmonary hypertension then discuss how pulmonary hypertension has become a significant risk factor for death in those with sickle cell disease. From there the importance of this finding in children will be discussed and a plan for future endeavors will be proposed