Intracardiac or intrapulmonary shunts were present in at least 35% of adults with homozygous sickle cell disease followed in an outpatient clinic

Sickle cell disease (SCD) is an inherited anemia in which the beta chain of adult hemoglobin (Hb) is mutated (‘sickle’, HbS). Deoxygenated HbS (deoxyHbS) polymerizes within red blood cells (RBCs), resulting in membrane damage and hemolysis, which is toxic to the vascular endothelium. SCD is