Cardiovascular function and dysfunction in sickle cell anemia.

The 100th anniversary of the discovery of sickle cell anemia (SCA) as a distinct clinical entity by James B. Herrick in 1910 will soon be a reality. SCA continues to present opportunities for elemental observations of basic science and pathophysiologic clinical mechanisms-in particular, those associated with cardiopulmonary and circulatory disorders. Data indicate that cardiomegaly results from increased work caused by the anemia and that myocardial ischemia may result from the combined effects of severe anemia, microthrombi, and increased blood viscosity producing myocardial dysfunction, scarring, and elevated filling pressures. Sudden death has resulted from frank myocardial infarction and ischemia-induced rhythm disturbances. Myocardial injury may also be associated with bone marrow embolism. Mortality risk factors include systemic hypertension, pulmonary hypertension, and possibly subclinical electrical instability.