João Carlos Moron Saes Braga et al. Fábio Villaça Guimarães Filho Alexandre Rodrigues Raphael Aparecido Barreto
Abstract
Sickle cell disease (SCD) is recognized as a global problem in public health, characterized by the alteration in the red blood cells to the sickle form. Moreover, chronic anemia can also be observed through the change in the rheology of the red blood cells, leading to a scenario of inflammation and oxidative stress, making SCD a multisystem disease.
Cardiac output (CO) proved to be high, leading to an overall increase in the heart chambers and an eccentric myocardial hypertrophy. These heart alterations were attributed only to adaptive reactions to chronic anemia. Recent studies have more clearly recognized an association with pulmonary hypertension (PH), left ventricular diastolic dysfunction, arrhythmias, and sudden death. Moreover, what has also arisen in this context is the hypothesis of the existence of a sickle-cell cardiomyopathy, characterized by diastolic dysfunction and restrictive physiology. The echocardiogram represents a key tool in determining cavitary volumes, diastolic dysfunction, and the estimation of pulmonary pressure, as well as constitutes a valuable resource in the diagnosis and therapeutic treatment of acute chest syndrome. The myocardial strain, rotational variables, myocardial work, and 3D echocardiography can be applied in an attempt to aid in the early detection of patients who are at a higher risk of developing complications and evolving to death related to SCD.