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.
Left atrial (LA) dilation is a common indicator of diastolic dysfunction, and its analysis through a volume calculation reflects the cumulative effects of the left ventricular (LV) filling pressures. However, an increase in LA volume is not exclusive to diastolic dysfunction, which has also been observed in other clinical conditions. Thus, the evaluation of the LA strain enables a functional study of this chamber, adding to the morphological analysis through the volume calculation.The LA strain, measured using the speckle tracking technique, brings information on the reservoir, conduction, and contractile functions of the LA, and is related to the LV function. Moreover, the changes in the LA strain precede the volumetric changes by nearly a decade, and correlate inversely with the degree of LA fibrosisthis has an important relationship with the diastolic dysfunction and its grading system. Albeit insufficient to explain its totality, LA fibrosis can partially justify the functional changes of this heart chamber and can favor the use of this variable as a complement to the current protocols for the analysis of the diastolic function.Although further study is still warranted to establish other clinical applications, the LA strain stands out in the analysis of diastolic dysfunction and can be considered ready to use, offering a great potential to improve the evaluation of the overall cardiac function.
A doença falciforme (DF) notabiliza-se como problema global de saúde pública, caracterizando-se pela mudança da hemácia para a forma de foice, além da anemia crônica verifica-se alteração da reologia dos glóbulos vermelhos, ocasionando um cenário deinflamação e estresse oxidativo, fazendo da DF uma enfermidade multissistêmica. O débito cardíaco (DC) encontra-se elevado, acarretando aumento das câmaras cardíacas globalmente e hipertrofia miocárdica tipo excêntrica. Essas alterações cardíacas eram atribuídas apenas a reações adaptativas ao estado anêmico crônico. Estudos recentes reconhecem de maneira patente aassociação com hipertensão pulmonar (HP), disfunção diastólica do ventrículo esquerdo, arritmias e morte súbita; e, nesse contexto, surge recentemente a hipótese da existência de uma cardiomiopatia falcêmica, caracterizada por disfunção diastólica e fisiologia restritiva. O ecocardiograma configura como instrumento fundamental na determinação dos volumes cavitários, da função diastólica e da estimativa da pressão pulmonar, e constitui recurso valioso no diagnóstico e na condução terapêutica na síndrome torácica aguda. A utilização do strain miocárdico, as variáveis rotacionais, o trabalho miocárdico e a ecocardiografia 3D podem ser utilizados na tentativa de auxiliar a identificação precoce dos pacientes que estão sob maior risco de desenvolverem complicações e morte relacionada à DF.
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