Sickle cell disease and ventricular myocardial strain: A systematic review

Abstract

Cardiac disease is the primary cause of death in sickle cell disease (SCD). Cardiac abnormalities begin in childhood and progress throughout life. Right and left ventricular (RV, LV) myocardial strain are early markers of systolic dysfunction but are not well investigated among individuals with SCD. The objectives of this review were to (1) identify all published studies that have evaluated ventricular myocardial strain, (2) summarize their values, and (3) compare findings with those obtained from controls. From search results of four electronic databases—Medline, Embase, Scopus, and Web of Science—42 potential articles were identified, of which 18 articles and 17 studies met eligibility criteria for inclusion. The evaluated studies demonstrate that RV and LV myocardial strain are generally abnormal in individuals with SCD compared with controls, despite having normal ejection/shortening fraction. Myocardial strain has been inconsistently evaluated in this population and should be considered any time an echocardiogram is performed.

Rapid MRI Assessment of Long‐Axis Strain to Indicate Systolic Dysfunction in Patients With Sickle Cell Disease

“…Cardiomyopathy in SCD represents a special form that is characterized by signs of diastolic dysfunction early in life, whereas manifestation of systolic dysfunction becomes evident later. 16 In addition, LV dilatation and hypertrophy are typical features of myocardial remodeling due to hyperdynamic physiology in chronic anemia. 17,18 In SCD patients, most studies dealing with cardiac function refer to echocardiographic parameters.…”

“…20 Instead, myocardial strain assessed by echocardiography was generally abnormal and an early marker of systolic dysfunction in SCD patients with normal LVEF. 16 With the aid of a mathematical model, Dumesnil et al showed that longitudinally oriented subendocardial fiber shortening is translated into LV long-axis function, which may better reflect the contractile function of the myocardium than circumferentially dependent parameters as used in LVEF calculation. 21 This theory was supported in our study by the fact that identification of SCD patients with cardiac symptoms was more reliable with LAS compared to LVEF.…”

“…In SCD patients, a meta‐analysis reported no differences in load‐dependent systolic indices, such as LVEF, between patients and controls 20 . Instead, myocardial strain assessed by echocardiography was generally abnormal and an early marker of systolic dysfunction in SCD patients with normal LVEF 16 . With the aid of a mathematical model, Dumesnil et al showed that longitudinally oriented subendocardial fiber shortening is translated into LV long‐axis function, which may better reflect the contractile function of the myocardium than circumferentially dependent parameters as used in LVEF calculation 21 .…”

Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy

“… 28 Myocardial strain, an early marker of systolic dysfunction, has also been inconsistently evaluated in this population. 29 Therefore, screening echocardiogram should include additional parameters such as measures of LV diastolic function, left atrial volume, and myocardial strain to improve the accuracy of noninvasive screening for cardiac complications in SCA.…”

Biomarcadores cardíacos em pacientes adultos com doenças falciformes

“…A técnica também pode ser usada no strain de átrio e no cálculo de trabalho miocárdico 131 . Há diversos trabalhos em doença falciforme utilizando essa técnica, com uma meta-análise de 2021 mostrando que houve valores reduzidos de GLS tanto para ventrículo esquerdo quanto para o direito quando comparado com controles normais, mas notando grande sobreposição com valores normais 132 . Os dois artigos com maior casuística não mostraram diferença com os valores dos controles, ressaltando a necessidade de mais trabalhos nessa área 133,134 .…”