Sickle cell disease and ventricular myocardial strain: A systematic review

Pediatr Blood Cancer. 2021 Jun;68(6):e28973. doi: 10.1002/pbc.28973. Epub 2021 Mar 19.

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.

Keywords: global longitudinal strain; heart; sickle cell anemia; speckle tracking; strain.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Systematic Review

MeSH terms

  • Adult
  • Anemia, Sickle Cell / pathology*
  • Cardiomyopathies / pathology*
  • Child
  • Echocardiography
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / pathology
  • Humans
  • Infant
  • Middle Aged
  • Stroke Volume / physiology
  • Ventricular Dysfunction, Left / pathology*
  • Ventricular Dysfunction, Right / pathology*