Ventricular global longitudinal strain is altered in children with sickle cell disease

Br J Haematol. 2018 Dec;183(5):796-806. doi: 10.1111/bjh.15607. Epub 2018 Nov 19.


Cardiac disease is the primary cause of death in sickle cell disease (SCD). Right and left ventricular global longitudinal strain (RVGLS, LVGLS) are early markers of systolic dysfunction but are not well investigated among children with SCD. One hundred and forty-three patients with HbSS or HbSβ0 -thalassaemia (median age 11 years, range 5-19 years) and 71 controls matched for age and sex were compared. RVGLS and LVGLS were measured and compared with conventional measures of echocardiography and markers of haemolysis and inflammation. RVGLS was higher in children with SCD than in controls (-25·72% ± 3·45% vs. -24·54% ± 2·41%, P = 0·005); LVGLS was not different. RVGLS decreased with older age in children with SCD (ρ = 0·338, P < 0·001) but not among controls. Decreased RVGLS was associated with increased left atrial end diastolic volume (ρ = 0·181, P = 0·04); RVGLS increased with cardiac output (r = -0·279, P = 0·01). RVGLS and LVGLS were not associated with disease-modifying therapies, degree of anaemia or haemolysis markers. Elevated RVGLS may indicate an early RV compensatory mechanism in response to upstream myocardial insults and elevated cardiac output. Global longitudinal strain may serve as an early marker of altered myocardial function in children with SCD.

Trial registration: NCT00842621.

Keywords: cardiomyopathy; global longitudinal strain; heart; sickle cell disease; speckle-tracking; strain.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / physiopathology
  • Cardiomyopathies / diagnosis
  • Cardiomyopathies / etiology
  • Cardiomyopathies / physiopathology
  • Case-Control Studies
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Echocardiography
  • Female
  • Heart / physiology*
  • Heart Ventricles
  • Humans
  • Male
  • Stress, Physiological / physiology
  • Stroke Volume / physiology
  • Ventricular Dysfunction, Left / diagnosis
  • Ventricular Dysfunction, Left / etiology*
  • Ventricular Dysfunction, Left / physiopathology
  • Young Adult

Associated data