Sickle cell disease and ventricular myocardial strain: A systematic review

Area of Science:

• Cardiology
• Hematology
• Medical Imaging

Background:

• Cardiac disease is the leading cause of mortality in sickle cell disease (SCD).
• Cardiac abnormalities manifest early in childhood and worsen over time in individuals with SCD.
• Ventricular myocardial strain (right and left) are sensitive indicators of systolic dysfunction, yet their role in SCD remains under-researched.

Purpose of the Study:

• To systematically identify and review studies assessing ventricular myocardial strain in individuals with SCD.
• To summarize reported values of ventricular myocardial strain in SCD.
• To compare these findings against control populations.

Main Methods:

• A comprehensive literature search was conducted across four major electronic databases: Medline, Embase, Scopus, and Web of Science.
• Studies were screened for eligibility, with 18 articles and 17 distinct studies meeting the inclusion criteria.
• Data on ventricular myocardial strain from included studies were extracted and synthesized.

Main Results:

• The review found that both right and left ventricular myocardial strain are generally abnormal in individuals with SCD when compared to healthy controls.
• These abnormalities in myocardial strain were observed even in patients with a normal ejection fraction or shortening fraction.
• The evaluation of myocardial strain in the SCD population has been inconsistent across studies.

Conclusions:

• Ventricular myocardial strain is frequently impaired in individuals with sickle cell disease, indicating subclinical systolic dysfunction.
• Myocardial strain assessment should be considered a valuable tool during echocardiography in patients with SCD.
• Further consistent evaluation of myocardial strain is warranted to better understand and manage cardiac complications in SCD.