ABSTRACT
BACKGROUND:
Beta thalassemia major (Beta-TM) is an inherited condition which presents at around two years of
life.
Patients with Beta-;TM may develop cardiac
iron toxicity secondary to transfusion dependence. Cardiovascular
magnetic resonance (CMR) T2*, a
technique designed to quantify myocardial
iron deposition, is a driving component of
disease management. A decreased T2* value represents increasing cardiac
iron overload. The clinical manifestation is a decline in ejection fraction (EF). However, there may be early subclinical changes in cardiac function that are not detected by changes in EF. CMR-derived
strain assesses myocardial dysfunction prior to decline in EF. Our primary aim was to assess the correlation between CMR
strain and T2* in the Beta-TM
population.
METHODS:
Circumferential and longitudinal
strain was analyzed. Pearson's correlation was calculated for T2* values and
strain in the Beta-TM
population.
RESULTS:
We identified 49
patients and 18 controls.
Patients with severe
disease (low T2*) were found to have decreased global circumferential
strain (GCS) in comparison to other T2* groups. A correlation was identified between GCS and T2* (r = 0.5; p < 0.01).
CONCLUSION:
CMR-derived
strain can be a clinically useful tool to predict early myocardial dysfunction in Beta-TM.