Early initiation of disease-modifying therapy can impede or prevent diffuse myocardial fibrosis in sickle cell anemia

Abstract

Cardiovascular disease is a major cause of mortality in patients with sickle cell disease (SCD). Niss et al previously reported that cardiac magnetic resonance in 25 patients showed universal myocardial fibrosis, which they correlated with increased extracellular volume fraction (ECV). In the current study, they compared patients with SCD who were treated with hydroxyurea or transfusion at age <6 years to a group of patients with SCD without therapy. They documented that patients treated early had ECV levels comparable to normal controls, potentially preventing subsequent myocardial fibrosis.

Figure 1.

Prevention of diffuse myocardial fibrosis in SCA with early initiation of disease-modifying therapy. Patients who had early initiation and uninterrupted use of hydroxyurea or chronic transfusions had significantly less myocardial fibrosis than patients with no early therapy. Mean ECV was 0.30, which was significantly lower than the ECV of individuals with SCA who have not received early uninterrupted therapy (0.44, P = .002) and not different from normal controls (0.26 ± 0.02; P = .11).