Longitudinal changes and predictors of cardiac extracellular volume fraction in sickle cell anemia.

ABSTRACT: Diffuse myocardial fibrosis is a key feature of the cardiomyopathy of sickle cell anemia (SCA) that is linked to diastolic dysfunction. Cardiac MRI (CMR)-derived extracellular volume fraction (ECV) is a quantitative biomarker of myocardial fibrosis that is elevated in SCA. The stability of ECV in SCA and its clinical associations over time are unknown. To measure longitudinal changes in ECV, without specific intervention, and identify correlates of ECV change in patients with SCA, we conducted a prospective, 2-year study involving annual CMR, echocardiography, and laboratory assessments in 24 patients with SCA (mean age 21.4±10 years). ECV was calculated using T1 mapping pre- and post-gadolinium, and extracellular matrix and cellular volumes were calculated to account for relative changes.

SUBMITTER: Niss O 

PROVIDER: S-EPMC12652408 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature