In our latest publication in @BloodJournal, @pigtowner and I report an increase in myocardial extracellular volume (ECV) in patients with #SickleCell disease despite early intervention with disease-modifying therapies ☹️ sciencedirect.com/science/articl…
Increased ECV fraction correlates strongly with histologically quantified myocardial fibrosis👉leading to cardiomyopathy (which is a leading cause of death in individuals with #SickleCell disease).
We used Cardiac MRI to study the individuals with #SickleCell disease who started disease-modifying therapy early in childhood vs later. We found no differences in the ECV (aka myocardial fibrosis) @BloodJournal sciencedirect.com/science/articl… 
Even more concerning is that diffuse myocardial fibrosis is nearly universal in children and young adults with #SickleCell disease, even those patients with normal cardiac size and function.
Our findings suggest that damage to the myocardium occurs before symptoms or clinical markers of cardiac dysfunction and that our current screening protocols may require revision to include earlier cardiac assessments. #SickleCell #StartScreeningEarly sciencedirect.com/science/articl…
@threadreaderapp unroll please 🙏
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