TO THE EDITOR:
The cardiomyopathy of sickle cell anemia (SCA) is characterized by ventricular diastolic dysfunction that is superimposed on an anemia-related hyperdynamic state.1-3 Diastolic dysfunction has been recognized before and shown to portend early mortality in SCA,4 but the underlying causes of diastolic dysfunction were not understood.5 We recently showed that diffuse myocardial fibrosis is a common, if not defining, feature of the cardiomyopathy of SCA that is strongly associated with diastolic dysfunction.6,7 Every participant with SCA (N = 25) who underwent cardiac magnetic resonance imaging (CMR) had marked diffuse myocardial fibrosis.6 Three quarters were receiving hydroxyurea at the time of their imaging,6 which had been first prescribed at a mean age of 11.3 years (range, 4.1-25.6 years). By design, individuals receiving chronic transfusions were excluded.
Given that fibrosis was universally present in...
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