Diffuse myocardial fibrosis occurs in young patients with sickle cell anemia despite early disease-modifying therapy

TO THE EDITOR:

We read with great interest the recent report from Niss et al.¹ Our group has recently reviewed data from a similar population at our institution, which does not support the conclusion of Niss et al that diffuse myocardial fibrosis is prevented by early initiation of disease-modifying therapy (DMT).

Cardiovascular complications are the leading cause of early mortality in patients with sickle cell anemia (SCA).² Yet, the pathobiology of sickle cell cardiomyopathy is incompletely understood and likely multifactorial.³ Chronic anemia leads to global cardiac enlargement and myocardial hypertrophy. Global cardiac chamber dilation is a common feature in patients with SCA, and enlarged left atrial (LA) volume is a marker of diastolic dysfunction in SCA.⁴ Repetitive microvascular ischemic insults and associated reperfusion injury may contribute to diffuse myocardial fibrosis and myocardial remodeling in individuals with SCA....