Sickle cell disease is an inherited disorder in which microvascular occlusion causes complications across multiple organ systems. Acute myocardial infarction is increasingly recognized as a feature of sickle cell disease. Acute myocardial infarction is often clinically overshadowed by more substantial presentations of vasoocclusion such as musculoskeletal pain. However, one-third of adults with sickle cell disease suffer from left ventricular dysfunction that may be related to recurrent micro-injury, which may have begun years prior to a heart failure presentation. Pathological studies also have demonstrated degenerative myocardial changes, fibrosis, healed infarcts, and clogging of intramural (micro) coronary vessels by sickle cell aggregates. Quantification of myocardial iron deposition is the most recognizable use of cardiac magnetic resonance imaging (MRI) in sickle cell disease patients. The other unique advantage of cardiac MRI in sickle cell disease is its ability to assess myocardial tissue characteristics, making it an appealing modality to provide valuable data that could not be acquired previously without tissue biopsy.
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-Shawn Lee, MD, Scott Kaplin, MD, Jacqueline Tamis-Holland, MD, Soheila Talebi, MD
