Sickle cell disease (SCD) is a frequent indication for chronic transfusion, which can cause iron overload. Excess iron often affects the liver, but not the heart in SCD. Magnetic resonance (MR) is recommended to detect myocardial iron overload (MIO) but its elevated cost requires optimized indication. We aimed to compile all published data on MIO in SCD upon the description of a fatal case of severe MIO in our institution, and to determine associated risk factors. We performed a systematic review using the PRISMA guidelines in two databases (PubMed and Web of Science). Inclusion criteria were publication in English, patients diagnosed with SCD, and reporting ferritin and MIO by MR. Twenty publications reported on 865 SCD adult and pediatric patients, with at least 10 other cases of MIO. The prevalence of MIO in chronically transfused SCD patients can be estimated to be 3% or less, and is associated with high transfusion burden, top-up transfusions, and low adherence to iron chelation. Cardiac siderosis in SCD is rarely reported, and increased awareness with better use of the available screening tools are necessary. Prospective studies should define the recommended chelation regimens depending on the severity of MIO.

镰状细胞病（SCD）是慢性输血的常见指征，可能导致铁超负荷。在SCD中，铁过多通常会影响肝脏，但不会影响心脏。建议使用磁共振（MR）来检测心肌铁超负荷（MIO），但其成本升高需要优化适应症。我们的目的是在描述本机构中严重MIO的致命病例时，汇编SCD中所有有关MIO的已发布数据，并确定相关的风险因素。我们使用PRISMA指南在两个数据库（PubMed和Web of Science）中进行了系统的审查。纳入标准以英文发表，诊断为SCD的患者以及通过MR报告铁蛋白和MIO的患者。二十篇出版物报道了865名SCD成年和小儿患者，以及至少10例其他MIO病例。慢性输血的SCD患者中MIO的患病率估计为3％或更低，并且与高输血负担，充血输血和对铁螯合的依从性低相关。很少有关于SCD中的心脏铁屑病的报道，因此有必要通过更好地使用可用的筛查工具来提高意识。前瞻性研究应根据MIO的严重程度确定推荐的螯合方案。