PDF(Pubmed)
Abstract：
UNASSIGNED: Left ventricular systolic dysfunction (LVSD) is an uncommon but life-threatening complication of sickle cell disease (SCD), with poorly characterized aetiology. We present three SCD patients with LVSD due to different underlying mechanisms.
UNASSIGNED: The first case describes rapid deterioration in LV function secondary to severe cardiac iron overload in a 37-year-old female with poor chelation compliance after 10 years of top-up transfusions for SCD. The second case is a severe non-ischaemic dilated cardiomyopathy (DCM) in a 42-year-old SCD patient with longstanding sickle nephropathy and hypertension. The final case demonstrates severe LVSD with large transmural infarcts (ischaemic DCM) in the absence of epicardial coronary disease in a 52-year-old SCD patient.
UNASSIGNED: This case series presents the first attempt to characterize the aetiology of LVSD in SCD. We identified three phenotypes: iron-overload cardiomyopathy, non-ischaemic DCM, and ischaemic DCM. These contrasting cases highlight the significance of understanding the underlying pathology in determining individualized treatment plans for these high-risk patients. We discuss the role of cardiac MRI (CMR) in characterizing LV dysfunction, and we believe that this case series will form the basis of prospective studies to further delineate the pathophysiology of LVSD in SCD.
UNASSIGNED: The first case describes rapid deterioration in LV function secondary to severe cardiac iron overload in a 37-year-old female with poor chelation compliance after 10 years of top-up transfusions for SCD. The second case is a severe non-ischaemic dilated cardiomyopathy (DCM) in a 42-year-old SCD patient with longstanding sickle nephropathy and hypertension. The final case demonstrates severe LVSD with large transmural infarcts (ischaemic DCM) in the absence of epicardial coronary disease in a 52-year-old SCD patient.
UNASSIGNED: This case series presents the first attempt to characterize the aetiology of LVSD in SCD. We identified three phenotypes: iron-overload cardiomyopathy, non-ischaemic DCM, and ischaemic DCM. These contrasting cases highlight the significance of understanding the underlying pathology in determining individualized treatment plans for these high-risk patients. We discuss the role of cardiac MRI (CMR) in characterizing LV dysfunction, and we believe that this case series will form the basis of prospective studies to further delineate the pathophysiology of LVSD in SCD.
摘要：
左心室收缩功能障碍（LVSD）是镰状细胞病（SCD）的一种罕见但危及生命的并发症，病因特征不佳。我们介绍了三名SCD患者由于不同的潜在机制而导致的LVSD。
■第一个病例描述了一名37岁女性，在经过10年的SCD充值输血后，由于严重的心脏铁超负荷，螯合依从性差，左心室功能迅速恶化。第二例是一名42岁的SCD患者的严重非缺血性扩张型心肌病（DCM），患有长期镰状肾病和高血压。最终病例显示，在52岁的SCD患者中，在没有心外膜冠状动脉疾病的情况下，严重的LVSD伴有大的透壁性梗塞（缺血性DCM）。
■本病例系列首次尝试描述SCD中LVSD的病因。我们确定了三种表型：铁过载心肌病，非缺血性DCM，和缺血性DCM。这些对比案例强调了了解潜在病理在确定这些高危患者的个体化治疗计划中的重要性。我们讨论心脏MRI（CMR）在表征LV功能障碍中的作用，我们相信这个病例系列将成为进一步描述SCD中LVSD的病理生理学的前瞻性研究的基础。
■第一个病例描述了一名37岁女性，在经过10年的SCD充值输血后，由于严重的心脏铁超负荷，螯合依从性差，左心室功能迅速恶化。第二例是一名42岁的SCD患者的严重非缺血性扩张型心肌病（DCM），患有长期镰状肾病和高血压。最终病例显示，在52岁的SCD患者中，在没有心外膜冠状动脉疾病的情况下，严重的LVSD伴有大的透壁性梗塞（缺血性DCM）。
■本病例系列首次尝试描述SCD中LVSD的病因。我们确定了三种表型：铁过载心肌病，非缺血性DCM，和缺血性DCM。这些对比案例强调了了解潜在病理在确定这些高危患者的个体化治疗计划中的重要性。我们讨论心脏MRI（CMR）在表征LV功能障碍中的作用，我们相信这个病例系列将成为进一步描述SCD中LVSD的病理生理学的前瞻性研究的基础。
