镰状细胞病的心血管并发症:机制、诊断与治疗进展
本文综述镰状细胞病(SCD)心血管并发症的病理机制、诊断工具与治疗进展,涵盖心肌功能障碍、肺动脉高压、心脏铁过载、心律失常、心肌梗死、卒中和猝死。文章强调综合多模态影像与SCD特异性管理策略,以促进早期识别和个体化治疗。
要点
- SCD心血管并发症表现多样,包括心肌功能障碍、肺动脉高压和心律失常等。
- 心脏磁共振T2* mapping、斑点追踪超声和侵入性运动试验有助于识别独特表型。
- 管理需整合羟基脲、输血、抗凝和基因治疗等SCD特异性方法。
核心结论
- 心血管并发症是SCD患者发病和早期死亡的重要驱动因素,但诊断不足且认识不够。
研究设计与样本
- 本综述综合当前知识,涵盖SCD心血管表现、病理机制、诊断工具和管理方法。
主要发现
- SCD心血管表现包括心肌功能障碍、肺动脉高压、心脏铁过载、心律失常、心肌梗死、卒中和猝死。
- 新兴诊断工具如心脏磁共振T2* mapping、细胞外容积序列、斑点追踪超声和侵入性运动试验可揭示限制性心肌病合并高输出量心衰的独特表型。
关键数值与推荐
- 文中提出风险分层和疾病监测的实用算法,并汇总基于证据的SCD特异性管理方法。
临床怎么用
- 临床应整合多模态影像、更新指南推荐和近期临床见解,以支持早期识别、个体化治疗和改善心血管结局。
以上解读依据论文摘要(全文未开放获取或许可不允许翻译转载)。
原文
Cardiovascular complications are increasingly recognised as a major driver of morbidity and early mortality in patients with sickle cell disease (SCD), yet they remain underdiagnosed and underappreciated. This contemporary review synthesises current knowledge across a spectrum of cardiovascular manifestations-including myocardial dysfunction, pulmonary hypertension, cardiac iron overload, arrhythmias, myocardial infarction, stroke and sudden death-with emphasis on their unique pathophysiological mechanisms in SCD. We highlight emerging diagnostic tools such as cardiac magnetic resonance with T2* mapping and extracellular volume sequences, speckle-tracking echocardiography and invasive exercise testing, which can revealing a distinct phenotype combining restrictive cardiomyopathy and high-output heart failure. Practical algorithms for risk stratification and disease monitoring are presented alongside evidence-based and SCD-specific management approaches, including the role of hydroxyurea, transfusions, anticoagulation and gene therapy. By integrating multimodality imaging, updated guideline recommendations and recent clinical insights, this review provides a comprehensive resource to support early recognition, personalised therapy and improved cardiovascular outcomes in SCD.
引用
- Haroun E, Agrawal A, Arockiam AD, et al. Cardiovascular disease in Sickle cell: mechanisms, diagnostics and therapeutic advances. Heart. 2026. PMID: 40780824. doi:10.1136/heartjnl-2025-325837
供执业医师工作参考,不替代临床判断;数值与分级以指南原文为准。