Wiernek Szymon L, Jiang Bo, Gustafson Gregory M, Dai Xuming
Division of Cardiology, McAllister Heart Institute, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, United States.
Division of Cardiology, Lang Research Center, New York Presbyterian Medical Group - Queens Hospital, Flushing, NY 11355, United States.
World J Cardiol. 2018 Dec 26;10(12):254-266. doi: 10.4330/wjc.v10.i12.254.
Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder that essentially can affect any organ in the human body. The hallmark of the pathogenesis in TTP is the large von Willebrand factor multimers on platelet-mediated micro-thrombi formation, leading to microvascular thrombosis. Autopsy studies showed that cardiac arrest and myocardial infarction are the most common immediate causes of death in these patients. Clinical manifestations of cardiac involvement in TTP vary dramatically, from asymptomatic elevation of cardiac biomarkers, to heart failure, MI and sudden cardiac death. There is limited knowledge about optimal cardiac evaluation and management in patients with TTP. The absence of typical cardiac symptoms, combined with complicated multi-organ involvement in TTP, may contribute to the under-utilization of cardiac evaluation and treatment. Prompt diagnosis and timely initiation of effective therapy could be critically important in selected cases. Based on our experience and this review of the literature, we developed several recommendations for focused cardiac evaluation for patients with acute TTP: (1) patients with suspected or confirmed TTP should be screened for the potential presence of cardiac involvement with detailed history and physical, electrocardiogram and cardiac enzymes; (2) clinical deterioration of TTP patients warrants immediate cardiac reevaluation; (3) TTP patients with clinical evidence of cardiac involvement should be monitored for telemetry, cardiac biomarkers and evaluated with transthoracic echocardiography. These patients require urgent targeted TTP treatment as well as cardiac-specific treatment. Aspirin therapy is indicated for all TTP patients. Since epicardial coronary artery involvement is rare, cardiac catheterization is usually not required, given the high risk for hemorrhage and kidney injury; (4) we recommend evidence-based medical therapy for ischemic symptoms and heart failure. TTP patients with evidence of cardiac involvement would also benefit from routine cardiology follow up during remission.
血栓性血小板减少性紫癜（TTP）是一种多系统疾病，实际上可累及人体的任何器官。TTP发病机制的标志是血小板介导的微血管血栓形成过程中出现大量血管性血友病因子多聚体，从而导致微血管血栓形成。尸检研究表明，心脏骤停和心肌梗死是这些患者最常见的直接死亡原因。TTP中心脏受累的临床表现差异很大，从心脏生物标志物无症状升高到心力衰竭、心肌梗死和心源性猝死。关于TTP患者最佳心脏评估和管理的知识有限。缺乏典型的心脏症状，再加上TTP复杂的多器官受累，可能导致心脏评估和治疗的使用不足。在某些情况下，及时诊断并及时开始有效治疗可能至关重要。基于我们的经验和对文献的综述，我们针对急性TTP患者的重点心脏评估提出了以下几条建议：（1）对于疑似或确诊TTP的患者，应通过详细的病史、体格检查、心电图和心脏酶检查筛查是否存在心脏受累的可能性；（2）TTP患者的临床病情恶化需要立即进行心脏重新评估；（3）有心脏受累临床证据的TTP患者应进行心电监测、检测心脏生物标志物，并通过经胸超声心动图进行评估。这些患者需要紧急进行针对性的TTP治疗以及心脏特异性治疗。所有TTP患者均需使用阿司匹林治疗。由于心外膜冠状动脉受累罕见，考虑到出血和肾损伤风险高，通常不需要进行心脏导管检查；（4）对于缺血症状和心力衰竭，我们建议采用循证医学治疗。有心脏受累证据的TTP患者在缓解期也将受益于常规的心脏病学随访。
