解答心脏淀粉样变性诊断与管理中遇到的常见问题。
Addressing Common Questions Encountered in the Diagnosis and Management of Cardiac Amyloidosis.
作者信息
Maurer Mathew S, Elliott Perry, Comenzo Raymond, Semigran Marc, Rapezzi Claudio
机构信息
From Columbia University Medical Center, New York, NY (M.S.M.); University College London and St. Bartholomew's Hospital, UK (P.E.); Tufts Medical Center, Boston, MA (R.C.); Massachusetts General Hospital, Harvard University, Boston (M.S.); and Department of Experimental, Diagnostic and Specialty Medicine, Alma Mater Studiorum University of Bologna, Italy (C.R.).
出版信息
Circulation. 2017 Apr 4;135(14):1357-1377. doi: 10.1161/CIRCULATIONAHA.116.024438.
Advances in cardiac imaging have resulted in greater recognition of cardiac amyloidosis in everyday clinical practice, but the diagnosis continues to be made in patients with late-stage disease, suggesting that more needs to be done to improve awareness of its clinical manifestations and the potential of therapeutic intervention to improve prognosis. Light chain cardiac amyloidosis, in particular, if recognized early and treated with targeted plasma cell therapy, can be managed very effectively. For patients with transthyretin amyloidosis, there are numerous therapies that are currently in late-phase clinical trials. In this review, we address common questions encountered in clinical practice regarding etiology, clinical presentation, diagnosis, and management of cardiac amyloidosis, focusing on recent important developments in cardiac imaging and biochemical diagnosis. The aim is to show how a systematic approach to the evaluation of suspected cardiac amyloidosis can impact the prognosis of patients in the modern era.
心脏成像技术的进步使得在日常临床实践中对心脏淀粉样变性有了更深入的认识，但诊断仍多在疾病晚期的患者中进行，这表明仍需采取更多措施来提高对其临床表现以及治疗干预改善预后潜力的认识。特别是轻链型心脏淀粉样变性，如果能早期识别并采用靶向浆细胞疗法进行治疗，是可以得到非常有效的管理的。对于转甲状腺素蛋白淀粉样变性患者，目前有多种疗法正处于晚期临床试验阶段。在本综述中，我们探讨了临床实践中在心脏淀粉样变性的病因、临床表现、诊断和管理方面遇到的常见问题，重点关注心脏成像和生化诊断方面的近期重要进展。目的是展示对疑似心脏淀粉样变性进行系统评估的方法如何影响现代患者的预后。
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