Brunjes Danielle L, Castano Adam, Clemons Autumn, Rubin Jonah, Maurer Mathew S
Division of Cardiology, Department of Medicine, Columbia University Medical Center, New York, New York.
Division of Cardiology, Department of Medicine, Columbia University Medical Center, New York, New York.
J Card Fail. 2016 Dec;22(12):996-1003. doi: 10.1016/j.cardfail.2016.10.008. Epub 2016 Oct 18.
Wild-type transthyretin cardiac amyloidosis (ATTRwt), formerly called senile cardiac amyloidosis (SCA), is almost exclusively a disorder of older adults. As the population ages, the diagnosis of ATTRwt will increase, making it the most common form of cardiac amyloidosis. An important precondition to reduce underdiagnosis and misdiagnosis is to maintain a high index of suspicion for cardiac amyloidosis. Several clues can be gleaned from the clinical history, physical exam, electrocardiography, and noninvasive imaging techniques. Nuclear scintigraphy agents using Tc-phosphate derivatives combined with assessment for monoclonal proteins are eliminating the need for tissue confirmation in ATTR. Morbidity and mortality from ATTRwt cardiac amyloid is high and the emergence of numerous therapies based on a biologic understanding of the pathophysiology of this condition, including drugs to inhibit the synthesis of TTR, stabilize TTR, and degrade or extract amyloid, provides new hope for those afflicted. This review briefly covers the epidemiology, pathophysiology, and clinical manifestations, as well as diagnostic strategies and treatment, of ATTR in older adults.
野生型转甲状腺素蛋白心脏淀粉样变(ATTRwt),以前称为老年心脏淀粉样变(SCA),几乎仅见于老年人。随着人口老龄化,ATTRwt的诊断将会增加,使其成为心脏淀粉样变最常见的形式。减少漏诊和误诊的一个重要前提是对心脏淀粉样变保持高度的怀疑指数。从临床病史、体格检查、心电图和无创成像技术中可以收集到一些线索。使用锝磷酸盐衍生物的核闪烁显像剂结合单克隆蛋白评估正在消除ATTR中组织确认的必要性。ATTRwt心脏淀粉样变的发病率和死亡率很高,基于对这种疾病病理生理学的生物学理解而出现的众多疗法,包括抑制TTR合成、稳定TTR以及降解或提取淀粉样蛋白的药物,为患者带来了新的希望。本综述简要介绍了老年人ATTR的流行病学、病理生理学、临床表现以及诊断策略和治疗方法。