一篇关于浸润心脏的淀粉样变性病的综述。
A review of the amyloidoses that infiltrate the heart.
作者信息
McCarthy R E, Kasper E K
机构信息
Division of Cardiology, Johns Hopkins Hospital, Baltimore, MD 21205, USA.
出版信息
Clin Cardiol. 1998 Aug;21(8):547-52. doi: 10.1002/clc.4960210804.
Abstract
Primary amyloidosis, systemic senile amyloidosis, isolated atrial amyloidosis, and transthyretin isoleucine 122 amyloidosis frequently involve the heart. Amyloid fibrils infiltrate the myocardium, impairing ventricular contraction and relaxation. The clinical manifestations of cardiac infiltration in these disorders are protean, though congestive heart failure and arrhythmias are most common. Treatment of cardiac amyloidosis is directed at the underlying cause and at relief of symptoms. Heart transplantation is not a viable treatment option for patients with primary amyloidosis; its role in the other amyloidoses has not been established. The prognosis of patients with cardiac amyloidosis varies and is largely determined by the underlying disorder responsible for amyloid infiltration.
摘要
原发性淀粉样变性、全身性老年性淀粉样变性、孤立性心房淀粉样变性和转甲状腺素蛋白异亮氨酸122淀粉样变性常累及心脏。淀粉样纤维浸润心肌，损害心室收缩和舒张功能。这些疾病中心脏浸润的临床表现多种多样，尽管充血性心力衰竭和心律失常最为常见。心脏淀粉样变性的治疗针对潜在病因和缓解症状。心脏移植对原发性淀粉样变性患者不是一个可行的治疗选择；其在其他淀粉样变性中的作用尚未明确。心脏淀粉样变性患者的预后各不相同，很大程度上取决于导致淀粉样浸润的潜在疾病。
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