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題名 | Restrictive Cardiomyopathy in a Patient with Primary (AL) Cardiac Amyloidosis: Should Heart Transplantation be Performed﹖-- A Case Report and Review=原發性心肌類澱粉症之限制型心肌病變者: 是否應該考慮心臟移植﹖--一病例報告及文獻回顧 |
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作者 | 黃建銘; 殷偉賢; 任勗龍; 楊茂勳; Huang, Chien-ming; Yin, Wei-hsian; Jen, Hsu-lung; Young, Mason-shing; |
期刊 | Acta Cardiologica Sinica |
出版日期 | 19980400、19980500、19980600 |
卷期 | 14:2 民87.04-06 |
頁次 | 頁76-81 |
分類號 | 415.3165 |
語文 | eng |
關鍵詞 | 心肌類澱粉沉著症; 限制型心肌病變; 心臟移植; Cardiac amyloidosis; Cardiomyopathy; Cardiac transplantation; |
中文摘要 | 背景:原發性心肌類澱粉沉著症通常在起始症狀發生後兩年內會致死。因為類澱粉質很可能會再度沉著在被移植的心臟,因此,類澱粉症者過去一直被認為不得做心臟移植。然而,有些醫學中心的成功移植的案例,則有效改善類澱粉症患者的預後。 方法與結果:一名50歲男性,因暈倒及猝死且心電圖類似廣泛性陳舊心肌梗塞以及完全性房室傳導阻斷住院,經証實為原發性心肌類澱粉沉著症。由於患者心臟以外的器官並無明顯類澱粉沉著之証據,然而,其心衰竭的症狀卻與日劇增且呈現嚴重之限制型心肌病變症候,因預期內科治療必定失敗,而謹慎考慮心臟移植治療之可行性。可惜患者在心臟移植前,心衰竭再度復發而導致死亡,從發病後僅存活18個月。 結論:這個案例主要在闡明原發性心肌類澱粉症的自然病史,從國外文獻回顧知,謹慎篩選病患是心臟移植成功的關鍵,心臟移植有可能改善原發性心肌類澱粉症患者的預後。 |
英文摘要 | Background: Amyloid heart disease causes patient death shortly after the onset of symptoms. It has been considered a theoretical contraindication for cardiac transplantation because of its potential for recurrence of amyloid deposition in the allograft. Despite these concerns, several centers have performed heart transplantation for amyloidosis in a limited number of cases. Methods and Results: A 50 year-old man., whose primary cardiac amyloidosis manifested as an old extensive anterior wall myocardial infarction, developed complete heart block. progressively worsening heart failure and sudden death. He had survived for 18 months by medical therapy after the initial symptoms and died finally in refractory heart failure. Conclusions: Based on data from several centers, for some patients with amyloidosis, cardiac transplantation may be considered, with good intermediate-term results. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。