CAJ | 학술논문

目的分析心肌淀粉样变性患者临床表现、诊断治疗及预后情况.方法回顾性分析1995-2005年18例经心内膜心肌活检(EMB)确诊为心肌淀粉样变性患者的临床特征及预后情况.结果 18例心肌淀粉样变性患者均存在心脏舒张功能下降,其中12例二尖瓣舒张早期血流峰速度/舒张晚期血流峰速度(E/A) ＞2.0,且心室舒张早期充盈减速时间(DT) ＜150 ms;12例存在心脏收缩功能受损,左室射血分数(LVEF) ＜50％,13例纽约心脏病学会心功能分级(NYHA)Ⅲ、Ⅳ级.18例心肌淀粉样变性患者1、2和5年生存率分别为67％、44％和17％.Kaplan-Meier分析显示NYHA＞Ⅱ级、E/A ＞2.0且心室舒张早期充盈减速时间(DT)＜ 150 ms与病死率增加相关(log-rank P=0.026和0.001).心力衰竭前接受化疗者有生存期延长趋势.结论随着发病时间延长,患者生存率逐渐下降,病死率上升.NYHA＞Ⅱ级、E/A ＞2.0且DT＜ 150 ms与病死率增加相关.
목적 분석심기정분양변성환자림상표현、진단치료급예후정황.방법 회고성분석1995-2005년18례경심내막심기활검(EMB)학진위심기정분양변성환자적림상특정급예후정황.결과 18례심기정분양변성환자균존재심장서장공능하강,기중12례이첨판서장조기혈류봉속도/서장만기혈류봉속도(E/A) ＞2.0,차심실서장조기충영감속시간(DT) ＜150 ms;12례존재심장수축공능수손,좌실사혈분수(LVEF) ＜50％,13례뉴약심장병학회심공능분급(NYHA)Ⅲ、Ⅳ급.18례심기정분양변성환자1、2화5년생존솔분별위67％、44％화17％.Kaplan-Meier분석현시NYHA＞Ⅱ급、E/A ＞2.0차심실서장조기충영감속시간(DT)＜ 150 ms여병사솔증가상관(log-rank P=0.026화0.001).심력쇠갈전접수화료자유생존기연장추세.결론 수착발병시간연장,환자생존솔축점하강,병사솔상승.NYHA＞Ⅱ급、E/A ＞2.0차DT＜ 150 ms여병사솔증가상관.
Objective To analyze the clinical characteristics,diagnosis,treatment and outcome of patients with cardiac amyloidosis (CA).Methods Clinical data from 18 patients diagnosed as CA by endomyocardial biopsy (EMB) from 1995 to 2005 were retrospectively analyzed.Results Among the 18 patients with CA,all patients had reduced diastolic dysfunction; 12 had mitral valve early diastolic blood flow peak velocity/late diastolic blood flow peak velocity (E/A) ＞ 2.0 and ventricular diastolic early filling deceleration time (DT) ＜ 150 ms; 12 had left ventricular ejection fraction (LVEF) ＜50％ ; and 13 had New York Heart Association (NYHA) classification Ⅲ or Ⅳ.The 1-year,3-year and 5-year survival rates of 18 patients with CA were 67％,44％ and 17％,respectively.Kaplan-Meier analysis showed,NYHA functional class ＞ Ⅱ,E/A ＞ 2.0 and DT ＜ 150 ms were associated with increased mortality (log-rank statistic P =0.026 and 0.001,respectively).CA patients with chemotherapy before heart failure were associated with decreased mortality and extend survival.Conclusions The mortality rate goes up and survival rate gradually descends as prolonged onset time.NYHA functional class ＞ Ⅱ and E/A ＞ 2.0 (DT ＜150 ms) are associated with mortality.