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.