心肌淀粉样变性预后影响因素分析

An analysis of factors affecting the prognosis of patients with cardiac amyloidosis

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）〈150ms；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）IlI、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〉1I级、E／A〉2．0且心室舒张早期充盈减速时间（DT）〈150ms与病死率增加相关（10g—rankP=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〉1I级、E／A〉2．0且DT〈150rtls与病死率增加相关。

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 endomyoeardial 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 m 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 〉 1I , 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 〉 11 and E/A 〉 2. 0 （ DT 〈 150 ms） are associated with mortality.