62例心肌淀粉样变性患者的临床特点及预后分析

Clinical characteristics and survival of 62 patients with cardiac amyloidosis

  • 摘要: 目的 探讨心肌淀粉样变性(cardiac amyloidosis,CA)患者的临床特征及预后。 方法 回顾性分析解放军总医院经病理活检确诊为CA的62例患者临床资料,并采用COX回归模型分析影响CA预后的独立危险因素。 结果 CA患者临床表现多样;心电图(ECG)以肢体导联低电压及胸前导联R波递增不良常见;心脏超声(UCG)示室间隔增厚,限制性舒张功能不全,心肌颗粒样闪光回声增强;心脏核磁(CMR)为双房扩大,左室向心性肥厚,舒张功能受限,延迟钆现象呈不同程度延迟强化。COX回归分析,影响CA预后的因素为NYHA分级、二尖瓣舒张早期血流峰速/舒张晚期血流峰速(E/A)> 1.5且心室舒张早期充盈减速时间(DT)< 150 ms。 结论 CA临床表现多样缺乏特异性,心外组织活检结合临床表现、ECG、UCG及CMR可确诊。CA预后不佳,NYHA分级、E/A> 1.5且DT< 150 ms是CA预后的影响因素。

     

    Abstract: Objective To study the clinical characteristics and prognosis of patients with cardiac amyloidosis(CA). Methods Clinical data about 62 CA patients admitted to our hospital were retrospectively analyzed.Independent risk factors for the prognosis of CA patients were analyzed according to the COX regression model. Results The CA patients presented a variety of clinical manifestations.Low voltage in limb leads and poor R wave progression in precordial leads were frequently observed on ECG.UCG showed thicker interventricular septum, concentric left ventricular hypertrophy and diastolic dysfunction, enhanced echo of myocardial granules, And CMR reveal different patterns of abnormal late gadolinium enhancement(LGE).Cox regression analysis showed that NYHA classification, E/A> 1.5 and DT< 150 ms were the influencing factors for the prognosis of CA patients. Conclusion The clinical manifestations of CA patients lack of specificity.CA can be diagnosed by biopsy of exocardial tissue in combination with ECG, UCG and CMR.The prognosis of CA patients is poor.NYHA classification, E/A> 1.5 and DT< 150 ms are the influencing factors for the prognosis of CA patients.

     

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