Department of Cardiology, Heart and Diabetes Center North Rhine-Westphalia, Ruhr University Bochum, Bad Oeynhausen, Germany.
Amyloid. 2010 Mar;17(1):1-9. doi: 10.3109/13506121003619310.
To detect cardiac amyloidosis (CA) earlier, it is inevitable to improve diagnostic strategies.
The impact of ECG, echocardiography including tissue Doppler imaging (TDI) and strain, and myocardial biopsies was evaluated in 30 patients (63% (n = 19) men, mean age 66 +/- 8 years, NYHA 3.0 +/- 0.5, 73% with prior myocardial decompensation), in whom we proved CA. Amyloid was confirmed by apple-green birefringence under polarised light, and the causing protein by immunohistochemical examinations. Genetic analyses excluded familial CA. All patients (AL-lambda (n = 22), AL-kappa (n = 3), senile amyloidosis (n = 5)) had echocardiographic signs of restrictive cardiomyopathy (RCM), typical TDI and strain parameters (E'septal; E' lateral < 8 cm/s; E/E' > 8; S' < or =9 cm/s; global longitudinal strain (GLS) -7.9 +/- 3.8%). Pericardial effusions were present in 63% of patients. ECGs were suspicious in many patients: 19 (63%) had low-voltage, 23 (77%) reduced R waves in V(1)-V(4), and 57% both. Abnormalities, retrospectively had been present for 0.5-4 years. Twenty (67%) patients died 232 +/- 268 [2-1020] days after CA was diagnosed, but 502 +/- 333 [30-1440] days after the first symptom.
Accurate ECG evaluations, careful echocardiographic search for RCM, reduced strain/strain rates, and general indications to myocardial biopsies with correct analyses are needed to diagnose CA.
为了更早地发现心脏淀粉样变性（CA），提高诊断策略是不可避免的。
在 30 名患者（63%（n=19）为男性，平均年龄 66±8 岁，NYHA 3.0±0.5，73%有心肌失代偿史）中评估了心电图、包括组织多普勒成像（TDI）和应变在内的超声心动图以及心肌活检的影响，我们在这些患者中证实了 CA。通过偏光下的苹果绿双折射和免疫组织化学检查来证实淀粉样物质，通过免疫组织化学检查来确定导致该病的蛋白质。遗传分析排除了家族性 CA。所有患者（AL-λ（n=22）、AL-κ（n=3）、老年性淀粉样变性（n=5））均有限制性心肌病（RCM）的超声心动图征象，典型的 TDI 和应变参数（E'间隔；E'侧壁<8cm/s；E/E' > 8；S' <或=9cm/s；整体纵向应变（GLS）-7.9±3.8%）。63%的患者存在心包积液。许多患者的心电图存在可疑情况：19 例（63%）有低电压，23 例（77%）V（1）-V（4）的 R 波降低，57%两者均存在。这些异常，回顾性地存在了 0.5-4 年。20 例（67%）患者在 CA 确诊后 232±268[2-1020]天死亡，但在首次症状后 502±333[30-1440]天死亡。
需要进行准确的心电图评估、仔细的超声心动图寻找 RCM、应变/应变速率降低以及正确分析后进行心肌活检的一般性指征，以诊断 CA。
