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首页> 外文期刊>Amyloid: the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis >How to diagnose cardiac amyloidosis early: impact of ECG, tissue Doppler echocardiography, and myocardial biopsy.
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How to diagnose cardiac amyloidosis early: impact of ECG, tissue Doppler echocardiography, and myocardial biopsy.

机译：如何早期诊断心脏淀粉样变性：ECG，组织多普勒超声心动图和心肌活检的影响。

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AIMS: To detect cardiac amyloidosis (CA) earlier, it is inevitable to improve diagnostic strategies. METHODS AND RESULTS: 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. CONCLUSION: 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.
机译：目的：为了及早发现心脏淀粉样变性（CA），改善诊断策略是不可避免的。方法和结果：评价了30例男性患者（63％（n = 19），平均年龄66 +/- 8岁，NYHA 3.0 +）中的心电图，超声心动图（包括组织多普勒成像（TDI）和应变）以及心肌活检的影响。 /-0.5，先前有心肌失代偿的73％），其中我们证明了CA。淀粉样蛋白在偏振光下被苹果绿双折射所证实，而免疫组织化学检查证实了所产生的蛋白质。遗传分析排除了家族性CA。所有患者（AL-lambda（n = 22），AL-kappa（n = 3），老年淀粉样变性病（n = 5））均具有限制性心肌病（RCM），典型TDI和应变参数（E'septal; E）的超声心动图征象。侧向<8 cm / s; E / E'> 8; S'<或= 9 cm / s;总纵向应变（GLS）-7.9 +/- 3.8％）。 63％的患者出现心包积液。 ECG在许多患者中令人怀疑：V（1）-V（4）中的19例（63％）具有低压，23例（77％）的R波减少，两者均占57％。异常情况回顾了0.5-4年。二十例（67％）患者在诊断出CA后232 +/- 268 [2-1020]天时死亡，但在首次出现症状后502 +/- 333 [30-1440]天后死亡。结论：准确的心电图评估，仔细的RCM超声心动图搜索，降低的应变/应变率以及对心肌活检的一般指征和正确分析才能诊断CA。

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