Cappelli Francesco, Baldasseroni Samuele, Bergesio Franco, Perlini Stefano, Salinaro Francesco, Padeletti Luigi, Attanà Paola, Paoletti Perini Alessandro, Moggi Pignone Alberto, Grifoni Elisa, Fabbri Alessia, Marchionni Niccolò, Gensini Gian Franco, Perfetto Federico
Intensive Cardiac Care Unit, Heart and Vessel Department, University Hospital Careggi, Florence, Italy; Tuscan Regional Amyloid Center, University Hospital Careggi, Florence, Italy.
Clin Cardiol. 2015 Feb;38(2):69-75. doi: 10.1002/clc.22353. Epub 2015 Feb 2.
Few studies have analyzed the clinical and echocardiographic differences between light-chain (AL) and transthyretin (TTR) amyloidosis.
The aim of the present research was to compare, in a real-world setting, the clinical and echocardiographic profiles of these kinds of amyloidosis, at the time of diagnosis, using new-generation echocardiography.
Seventy-nine patients with AL and 48 patients with TTR amyloidosis were studied.
According to the criterion of mean left ventricular (LV) thickness >12 mm, 45 AL (C-AL) and all TTR patients had cardiac amyloidotic involvement, whereas 34 AL patients did not. TTR patients had increased right ventricular (RV) and LV chambers with increased RV and LV wall thickness and reduced LV ejection fraction and fractional shortening. Furthermore, TTR patients showed lower N-terminal pro Brain Natriuretic Peptide concentrations and New York Heart Association functional class when compared with C-AL.
Our data show that at time of first diagnosis, TTR patients have a more advanced amyloidotic involvement of the heart, despite less severe symptoms and biohumoral signs of heart failure. We can hypothesize that we observed different diseases at different stages. In fact, AL amyloidosis is a multiorgan disease with quick progression rate, that becomes rapidly symptomatic, whereas TTR amyloidosis might have a slow progression rate and might remain poorly symptomatic for a greater amount of time.
很少有研究分析轻链（AL）淀粉样变性和转甲状腺素蛋白（TTR）淀粉样变性之间的临床和超声心动图差异。
本研究的目的是在现实环境中，使用新一代超声心动图比较这些类型淀粉样变性在诊断时的临床和超声心动图特征。
对79例AL淀粉样变性患者和48例TTR淀粉样变性患者进行了研究。
根据平均左心室（LV）厚度>12 mm的标准，45例AL（C-AL）患者和所有TTR患者均有心脏淀粉样变性累及，而34例AL患者没有。TTR患者的右心室（RV）和LV腔增大，RV和LV壁厚度增加，LV射血分数和缩短分数降低。此外，与C-AL患者相比，TTR患者的N末端脑钠肽浓度和纽约心脏协会功能分级较低。
我们的数据表明，在首次诊断时，TTR患者的心脏淀粉样变性累及更为严重，尽管心力衰竭的症状和生物体液征象较轻。我们可以推测我们观察到的是处于不同阶段的不同疾病。事实上，AL淀粉样变性是一种多器官疾病，进展速度快，很快就会出现症状，而TTR淀粉样变性可能进展速度慢，在较长时间内症状可能不明显。
