title: Echocardiographic and biohumoral characteristics in patients with AL and TTR amyloidosis at diagnosis.
abstract: BACKGROUND
  Few studies have analyzed the clinical and echocardiographic differences between light-chain (AL) and transthyretin (TTR) amyloidosis.
  
  
  OBJECTIVE
  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.
  
  
  METHODS
  Seventy-nine patients with AL and 48 patients with TTR amyloidosis were studied.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Intensive Cardiac Care Unit, Heart and Vessel Department, University Hospital Careggi, Florence, Italy; Tuscan Regional Amyloid Center, University Hospital Careggi, Florence, Italy.
authors: Francesco
authors: F
authors: Cappelli
authors: Samuele
authors: S
authors: Baldasseroni
authors: Franco
authors: F
authors: Bergesio
authors: Stefano
authors: S
authors: Perlini
authors: Francesco
authors: F
authors: Salinaro
authors: Luigi
authors: L
authors: Padeletti
authors: Paola
authors: P
authors: Attanà
authors: Alessandro
authors: A
authors: Paoletti Perini
authors: Alberto
authors: A
authors: Moggi Pignone
authors: Elisa
authors: E
authors: Grifoni
authors: Alessia
authors: A
authors: Fabbri
authors: Niccolò
authors: N
authors: Marchionni
authors: Gian Franco
authors: GF
authors: Gensini
authors: Federico
authors: F
authors: Perfetto
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D028227:Amyloid Neuropathies, Familial / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D000686:Amyloidosis / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D015415:Biomarkers / Q000032:analysis / Q000097:blood; D009202:Cardiomyopathies / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology; D003430:Cross-Sectional Studies; D018450:Disease Progression; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D010446:Peptide Fragments / Q000097:blood*; D011228:Prealbumin / Q000235:genetics; D011237:Predictive Value of Tests; D012720:Severity of Illness Index; D019210:Troponin I / Q000097:blood*; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right
chemical_list: D015415:Biomarkers; D007147:Immunoglobulin Light Chains; D010446:Peptide Fragments; D011228:Prealbumin; D019210:Troponin I; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
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