title: Role of natriuretic peptide to predict cardiac abnormalities in patients with hereditary transthyretin amyloidosis.
abstract: BACKGROUND
  Familial amyloid polyneuropathy (FAP) mainly targets the peripheral nervous system and heart. Early noninvasive detection of cardiac impairment is critical for therapeutic management.
  
  
  OBJECTIVE
  To assess if amino-terminal pro-brain natriuretic peptide (NT-proBNP) or troponin T (cTnT) can predict echocardiographic left-ventricle (LV) impairment in FAP.
  
  
  METHODS
  Thirty-six asymptomatic carriers and patients with FAP had echocardiographic measurement of left-ventricular (LV) systolic function, hypertrophy (LVH) and estimation of filling pressure (FP).
  
  
  RESULTS
  Overall, median age, NT-proBNP, and LV ejection fraction were, respectively, 59 years (41-74), 323 pg/ml (58-1960), and 60% (51-66). Twelve patients had increased cTnT. Prevalence of ATTR gene mutations was 53% for Val30Met. Four individuals were asymptomatic, 6 patients had isolated neurological clinical signs, and 26 had echo-LV abnormalities. The ROC curve identified NT-proBNP patients with echo-LV abnormalities (area: 0.92; (0.83-0.99), p = 0.001) at a threshold >82 pg/ml with a sensitivity of 92%, and a specificity of 90%. Increased in NT-proBNP occurred in patients with SD and/or LVH with or without increase in FP. Elevated cTnT (>0.01 ng/ml) was only observed in patients with LVH and systolic dysfunction, with or without FP.
  
  
  CONCLUSIONS
  In FAP, NT-proBNP was associated with cardiac impairment suggesting that NT-proBNP could be used in carriers or in FAP patients with only neurologic symptoms for identifying the appropriate time to start cardiac echocardiographic assessment and follow-up. cTnT identified patients with severe cardiac disease.
authors: Department of cardiology .
authors: Thibaud
authors: T
authors: Damy
authors: Jean-François
authors: JF
authors: Deux
authors: Stéphane
authors: S
authors: Moutereau
authors: Soulef
authors: S
authors: Guendouz
authors: Dania
authors: D
authors: Mohty
authors: Stéphane
authors: S
authors: Rappeneau
authors: Aziz
authors: A
authors: Guellich
authors: Luc
authors: L
authors: Hittinger
authors: Sylvain
authors: S
authors: Loric
authors: Jean-Pascal
authors: JP
authors: Lefaucheur
authors: Violaine
authors: V
authors: Plante-Bordeneuve
mesh_terms: D000328:Adult; D000368:Aged; D028227:Amyloid Neuropathies, Familial / Q000097:blood* / Q000473:pathology*; D009202:Cardiomyopathies / Q000097:blood* / Q000473:pathology*; D004452:Echocardiography; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D010446:Peptide Fragments / Q000097:blood*; D020107:Troponin T / Q000097:blood
chemical_list: D010446:Peptide Fragments; D020107:Troponin T; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain