title: Importance of the echocardiographic evaluation of right ventricular function in patients with AL amyloidosis.
abstract: BACKGROUND
  Patients with AL amyloidosis often present with signs of congestive heart failure.
  
  
  OBJECTIVE
  This study was prospectively designed to assess the significance of RV dysfunction in AL amyloidosis.
  
  
  RESULTS
  Seventy-four patients with biopsy proven AL amyloidosis underwent a thorough echocardiographic evaluation. A tricuspid annular plane systolic excursion (TAPSE)<17 mm was taken as marker of RV dysfunction. Plasma NT-proBNP determinations were performed in all cases. RV function was normal in 60 patients and reduced in 14 patients. Patients with RV dysfunction had thicker left ventricular (LV) walls (p<0.01), lower LV end-diastolic volumes (p<0.01), lower LV ejection fraction (p<0.01) and more frequently a restrictive LV filling pattern (p<0.01). RV dimensions and RV free wall thickness were not significantly different in the two groups. A thick interventricular septum and a reduced TAPSE were associated with high NT-proBNP levels (both p<0.01). Seven patients died during a median follow-up period of 19 months; TAPSE<17 mm was the only echocardiographic parameter associated with poor survival.
  
  
  CONCLUSIONS
  In patients with AL amyloidosis, RV dysfunction is associated with more severe involvement of the left ventricle, higher plasma levels of NT-proBNP and with poor prognosis.
authors.affiliation: Division of Cardiology, Fondazione IRCCS Policlinico S. Matteo and University of Pavia, 27100 Pavia, Italy. s.ghio@smatteo.pv.it
authors.forename: Stefano
authors.initials: S
authors.lastname: Ghio
authors.forename: Stefano
authors.initials: S
authors.lastname: Perlini
authors.forename: Giovanni
authors.initials: G
authors.lastname: Palladini
authors.forename: Nina Ajmone
authors.initials: NA
authors.lastname: Marsan
authors.forename: Giovanna
authors.initials: G
authors.lastname: Faggiano
authors.forename: Monia
authors.initials: M
authors.lastname: Vezzoli
authors.forename: Catherine
authors.initials: C
authors.lastname: Klersy
authors.forename: Carlo
authors.initials: C
authors.lastname: Campana
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
authors.forename: Luigi
authors.initials: L
authors.lastname: Tavazzi
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000503:physiopathology*; D015150:Echocardiography, Doppler; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011379:Prognosis; D011446:Prospective Studies; D013318:Stroke Volume; D018487:Ventricular Dysfunction, Left; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging*; D016278:Ventricular Function, Right
chemical_list: D010446:Peptide Fragments; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain