title: Right ventricular function in AL amyloidosis: characteristics and prognostic implication.
abstract: OBJECTIVE
  The importance of right ventricle (RV) dysfunction in AL amyloidosis has been underestimated. This study was designed to comprehensively evaluate RV function and its prognostic role in patients with AL amyloidosis with and without echocardiographic evidence of cardiac involvement.
  
  
  RESULTS
  Fifty-two biopsy-proven AL amyloidosis patients underwent a thorough echocardiographic evaluation. Twenty-seven patients (CA) met the international echocardiographic criteria for cardiac involvement [left ventricular (LV) wall thickness ≥ 12 mm] and 25 patients had no cardiac amyloidosis features (NCA). Patients were compared with a sex- age-matched control group. Patients and controls underwent traditional, tissue Doppler (TDI), speckle-tracking left and RV echocardiographic evaluation. No difference was observed between groups in RV diastolic diameter, whereas CA patients showed increased RV free wall thickness (P< 0.0001). Compared with controls and NCA patients, traditional echocardiography, TDI, and speckle-tracking evaluation detected significantly (P< 0.0001) depressed RV longitudinal systolic function in CA patients. No difference was observed between groups at Doppler diastolic evaluation, whereas at tricuspidal annulus TDI analysis, CA subject showed significantly lower E' and A' values with increased E/E' ratio (P< 0.0001). Over a 19 months median follow-up period, 18 patients died. Cox multivariate analysis showed that N-terminal pro-Brain natriuretic peptide and RV longitudinal strain were the strongest death predictor.
  
  
  CONCLUSIONS
  Our data show that in patients with AL amyloidosis, RV involvement develops later than LV amyloid deposition but when it occurs, prognosis dramatically worsens. Moreover RV longitudinal strain was the only echocardiographic predictor of prognosis. We suggest that RV function analysis should be performed routinely as a part of echocardiographic evaluation in these patients.
authors.affiliation: Intensive Cardiac Care Unit, Heart and Vessel Department, Azienda Ospedaliero Universitaria Careggi, University of Florence, Largo Brambilla 3, 50127 Florence, Italy. cappellifrancesco@inwind.it
authors.forename: Francesco
authors.initials: F
authors.lastname: Cappelli
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Porciani
authors.forename: Franco
authors.initials: F
authors.lastname: Bergesio
authors.forename: Stefano
authors.initials: S
authors.lastname: Perlini
authors.forename: Paola
authors.initials: P
authors.lastname: Attanà
authors.forename: Alberto
authors.initials: A
authors.lastname: Moggi Pignone
authors.forename: Francesco
authors.initials: F
authors.lastname: Salinaro
authors.forename: Francesco
authors.initials: F
authors.lastname: Musca
authors.forename: Luigi
authors.initials: L
authors.lastname: Padeletti
authors.forename: Federico
authors.initials: F
authors.lastname: Perfetto
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology*; D015415:Biomarkers; D016022:Case-Control Studies; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D008297:Male; D015999:Multivariate Analysis; D011379:Prognosis; D016016:Proportional Hazards Models; D011446:Prospective Studies; D012372:ROC Curve; D014463:Ultrasonography; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology*; D016278:Ventricular Function, Right
chemical_list: D015415:Biomarkers