title: Prognostic value of right ventricular systolic function in cardiac amyloidosis.
abstract: BACKGROUND
  Right ventricular (RV) dysfunction is a strong predictor of poor outcomes in heart failure. Its prognostic meaning in cardiac amyloidosis (CA) is under-investigated.
  
  
  METHODS
  Hundred and twenty nine patients with suspected CA and an interventricular septum thickness (IVST) ≥ 12 mm underwent echocardiography with measurement of left ventricular (LV) and RV longitudinal strain (LS), late gadolinium-enhancement (LGE) cardiac MRI, and standard evaluation.
  
  
  RESULTS
  Among 82 confirmed CA, types were immunoglobulin light chain (AL, n = 26), hereditary transthyretin (m-TTR, n = 37) and senile (WT-TTR, n = 19). Compared to those without, CA patients had significantly lower RV fractional shortening (RV-FS), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler systolic velocity, and global RV-LS, without any difference among the CA types. RV-LGE, observed in 62% of CA patients, was associated with lower global and basal RV-FS. Median follow-up was 8(2; 16) months. Using multivariate analysis, NYHA-class and low TAPSE independently predicted major adverse cardiac event (MACE) defined as death, heart transplantation and acute heart failure. Independent determinants of TAPSE < 14 mm, the best cut-off value, were LV ejection fraction (LVEF), estimated filling pressure (E/E'), NT-proBNP and pulmonary artery pressure, but not RV-LGE.
  
  
  CONCLUSIONS
  RV dysfunction is common in CA. Its routine evaluation by a simple TAPSE may be an aid in assessing the prognosis of CA patients.
authors.affiliation: a Department of Cardiology.
authors.forename: Diane
authors.initials: D
authors.lastname: Bodez
authors.affiliation: a Department of Cardiology.
authors.forename: Julien
authors.initials: J
authors.lastname: Ternacle
authors.affiliation: a Department of Cardiology.
authors.forename: Aziz
authors.initials: A
authors.lastname: Guellich
authors.affiliation: a Department of Cardiology.
authors.forename: Arnault
authors.initials: A
authors.lastname: Galat
authors.affiliation: a Department of Cardiology.
authors.forename: Pascal
authors.initials: P
authors.lastname: Lim
authors.affiliation: b Department of Cardiovascular Surgery.
authors.forename: Costin
authors.initials: C
authors.lastname: Radu
authors.affiliation: a Department of Cardiology.
authors.forename: Soulef
authors.initials: S
authors.lastname: Guendouz
authors.affiliation: b Department of Cardiovascular Surgery.
authors.forename: Eric
authors.initials: E
authors.lastname: Bergoend
authors.affiliation: b Department of Cardiovascular Surgery.
authors.forename: Jean-Paul
authors.initials: JP
authors.lastname: Couetil
authors.affiliation: a Department of Cardiology.
authors.forename: Luc
authors.initials: L
authors.lastname: Hittinger
authors.affiliation: a Department of Cardiology.
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Dubois-Randé
authors.affiliation: c Department of Neurology , and.
authors.forename: Violaine
authors.initials: V
authors.lastname: Plante-Bordeneuve
authors.affiliation: d Department of Radiology, AP-HP, Henri-Mondor Teaching Hospital, UPEC; IMRB-GRC Amyloid Research Institute , DHU A-TVB and Mondor Amyloidosis Network, Inserm U955, Inserm Clinical Investigation Center 1430 , Créteil , France , and.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Deux
authors.affiliation: e Department of Cardiology, Dupuytren Hospital, CHU Limoges , Pôle Cœur-Poumon-Rein, Limoges , France.
authors.forename: Dania
authors.initials: D
authors.lastname: Mohty
authors.affiliation: a Department of Cardiology.
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D009202:Cardiomyopathies / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D015150:Echocardiography, Doppler; D005260:Female; D006333:Heart Failure / Q000097:blood / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000473:pathology / Q000503:physiopathology*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D011228:Prealbumin / Q000378:metabolism; D011379:Prognosis; D011446:Prospective Studies; D013318:Stroke Volume; D016019:Survival Analysis; D018497:Ventricular Dysfunction, Right / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000401:mortality
chemical_list: D007147:Immunoglobulin Light Chains; D010446:Peptide Fragments; D011228:Prealbumin; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: Cardiac amyloidosis; echocardiography; heart failure; prognosis; right ventricle