title: Left atrial size is an independent predictor of overall survival in patients with primary systemic amyloidosis.
abstract: BACKGROUND
  Primary systemic amyloidosis is a severe plasma cell disorder characterized by the extracellular deposition of amyloid fibrils in different organs. Echocardiography is usually performed to assess cardiac involvement. We hypothesized that in patients with systemic amyloidosis, simple echocardiographic measurement of the left atrial (LA) diameter indexed to the body surface area might provide an important risk marker for this disease.
  
  
  METHODS
  Between 1997 and 2011, 134 patients were diagnosed with primary systemic amyloidosis and had echocardiography within 28 days; we collected their baseline characteristics and biological and echocardiographic data retrospectively. LA enlargement was defined as recommended as M-mode LA diameter greater or equal to 23 mm/m(2).
  
  
  RESULTS
  One hundred and eleven patients (83%) had echocardiographic LA dimension data available (mean age 63±11 years; 61% men; 31% previously diagnosed with systemic hypertension). Mean left ventricular ejection fraction (LVEF) and interventricular septum thickness (IVST) were 62±12% and 14±4 mm, respectively. Mean follow-up was 2.8±2.9 years (maximum 12 years). Patients with LA enlargement had a slightly lower LVEF (P=0.08) and a significantly greater IVST (P<0.0001). Overall, 5-year survival was 57±5%. However, 1-year and 5-year survival rates were markedly reduced in patients with LA enlargement versus those without LA enlargement (61±7% and 39±8% vs 83±5% and 72±7%, respectively; P=0.0007). On multivariable analysis, after adjusting for age, sex, LVEF, IVST, presence of hypertension and creatinine concentration, LA enlargement remained an independent predictor of overall mortality at 5 years (hazard ratio 2.47; 95% confidence interval 1.11-5.90; P=0.02).
  
  
  CONCLUSIONS
  LA enlargement, a surrogate marker of diastolic dysfunction, is an independent predictor of long-term mortality and may therefore help to enhance risk stratification and management of patients presenting with amyloidosis.
authors.affiliation: Pôle cœur-poumon-rein, service cardiologie, hôpital Dupuytren, CHU de Limoges, 87042 Limoges, France. dania.mohty@chu-limoges.fr
authors.forename: Dania
authors.initials: D
authors.lastname: Mohty
authors.forename: Philippe
authors.initials: P
authors.lastname: Pibarot
authors.forename: Jean G
authors.initials: JG
authors.lastname: Dumesnil
authors.forename: Nicole
authors.initials: N
authors.lastname: Darodes
authors.forename: David
authors.initials: D
authors.lastname: Lavergne
authors.forename: Najmeddine
authors.initials: N
authors.lastname: Echahidi
authors.forename: Patrice
authors.initials: P
authors.lastname: Virot
authors.forename: Dominique
authors.initials: D
authors.lastname: Bordessoule
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000401:mortality* / Q000503:physiopathology; D015150:Echocardiography, Doppler; D005260:Female; D005602:France; D006325:Heart Atria / Q000000981:diagnostic imaging; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000000981:diagnostic imaging* / Q000209:etiology / Q000401:mortality* / Q000503:physiopathology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011237:Predictive Value of Tests; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013318:Stroke Volume; D015996:Survival Rate; D013997:Time Factors; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000209:etiology / Q000401:mortality* / Q000503:physiopathology; D016277:Ventricular Function, Left