title: Prevalence and prognostic impact of left-sided valve thickening in systemic light-chain amyloidosis.
abstract: BACKGROUND
  Left heart valve thickening (LVT) was described in patients with light-chain amyloidosis (AL). This phenomenon reflects likely infiltration of the valve by amyloid proteins. However, the prevalence of LVT and its prognostic value have not been investigated in patients with AL.
  
  
  RESULTS
  Comprehensive transthoracic echocardiography was performed at baseline in 150 patients [median age 68 (33-87) years; 59% male] with confirmed AL. The presence of abnormal mitral and/or aortic valve thickening (>3 mm) was assessed in all included patients. Overall, 42% had LVT at the time of diagnosis. Compared to patients without LVT, those with LVT were older and had a more advanced NYHA functional class (63% in patients with NYHA III-IV vs. 33% in NYHA I-II, p < 0.001). They also had higher left ventricular (LV) wall thickness and mass, larger left atrium, higher mitral annulus E/E' ratio and systolic pulmonary artery pressures, and lower LV ejection fraction (all p < 0.05). Patients with more advanced Mayo Clinic stage had a higher incidence of LVT: 58% in stage III vs. 45% in stage II and 5% in stage I (p < 0.001). During a median follow-up of 2 years, 79 deaths occurred. The presence of LVT was significantly associated with reduced 5-year survival (32 ± 7 vs. 64 ± 6%). In multivariate analysis, after adjusting for age, gender, NYHA functional class, and LV ejection fraction, LVT remained significantly associated with higher all-cause mortality (hazard ratio 1.90, 95% CI 1.10-3.34, p = 0.02).
  
  
  CONCLUSIONS
  Left heart valve thickening is common in patients with AL and is associated with worse functional class, LV systolic and diastolic function, and more advanced stage of the disease. In addition, LVT appears to be a powerful marker of all-cause mortality.
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France. dania.mohty@chu-limoges.fr.|Faculté de médecine de Limoges, INSERM 1094, 2, Rue Marcland, 87000, Limoges, France. dania.mohty@chu-limoges.fr.|Department of Hematology, French National Reference Center of AL Amyloidosis Pôle «Onco-Hématologie», CHU Dupuytren, Limoges, France. dania.mohty@chu-limoges.fr.
authors.forename: Dania
authors.identifier: http://orcid.org/0000-0002-7160-2506
authors.initials: D
authors.lastname: Mohty
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.
authors.forename: Sarah
authors.initials: S
authors.lastname: Pradel
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.|Faculté de médecine de Limoges, INSERM 1094, 2, Rue Marcland, 87000, Limoges, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Magne
authors.affiliation: King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
authors.forename: Bahaa
authors.initials: B
authors.lastname: Fadel
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.
authors.forename: Cyrille
authors.initials: C
authors.lastname: Boulogne
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Petitalot
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.
authors.forename: Safaa
authors.initials: S
authors.lastname: Raboukhi
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.
authors.forename: Nicole
authors.initials: N
authors.lastname: Darodes
authors.affiliation: Department of Cardiology, AP-HP, Henri Mondor University-Hospital, Créteil, France.
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
authors.affiliation: Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France.|Faculté de médecine de Limoges, INSERM 1094, 2, Rue Marcland, 87000, Limoges, France.
authors.forename: Victor
authors.initials: V
authors.lastname: Aboyans
authors.affiliation: Department of Hematology, French National Reference Center of AL Amyloidosis Pôle «Onco-Hématologie», CHU Dupuytren, Limoges, France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001021:Aortic Valve / Q000000981:diagnostic imaging*; D015897:Comorbidity; D004452:Echocardiography / Q000706:statistics & numerical data; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis / Q000000981:diagnostic imaging* / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D008943:Mitral Valve / Q000000981:diagnostic imaging*; D009929:Organ Size; D015995:Prevalence; D011379:Prognosis; D015203:Reproducibility of Results; D018570:Risk Assessment / Q000379:methods; D012680:Sensitivity and Specificity; D015996:Survival Rate; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging* / Q000401:mortality*
keywords: Amyloidosis; Echocardiography; Light chains; Prognosis; Survival; Valve