title: Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types.
abstract: BACKGROUND
  Most studies of amyloidotic cardiomyopathy consider as a single entity the 3 main systemic cardiac amyloidoses: acquired monoclonal immunoglobulin light-chain (AL); hereditary, mutated transthyretin-related (ATTRm); and wild-type transthyretin-related (ATTRwt). In this study, we compared the diagnostic/clinical profiles of these 3 types of systemic cardiac amyloidosis.
  
  
  RESULTS
  We conducted a longitudinal study of 233 patients with clear-cut diagnosis by type of cardiac amyloidosis (AL, n=157; ATTRm, n=61; ATTRwt, n=15) at 2 large Italian centers providing coordinated amyloidosis diagnosis/management facilities since 1990. Average age at diagnosis was higher in AL than in ATTRm patients; all ATTRwt patients except 1 were elderly men. At diagnosis, mean left ventricular wall thickness was higher in ATTRwt than in ATTRm and AL. Left ventricular ejection fraction was moderately depressed in ATTRwt but not in AL or ATTRm. ATTRm patients less often displayed low QRS voltage (25% versus 60% in AL; P<0.0001) or low voltage-to-mass ratio (1.1+/-0.5 versus 0.9+/-0.5; P<0.0001). AL patients appeared to have greater hemodynamic impairment. On multivariate analysis, ATTRm was a strongly favorable predictor of survival, and ATTRwt predicted freedom from major cardiac events.
  
  
  CONCLUSIONS
  AL, ATTRm, and ATTRwt should be considered 3 different cardiac diseases, probably characterized by different pathophysiological substrates and courses. Awareness of the diversity underlying the cardiac amyloidosis label is important on several levels, ranging from disease classification to diagnosis and clinical management.
authors.affiliation: Istituto di Cardiologia, Policlinico S. Orsola-Malpighi, Via Massarenti n 9, 40125 Bologna, Italy. claudio.rapezzi@unibo.it
authors.forename: Claudio
authors.initials: C
authors.lastname: Rapezzi
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
authors.forename: Candida C
authors.initials: CC
authors.lastname: Quarta
authors.forename: Letizia
authors.initials: L
authors.lastname: Riva
authors.forename: Simone
authors.initials: S
authors.lastname: Longhi
authors.forename: Ornella
authors.initials: O
authors.lastname: Leone
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Salvi
authors.forename: Paolo
authors.initials: P
authors.lastname: Ciliberti
authors.forename: Francesca
authors.initials: F
authors.lastname: Pastorelli
authors.forename: Elena
authors.initials: E
authors.lastname: Biagini
authors.forename: Fabio
authors.initials: F
authors.lastname: Coccolo
authors.forename: Robin M T
authors.initials: RM
authors.lastname: Cooke
authors.forename: Letizia
authors.initials: L
authors.lastname: Bacchi-Reggiani
authors.forename: Diego
authors.initials: D
authors.lastname: Sangiorgi
authors.forename: Alessandra
authors.initials: A
authors.lastname: Ferlini
authors.forename: Michele
authors.initials: M
authors.lastname: Cavo
authors.forename: Elena
authors.initials: E
authors.lastname: Zamagni
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Fonte
authors.forename: Giovanni
authors.initials: G
authors.lastname: Palladini
authors.forename: Francesco
authors.initials: F
authors.lastname: Salinaro
authors.forename: Francesco
authors.initials: F
authors.lastname: Musca
authors.forename: Laura
authors.initials: L
authors.lastname: Obici
authors.forename: Angelo
authors.initials: A
authors.lastname: Branzi
authors.forename: Stefano
authors.initials: S
authors.lastname: Perlini
mesh_terms: D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000235:genetics / Q000401:mortality*; D001794:Blood Pressure; D009202:Cardiomyopathies / Q000000981:diagnostic imaging* / Q000235:genetics / Q000401:mortality*; D018450:Disease Progression; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D016015:Logistic Models; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009206:Myocardium / Q000473:pathology; D032383:Myocytes, Cardiac / Q000473:pathology; D017354:Point Mutation; D011228:Prealbumin / Q000235:genetics; D011669:Pulmonary Wedge Pressure; D012307:Risk Factors; D016019:Survival Analysis
chemical_list: D007147:Immunoglobulin Light Chains; D011228:Prealbumin