Document Detail

Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types.
MedLine Citation:
PMID:  19752327     Owner:  NLM     Status:  MEDLINE    
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. METHODS AND 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.
Claudio Rapezzi; Giampaolo Merlini; Candida C Quarta; Letizia Riva; Simone Longhi; Ornella Leone; Fabrizio Salvi; Paolo Ciliberti; Francesca Pastorelli; Elena Biagini; Fabio Coccolo; Robin M T Cooke; Letizia Bacchi-Reggiani; Diego Sangiorgi; Alessandra Ferlini; Michele Cavo; Elena Zamagni; Maria Luisa Fonte; Giovanni Palladini; Francesco Salinaro; Francesco Musca; Laura Obici; Angelo Branzi; Stefano Perlini
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Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't     Date:  2009-09-14
Journal Detail:
Title:  Circulation     Volume:  120     ISSN:  1524-4539     ISO Abbreviation:  Circulation     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-09-29     Completed Date:  2009-10-29     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0147763     Medline TA:  Circulation     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1203-12     Citation Subset:  AIM; IM    
Istituto di Cardiologia, Policlinico S. Orsola-Malpighi, Via Massarenti n 9, 40125 Bologna, Italy.
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MeSH Terms
Amyloidosis / genetics,  mortality*,  ultrasonography*
Blood Pressure
Cardiomyopathies / genetics,  mortality*,  ultrasonography*
Disease Progression
Follow-Up Studies
Immunoglobulin Light Chains / blood
Logistic Models
Longitudinal Studies
Middle Aged
Multivariate Analysis
Myocardium / pathology
Myocytes, Cardiac / pathology
Point Mutation
Prealbumin / genetics
Pulmonary Wedge Pressure
Risk Factors
Survival Analysis
Reg. No./Substance:
0/Immunoglobulin Light Chains; 0/Prealbumin

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