This Article Free upon publication Full Text Full Text (PDF) CME: Take the course for this article: Circulation: September 29, 2009, Volume 120, Number 13 All Versions of this Article: 120/13/1203    most recent CIRCULATIONAHA.108.843334v1 Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Request Permissions Google Scholar Articles by Rapezzi, C. Articles by Perlini, S. PubMed PubMed Citation Articles by Rapezzi, C. Articles by Perlini, S. Pubmed/NCBI databases Substance via MeSH Medline Plus Health Information Amyloidosis Cardiomyopathy Related Collections Myocardial cardiomyopathy disease Electrocardiology Echocardiography Related Article

(Circulation. 2009;120:1203-1212.)
© 2009 American Heart Association, Inc.

Heart Failure

Systemic Cardiac Amyloidoses

Disease Profiles and Clinical Courses of the 3 Main Types

Claudio Rapezzi, MD; Giampaolo Merlini, MD; Candida C. Quarta, MD; Letizia Riva, MD; Simone Longhi, MD; Ornella Leone, MD; Fabrizio Salvi, MD; Paolo Ciliberti, MD; Francesca Pastorelli, MD; Elena Biagini, MD; Fabio Coccolo, MD; Robin M.T. Cooke, MA; Letizia Bacchi-Reggiani, MSc, MStat; Diego Sangiorgi, MStat; Alessandra Ferlini, MD; Michele Cavo, MD; Elena Zamagni, MD; Maria Luisa Fonte, MD; Giovanni Palladini, MD; Francesco Salinaro, MD; Francesco Musca, MD; Laura Obici, MD; Angelo Branzi, MD; Stefano Perlini, MD

From the Institute of Cardiology (C.R., C.C.Q., L.R., S.L., P.C., E.B., F.C., R.M.T.C., L.B.-R., D.S., A.B.), Department of Pathology (O.L.), and Institute of Hematology (M.C., E.Z.), University of Bologna, and S. Orsola-Malpighi Hospital, Bologna; Center for Amyloidosis, Fondazione IRCCS San Matteo and University of Pavia, Pavia (G.M., M.L.F., G.P., F. Salinaro, F.M., L.O., S.P.); Department of Neurology, Bellaria Hospital, Bologna (F. Salvi, F.P.); and Department of Diagnostic and Experimental Medicine, Section of Medical Genetics, University of Ferrara, Ferrara (A.F.), Italy.

Correspondence to Professor Claudio Rapezzi, Istituto di Cardiologia, Policlinico S. Orsola-Malpighi, Via Massarenti n 9, 40125 Bologna, Italy. E-mail claudio.rapezzi{at}unibo.it

Received December 23, 2008; accepted July 17, 2009.

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.

---

 

CLINICAL PERSPECTIVE

Related Article:

Clinical Summaries
Circulation 2009 120: 1165-1167. [Extract] [Full Text]