Anales de Cardiología
Número 104, 14 de Enero de 2010
Noticia adicional
CARDIOLOGY: -Systemic cardiac amyloidosis
Background In this study, we compared the diagnostic/clinical profiles of these 3 types of systemic cardiac amyloidosis.
Most studies of amyloidotic cardiomyopathy consider as a single entity the 3 main systemic cardiac amyloidoses:
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.
Circulation. 2009;120:1203-1212
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@unibo.it
http://circ.ahajournals.org/cgi/content/abstract/120/13/1203
http://www.e-medicum.com/noticiasDelDia/verNoticia.php?noticia=84055
