Cardiomyopathies

Cardiomyopathies

Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, Salvi F, Ciliberti P, Pastorelli F, Biagini E, Coccolo F, Cooke RM, Bacchi-Reggiani L, Sangiorgi D, Ferlini A, Cavo M, Zamagni E, Fonte ML, Palladini G, Salinaro F, Musca F, Obici L, Branzi A, Perlini S. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation 2009;120(13):1203-12.

Synopsis

The authors presented the longitudinal study of 233 patients with clear-cut diagnosis by type of cardiac amyloidosis: acquired monoclonal immunoglobulin light-chain (AL), hereditary, mutated transthyretin-related (ATTRm) and wild-type transthyretin-related (ATTRwl).  Patients with AL were older than ATTRm patients, and most patients with ATTRwt were males.  At diagnosis, mean left ventricular wall thickness was higher and left ventricular ejection fraction was moderately depressed in ATTRwt patients as compared to 2 other types.  Low QRS voltage and low voltage-to-mass ratio was less often seen in ATTRm patients.  Unadjusted overall survival at 2 years was 63%, 98% and 100% for patients with AL, ATTRm and ATTRwt, respectively.  Freedom from MACEs at 2 years was 51%, 77% and 69%  patients with AL, ATTRm and ATTRwt, respectively.

Commentary

The presented paper provides evidence that systemic amyloidosis with myocardium involvement should not be considered as a single entity.  There are significant clinical differences, including the character and magnitude of heart dysfunction as well as prognosis, between 3 major types of cardiac amyloidosis, i.e. acquired monoclonal immunoglobulin light-chain (AL), hereditary, mutated transthyretin-related (ATTRm) and wild-type transthyretin-related (ATTRwl).  This diversity seems to affect both diagnostic measures and clinical management.