Cardiac involvement with amyloidosis: mechanisms of disease, diagnosis and management
Marcu, C.B.; Niessen, H.W.; Beek, A.M.; Brouwer, W.P.; Robbers, L.F.; Van Rossum, A.C.
Connecticut Medicine 75(10): 581-590
2011
ISSN/ISBN: 0010-6178 PMID: 22216672 Document Number: 650210
The amyloidoses represent a group of clinical disorders of diverse etiologies that have as a common pathophysiologic denominator the deposition of misfolded protein based amyloid fibrils in the interstitial space of various organs. They are uncommon diseases with protean clinical presentations. Cardiac involvement is the determining factor for a patient's prognosis. Clinicians have to maintain a high index of suspicion and actively search for signs and symptoms of cardiac involvement in patients with preexisting conditions known to be associated with the development of amyloidosis. Early diagnosis and accurate fibril typing are the first steps in managing the disease. Judicious use of various diagnostic modalities such as serum markers and imaging studies, and good communication among all the physicians involved in the care of these sick and frail patients, are keys to a better outcome.