Cardiac involvement with amyloidosis: mechanisms of disease, diagnosis and management.
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.
Department of Cardiology, VU University Medical Center, Amsterdam, The Netherlands.
Constantin B
CB
Marcu
Hans W
HW
Niessen
Aernout M
AM
Beek
Wessel P
WP
Brouwer
Lourens F
LF
Robbers
Albert C
AC
Van Rossum
D000686:Amyloidosis / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D016757:Death, Sudden, Cardiac / Q000517:prevention & control; D003952:Diagnostic Imaging; D042241:Early Diagnosis; D004562:Electrocardiography; D006801:Humans; D008854:Microscopy, Electron; D009206:Myocardium / Q000473:pathology; D011379:Prognosis