doi: 10.1161/CIRCULATIONAHA.104.489187
(Circulation. 2005;112:2047-2060.)
© 2005 American Heart Association, Inc.
Contemporary Reviews in Cardiovascular Medicine |
Diagnosis and Management of the Cardiac Amyloidoses
From the Department of Cardiology, Harvard Vanguard Medical Associates, and Cardiovascular Genetics Center, Brigham and Women’s Hospital, Boston, Mass.
Correspondence to Rodney H. Falk, MD, Department of Cardiology, Harvard Vanguard Medical Associates, 133 Brookline Ave, Boston, MA 02215. E-mail rfalk@partners.org
Key Words: amyloid • cardiomyopathy • echocardiography • heart diseases • heart failure
An extract of the first 250 words of the full text is provided, because this article has no abstract. |
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Introduction |
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Cardiac amyloidosis is a manifestation of one of several systemic diseases known as the amyloidoses.1,2 This uncommon disease is probably underdiagnosed, and even when a diagnosis of amyloidosis of the heart is made, the fact that there are several types of amyloid, each with its unique features and treatment, is often unrecognized. This can lead to errors in management and in the information conveyed to the patient. The purpose of this review is to familiarize the reader with the clinical features of amyloidosis and to address the approach to the patient with this disease, focusing on the various types of amyloidosis, their prognosis and treatment.
The common feature of this group of diseases is the extracellular deposition of a proteinaceous material that, when stained with Congo red, demonstrates apple-green birefringence under polarized light and that has a distinct color when stained with sulfated Alcian blue (Figure 1). Viewed with electron microscopy, the amyloid deposits are seen to be composed of a ß-sheet fibrillar material (Figure 2). These nonbranching fibrils have a diameter3 of 7.5 to 10 nm and are the result of protein misfolding.4,5 Cardiac involvement in amyloidosis may be the predominant feature or may be found on investigation of a patient presenting with another major organ involvement. The presence of cardiac amyloidosis and its relative predominance varies with the type of amyloidosis. Thus, senile systemic amyloidosis and some forms of transthyretin amyloidosis invariably affect the heart, whereas cardiac involvement ranges from absent to severe in
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