Current therapeutic strategies in cardiac amyloidosis

Shailja Parikh, James A de Lemos

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Amyloidosis is a systemic disease in which clinical manifestations are caused by the replacement of normal tissue with insoluble amyloid fibrils. Cardiac involvement causes a restrictive cardiomyopathy and is associated with poor functional outcomes. Cardiac magnetic resonance imaging and measurement of B-type natriuretic peptide are particularly helpful in distinguishing restrictive cardiomyopathy from constrictive pericarditis, but a tissue biopsy is required to make the diagnosis of amyloidosis. Although standard treatment options for congestive heart failure may provide symptomatic relief in cardiac amyloidosis, prognosis remains dismal. Judicious diuretic use remains the mainstay of therapy, but achieving optimal fluid balance is difficult because patients are usually "preload dependent." Angiotensin-converting enzyme inhibitors in low doses are often helpful but may lead to orthostatic hypotension, particularly in patients who also have involvement of the autonomic nervous system. β Blockers may be useful if given relatively early in the disease process, but should be used with caution in patients with advanced disease because they may exacerbate symptoms. Therapy aimed at the underlying disease process in primary systemic amyloidosis is based on treatment regimens used in multiple myeloma, such as melphalan and prednisone. These offer limited benefit when cardiac involvement is significant, but newer treatments, including the novel anthracycline 4′-iodo-4′-deoxy-doxorubicin, potentially combined with autologous stem cell transplantation, offer some hope for the future.

Original languageEnglish (US)
Pages (from-to)443-448
Number of pages6
JournalCurrent Treatment Options in Cardiovascular Medicine
Volume7
Issue number6
StatePublished - 2005

Fingerprint

Amyloidosis
Restrictive Cardiomyopathy
Constrictive Pericarditis
Therapeutics
Orthostatic Hypotension
Melphalan
Water-Electrolyte Balance
Brain Natriuretic Peptide
Anthracyclines
Autonomic Nervous System
Stem Cell Transplantation
Prednisone
Multiple Myeloma
Angiotensin-Converting Enzyme Inhibitors
Diuretics
Amyloid
Doxorubicin
Heart Failure
Magnetic Resonance Imaging
Biopsy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Current therapeutic strategies in cardiac amyloidosis. / Parikh, Shailja; de Lemos, James A.

In: Current Treatment Options in Cardiovascular Medicine, Vol. 7, No. 6, 2005, p. 443-448.

Research output: Contribution to journalArticle

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