Treatment of amyloid cardiomyopathy
- William J McKenna, MD
William J McKenna, MD
- Section Editor — Myopericardial Disease
- Professor of Cardiology
- University College, London
Amyloidosis refers to the extracellular deposition of fibrils that are composed of low molecular weight subunits (5 to 25 kD) of a variety of serum proteins. These fibrils adopt a beta-pleated sheet configuration that leads to characteristic histologic changes. Amyloid deposits can occur in a variety of organs, with involvement of the heart, kidney, liver, and autonomic nervous system most often being responsible for morbidity and mortality. (See "Overview of amyloidosis".)
The frequency of cardiac involvement varies among types of amyloidosis. The prognosis of amyloid cardiomyopathy also varies among types of amyloidosis, with high mortality rates particularly in light–chain (AL) amyloidosis.
This topic will review the treatment of amyloid cardiomyopathy. The clinical manifestations and diagnosis of amyloid cardiomyopathy are discussed separately. (See "Clinical manifestations and diagnosis of amyloid cardiomyopathy".)
General considerations — The treatment of symptomatic cardiac amyloidosis is twofold: therapy of heart failure and treatment of the underlying disease. Patients with TTR amyloidosis generally respond better to heart failure therapy than patients with AL amyloidosis. However, there are more therapeutic options for addressing the underlying disease in AL amyloidosis, and if the plasma cell dyscrasia can be controlled, there is often a relatively rapid decrease in serum biomarkers of heart failure.
Heart failure therapy
Approach to heart failure — Treatment of heart failure in patients with cardiac amyloidosis differs from the therapy generally recommended in patients with diastolic or systolic heart failure. While loop diuretics are a mainstay of treatment of cardiac amyloidosis, beta-blockers and ACE inhibitors may be harmful despite their efficacy in other types of systolic heart failure. Similarly, calcium channel blockers that may be useful in treatment of diastolic heart failure are contraindicated in amyloid cardiomyopathy. Adverse responses to drugs in cardiac amyloidosis are likely due to its unique pathophysiologic features.
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- General considerations
- Heart failure therapy
- - Approach to heart failure
- - Medical therapy
- - Heart transplantation and ventricular assist devices
- Treatment of atrial fibrillation
- Implantable cardioverter-defibrillator
- TREATMENT OF THE UNDERLYING PROTEIN MISFOLDING DISORDER
- Specific therapy for AL amyloidosis
- Specific therapy of TTR amyloidosis
- - Liver transplantation
- Investigational agents
- SUMMARY AND RECOMMENDATIONS