Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Treatment of amyloid cardiomyopathy

William J McKenna, MD
Section Editor
Robert A Kyle, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC


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 (HF) and treatment of the underlying disease. Patients with familial or senile (ATTR) amyloidosis generally respond better to HF 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 HF [1].

Heart failure therapy

Approach to heart failure — Treatment of HF in patients with cardiac amyloidosis differs from the therapy generally recommended in patients with diastolic or systolic HF. 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 HF. Similarly, calcium channel blockers that may be useful in treatment of diastolic HF are contraindicated in amyloid cardiomyopathy. Adverse responses to drugs in cardiac amyloidosis are likely due to its unique pathophysiologic features.

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Nov 02, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Maurer MS, Elliott P, Comenzo R, et al. Addressing Common Questions Encountered in the Diagnosis and Management of Cardiac Amyloidosis. Circulation 2017; 135:1357.
  2. Griffiths BE, Hughes P, Dowdle R, Stephens MR. Cardiac amyloidosis with asymmetrical septal hypertrophy and deterioration after nifedipine. Thorax 1982; 37:711.
  3. Gertz MA, Skinner M, Connors LH, et al. Selective binding of nifedipine to amyloid fibrils. Am J Cardiol 1985; 55:1646.
  4. Pollak A, Falk RH. Left ventricular systolic dysfunction precipitated by verapamil in cardiac amyloidosis. Chest 1993; 104:618.
  5. Dubrey SW, Cha K, Anderson J, et al. The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement. QJM 1998; 91:141.
  6. Dubrey S, Simms RW, Skinner M, Falk RH. Recurrence of primary (AL) amyloidosis in a transplanted heart with four-year survival. Am J Cardiol 1995; 76:739.
  7. Hosenpud JD, DeMarco T, Frazier OH, et al. Progression of systemic disease and reduced long-term survival in patients with cardiac amyloidosis undergoing heart transplantation. Follow-up results of a multicenter survey. Circulation 1991; 84:III338.
  8. Kpodonu J, Massad MG, Caines A, Geha AS. Outcome of heart transplantation in patients with amyloid cardiomyopathy. J Heart Lung Transplant 2005; 24:1763.
  9. Dubrey SW, Burke MM, Hawkins PN, Banner NR. Cardiac transplantation for amyloid heart disease: the United Kingdom experience. J Heart Lung Transplant 2004; 23:1142.
  10. Pelosi F Jr, Capehart J, Roberts WC. Effectiveness of cardiac transplantation for primary (AL) cardiac amyloidosis. Am J Cardiol 1997; 79:532.
  11. Maurer MS, Raina A, Hesdorffer C, et al. Cardiac transplantation using extended-donor criteria organs for systemic amyloidosis complicated by heart failure. Transplantation 2007; 83:539.
  12. Davis MK, Kale P, Liedtke M, et al. Outcomes after heart transplantation for amyloid cardiomyopathy in the modern era. Am J Transplant 2015; 15:650.
  13. Fuchs U, Zittermann A, Suhr O, et al. Heart transplantation in a 68-year-old patient with senile systemic amyloidosis. Am J Transplant 2005; 5:1159.
  14. Careddu L, Zanfi C, Pantaleo A, et al. Combined heart-liver transplantation: a single-center experience. Transpl Int 2015; 28:828.
  15. Thenappan T, Fedson S, Rich J, et al. Isolated heart transplantation for familial transthyretin (TTR) V122I cardiac amyloidosis. Amyloid 2014; 21:120.
  16. Frazier OH, Cohn WE. Continuous-flow total heart replacement device implanted in a 55-year-old man with end-stage heart failure and severe amyloidosis. Tex Heart Inst J 2012; 39:542.
  17. Tan NY, Mohsin Y, Hodge DO, et al. Catheter Ablation for Atrial Arrhythmias in Patients With Cardiac Amyloidosis. J Cardiovasc Electrophysiol 2016; 27:1167.
  18. Feng D, Edwards WD, Oh JK, et al. Intracardiac thrombosis and embolism in patients with cardiac amyloidosis. Circulation 2007; 116:2420.
  19. Feng D, Syed IS, Martinez M, et al. Intracardiac thrombosis and anticoagulation therapy in cardiac amyloidosis. Circulation 2009; 119:2490.
  20. Kristen AV, Dengler TJ, Hegenbart U, et al. Prophylactic implantation of cardioverter-defibrillator in patients with severe cardiac amyloidosis and high risk for sudden cardiac death. Heart Rhythm 2008; 5:235.
  21. Lin G, Dispenzieri A, Kyle R, et al. Implantable cardioverter defibrillators in patients with cardiac amyloidosis. J Cardiovasc Electrophysiol 2013; 24:793.
  22. Wechalekar AD, Schonland SO, Kastritis E, et al. A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood 2013; 121:3420.
  23. Kastritis E, Roussou M, Gavriatopoulou M, et al. Long-term outcomes of primary systemic light chain (AL) amyloidosis in patients treated upfront with bortezomib or lenalidomide and the importance of risk adapted strategies. Am J Hematol 2015; 90:E60.
  24. Dubrey SW, Davidoff R, Skinner M, et al. Progression of ventricular wall thickening after liver transplantation for familial amyloidosis. Transplantation 1997; 64:74.
  25. Raichlin E, Daly RC, Rosen CB, et al. Combined heart and liver transplantation: a single-center experience. Transplantation 2009; 88:219.
  26. Sattianayagam PT, Hahn AF, Whelan CJ, et al. Cardiac phenotype and clinical outcome of familial amyloid polyneuropathy associated with transthyretin alanine 60 variant. Eur Heart J 2012; 33:1120.
  27. Bulawa CE, Connelly S, Devit M, et al. Tafamidis, a potent and selective transthyretin kinetic stabilizer that inhibits the amyloid cascade. Proc Natl Acad Sci U S A 2012; 109:9629.
  28. Said G, Grippon S, Kirkpatrick P. Tafamidis. Nat Rev Drug Discov 2012; 11:185.
  29. Coelho T, Adams D, Silva A, et al. Safety and efficacy of RNAi therapy for transthyretin amyloidosis. N Engl J Med 2013; 369:819.