Cardiac amyloidosis is emerging as an underdiagnosed cause of heart failure and mortality. Growing literature suggests that a noninvasive diagnosis of cardiac amyloidosis is now feasible. However, the diagnostic criteria and utilization of imaging in cardiac amyloidosis are not standardized. In this paper, Part 2 of a series, a panel of international experts from multiple societies define the diagnostic criteria for cardiac amyloidosis and appropriate utilization of echocardiography, cardiovascular magnetic resonance imaging, and radionuclide imaging in the evaluation of patients with known or suspected cardiac amyloidosis.
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Amyloid immunoglobulin light chains
Gonzalez-Lopez E, Gallego-Delgado M, Guzzo-Merello G, de Haro-Del Moral FJ, Cobo-Marcos M, Robles C, et al. Wild-type transthyretin amyloidosis as a cause of heart failure with preserved ejection fraction. Eur Heart J 2015;36:2585-94.
Falk RH, Alexander KM, Liao R, Dorbala S. AL (light-chain) cardiac amyloidosis: a review of diagnosis and therapy. J Am Coll Cardiol 2016;68:1323-41.
Ruberg FL, Berk JL. Transthyretin (TTR) cardiac amyloidosis. Circulation 2012;126:1286-300.
Banypersad SM, Sado DM, Flett AS, Gibbs SD, Pinney JH, Maestrini V, et al. Quantification of myocardial extracellular volume fraction in systemic AL amyloidosis: an equilibrium contrast cardiovascular magnetic resonance study. Circ Cardiovasc Imaging 2013;6:34-9.
Castano A, Narotsky DL, Hamid N, Khalique OK, Morgenstern R, DeLuca A, et al. Unveiling transthyretin cardiac amyloidosis and its predictors among elderly patients with severe aortic stenosis undergoing transcatheter aortic valve replacement. Eur Heart J 2017;38:2879-87.
Sperry BW, Reyes BA, Ikram A, Donnelly JP, Phelan D, Jaber WA, et al. Tenosynovial and cardiac amyloidosis in patients undergoing carpal tunnel release. J Am Coll Cardiol 2018;72:2040-50.
Grogan M, Scott CG, Kyle RA, Zeldenrust SR, Gertz MA, Lin G, et al. Natural history of wild-type transthyretin cardiac amyloidosis and risk stratification using a novel staging system. J Am Coll Cardiol 2016;68:1014-20.
Knight DS, Zumbo G, Barcella W, Steeden JA, Muthurangu V, Martinez-Naharro A, et al. Cardiac structural and functional consequences of amyloid deposition by cardiac magnetic resonance and echocardiography and their prognostic roles. JACC Cardiovasc Imaging 2018.
Fitch KB, Bernstein SJ, Aguilar MD, Burnand B, LaCalle JR, Lazaro P, et al. The RAND/UCLA appropriateness method user’s manual (MR-1269-DG-XII/RE). Santa Monica, California: RAND; 2001.
Patel MR, Spertus JA, Brindis RG, Hendel RC, Douglas PS, Peterson ED, et al. ACCF proposed method for evaluating the appropriateness of cardiovascular Imaging J Am Coll Cardiol 2005;46:1606-13.
Aortic Stenosis Writing G, Bonow RO, Brown AS, Gillam LD, Kapadia SR, Kavinsky CJ, et al. ACC, AATS, AHA, ASE, EACTS, HVS, SCA, SCAI, SCCT, SCMR, STS, 2017 appropriate use criteria for the treatment of patients with severe aortic stenosis: a report of the American College of Cardiology Appropriate Use Criteria Task Force, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, European Association for Cardio-Thoracic Surgery, Heart Valve Society, Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. J Am Soc Echocardiogr 2018;2018(31):117-47.
Wolk MJ, Bailey SR, Doherty JU, Douglas PS, Hendel RC, Kramer CM, et al. ACCF/AHA/ASE/ASNC/HFSA/HRS/SCAI/SCCT/SCMR/STS 2013 multimodality appropriate use criteria for the detection and risk assessment of stable ischemic heart disease: a report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. J Am Coll Cardiol 2014;63:380-406.
American College of Cardiology Foundation Appropriate Use Criteria Task F, American Society of E, American Heart A, American Society of Nuclear C, Heart Failure Society of A, Heart Rhythm S, et al. ACCF/ASE/AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011 Appropriate Use Criteria for Echocardiography. A Report of the American College of Cardiology Foundation Appropriate Use Criteria Task Force, American Society of Echocardiography, American Heart Association, American Society of Nuclear Cardiology, Heart Failure Society of America, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Critical Care Medicine, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance American College of Chest Physicians. J Am Soc Echocardiogr 2011;24:229-67.
Brook RH, Chassin MR, Fink A, Solomon DH, Kosecoff J, Park RE. A method for the detailed assessment of the appropriateness of medical technologies. Int J Technol Assess Health Care 1986;2:53-63.
Doherty JU, Kort S, Mehran R, Schoenhagen P, Soman P. ACC/AATS/AHA/ASE/ASNC/HRS/SCAI/SCCT/SCMR/STS 2017 appropriate use criteria for multimodality imaging in valvular heart disease: a report of the american college of cardiology appropriate use criteria task force, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. J Am Coll Cardiol 2017;70:1647-72.
Rybicki FJ, Udelson JE, Peacock WF, Goldhaber SZ, Isselbacher EM, Kazerooni E, et al. 2015 ACR/ACC/AHA/AATS/ACEP/ASNC/NASCI/SAEM/SCCT/SCMR/SCPC/SNMMI/STR/STS appropriate utilization of cardiovascular imaging in emergency department patients with chest pain: a joint document of the American College of Radiology Appropriateness Criteria Committee and the American College of Cardiology Appropriate Use Criteria Task Force. J Am Coll Cardiol 2016;67:853-79.
Hendel RC, Patel MR, Allen JM, Min JK, Shaw LJ, Wolk MJ, et al. Appropriate use of cardiovascular technology: 2013 ACCF appropriate use criteria methodology update: a report of the American College of Cardiology Foundation appropriate use criteria task force. J Am Coll Cardiol 2013;61:1305-17.
Patel MR, White RD, Abbara S, Bluemke DA, Herfkens RJ, Picard M, et al. 2013 ACCF/ACR/ASE/ASNC/SCCT/SCMR appropriate utilization of cardiovascular imaging in heart failure: a joint report of the American College of Radiology Appropriateness Criteria Committee and the American College of Cardiology Foundation Appropriate Use Criteria Task Force. J Am Coll Cardiol 2013;61:2207-31.
Amis ES Jr, Butler PF, Applegate KE, Birnbaum SB, Brateman LF, Hevezi JM, et al. American College of Radiology white paper on radiation dose in medicine. J Am Coll Radiol 2007;4:272-84.
Bernstein SJ, Hofer TP, Meijler AP, Rigter H. Setting standards for effectiveness: a comparison of expert panels and decision analysis. Int J Qual Health Care 1997;9:255-63.
Kuntz KM, Tsevat J, Weinstein MC, Goldman L. Expert panel vs decision-analysis recommendations for postdischarge coronary angiography after myocardial infarction. JAMA 1999;282:2246-51.
Rowczenio D, Wechalekar, A mutations in hereditary amyloidosis; 2015.
Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN, et al. Definition of organ involvement and treatment response in immunoglobulin light chain amyloidosis (AL): a consensus opinion from the 10th international symposium on amyloid and amyloidosis, Tours, France, 18-22 April 2004. Am J Hematol 2005;79:319-28.
Dispenzieri A, Gertz MA, Kyle RA, Lacy MQ, Burritt MF, Therneau TM, et al. Serum cardiac troponins and N-terminal pro-brain natriuretic peptide: a staging system for primary systemic amyloidosis. J Clin Oncol 2004;22:3751-7.
Wechalekar AD, Schonland SO, Kastritis E, Gillmore JD, Dimopoulos MA, Lane T, et al. A European collaborative study of treatment outcomes in 346 patients with cardiac stage III AL amyloidosis. Blood 2013;121:3420-7.
Kumar S, Dispenzieri A, Lacy MQ, Hayman SR, Buadi FK, Colby C, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol 2012;30:989-95.
Palladini G, Campana C, Klersy C, Balduini A, Vadacca G, Perfetti V, et al. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. Circulation 2003;107:2440-5.
Go RS, Rajkumar SV. How I manage monoclonal gammopathy of undetermined significance. Blood 2018;131:163-73.
Merlini G, Stone MJ. Dangerous small B-cell clones. Blood 2006;108:2520-30.
Dolgin MAN, Fox AC, Gorlin R, Levin RI. Nomenclature and criteria for diagnosis of diseases of the heart and great vessels/the Criteria Committee of the New York Heart Association. Boston, MA: Lippincott Williams and Wilkins; 1994.
Criteria Committee NYHA, Inc. Diseases of the heart and blood vessels. Nomenclature and criteria for diagnosis. Boston: Little, Brown and Co. 1964, p. 114.
Yancy CW, Jessup M, Bozkurt B, Butler J, Casey DE Jr, Drazner MH, et al. 2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on practice guidelines. Circulation 2013;128:e240-327.
Nishimura RA, Otto CM, Bonow RO, Carabello BA, Erwin JP 3rd, Guyton RA, et al. 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Circulation 2014;129:2440-92.
American College of Cardiology Foundation Task Force on Expert Consensus D, Hundley WG, Bluemke DA, Finn JP, Flamm SD, Fogel MA, et al. ACCF/ACR/AHA/NASCI/SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents. Circulation 2010;121:2462-508
Keith MW, Masear V, Chung KC, Maupin K, Andary M, Amadio PC, et al. American Academy of Orthopaedic Surgeons clinical practice guideline on diagnosis of carpal tunnel syndrome. J Bone Joint Surg Am 2009;91:2478-9.
Gillmore JD, Maurer MS, Falk RH, Merlini G, Damy T, Dispenzieri A, et al. Nonbiopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 2016;133:2404-12.
Jhaveri T, Sarosiek S, Ruberg FL, Siddiqi O, Berk JL, Sanchorawala V. Once AL amyloidosis: not always AL amyloidosis. Amyloid 2018;25:139-40.
Rubin J, Alvarez J, Teruya S, Castano A, Lehman RA, Weidenbaum M, et al. Hip and knee arthroplasty are common among patients with transthyretin cardiac amyloidosis, occurring years before cardiac amyloid diagnosis: can we identify affected patients earlier? Amyloid 2017;24:226-30.
Kawel N, Turkbey EB, Carr JJ, Eng J, Gomes AS, Hundley WG, et al. Normal left ventricular myocardial thickness for middle-aged and older subjects with steady-state free precession cardiac magnetic resonance: the multi-ethnic study of atherosclerosis. Circ Cardiovasc Imaging 2012;5:500-8.
We would like to thank the reviewers of this document for their input, which has significantly improved the quality of this document, including Renée P. Bullock-Palmer, MD, FACC, FASNC, FASE, FSCCT; Dennis A. Calnon, MD, FASNC; Marcelo F. Di Carli, MD; Martha Grogan, MD; Phillip Hawkins, PhD, FMedSci; Wael A. Jaber, MD, FACC, FAHA; Prem Soman, MD, FASNC; James E. Udelson, MD, FACC; Ashutosh D. Wechalekar, DM, MRCP, FRCPath.
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This article is being jointly published in the Journal of Nuclear Cardiology, Journal of Cardiac Failure, and Circulation: Cardiovascular Imaging.
Part 1 – Evidence Base and Standardized Methods of Imaging is available at https://doi.org/10.1007/s12350-019-01760-6.
This document was approved for publication by the governing body of the American Society of Nuclear Cardiology (ASNC) and was endorsed by the American College of Cardiology (ACC), the American Heart Association (AHA), American Society of Echocardiography (ASE), the European Association of Nuclear Medicine (EANM), the Heart Failure Society of America (HFSA), the International Society of Amyloidosis (ISA), the Society for Cardiovascular Magnetic Resonance (SCMR), and the Society of Nuclear Medicine and Molecular Imaging (SNMMI).
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Dorbala, S., Ando, Y., Bokhari, S. et al. ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 2 of 2—Diagnostic criteria and appropriate utilization. J. Nucl. Cardiol. 27, 659–673 (2020). https://doi.org/10.1007/s12350-019-01761-5
- Cardiac amyloidosis
- Appropriate use
- Expert consensus