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Multimodality imaging approach to cardiac amyloidosis: part 2

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Abstract

With recent advances in cardiac imaging, genetics, and treatment options, cardiac amyloidosis (CA) is now recognized as an important and under diagnosed condition contributing to cardiovascular morbidity and mortality. Although still considered a rare disease, CA is now recognized as an important contributor to heart failure with preserved ejection fraction (HFPEF) and low gradient aortic stenosis, two important conditions commonly faced in clinical practice. This review uses clinical scenarios to highlight the complementary role of traditional imaging tools such as electrocardiogram (ECG) and echocardiography (echo) in conjunction with advanced cardiac imaging with cardiac magnetic resonance (CMR) and nuclear cardiac scintigraphy using bone avid tracers in the comprehensive workup of CA. We also highlight the importance of workup of light chain disease as part of integration of imaging findings and discuss the key aspects of various imaging modalities. Finally, an algorithm integrating clinical suspicion, laboratory testing, and imaging in the workup of CA is presented.

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modified from references in figure. Note: Patients get monoclonal screen completed prior to PYP-SPECT being done

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References

  1. Kyle RA, Linos A, Beard CM, Linke RP, Gertz MA, O’Fallon WM, Kurland LT (1992) Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989. Blood 79(7):1817–1822 (PMID: 1558973)

    Article  CAS  Google Scholar 

  2. Muchtar E, Gertz MA, Kumar SK, Lacy MQ, Dingli D, Buadi FK et al (2017) Improved outcomes for newly diagnosed AL amyloidosis over the years 2000–2014: cracking the glass ceiling of early death. Blood 129:2111–2119

    Article  CAS  Google Scholar 

  3. Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS (2019) Transthyretin amyloid cardiomyopathy: JACC state-of-the-art review. J Am Coll Cardiol 73(22):2872–2891. https://doi.org/10.1016/j.jacc.2019.04.003.PMID:31171094;PMCID:PMC6724183

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. Gillmore JD, Damy T, Fontana M et al (2018) A new staging system for cardiac transthyretin amyloidosis. Eur Heart J 39:2799–2806

    Article  CAS  Google Scholar 

  5. Dubrey SW, Cha K, Skinner M et al (1997) Familial and primary (AL) cardiac amyloidosis: echocardiographically similar diseases with distinctly different clinical outcomes. Heart 78:74–82

    Article  CAS  Google Scholar 

  6. Siddiqi OK, Ruberg FL (2018) Cardiac amyloidosis: an update on pathophysiology, diagnosis, and treatment. Trends Cardiovasc Med 28:10–21

    Article  CAS  Google Scholar 

  7. From AM, Maleszewski JJ, Rihal CS (2011) Current status of endomyocardial biopsy. Mayo Clin Proc 86(11):1095–1102. https://doi.org/10.4065/mcp.2011.0296

    Article  PubMed  PubMed Central  Google Scholar 

  8. Fine NM, Arruda-Olson AM, Dispenzieri A, Zeldenrust SR, Gertz MA, Kyle RA, Swiecicki PL, Scott CG, Grogan M (2014) Yield of noncardiac biopsy for the diagnosis of transthyretin cardiac amyloidosis. Am J Cardiol 113(10):1723–1727. https://doi.org/10.1016/j.amjcard.2014.02.030 (Epub 2014 Mar 2 PMID: 24698461)

    Article  CAS  PubMed  Google Scholar 

  9. Palladini G, Russo P, Bosoni T, Verga L, Sarais G, Lavatelli F, Nuvolone M, Obici L, Casarini S, Donadei S et al (2009) Identification of amyloidogenic light chains requires the combination of serum-free light chain assay with immunofixation of serum and urine. Clin Chem 55:499–504. https://doi.org/10.1373/clinchem.2008.117143

    Article  CAS  PubMed  Google Scholar 

  10. Katzmann JA, Abraham RS, Dispenzieri A, Lust JA, Kyle RA (2005) Diagnostic performance of quantitative kappa and lambda free light chain assays in clinical practice. Clin Chem 51:878–881. https://doi.org/10.1373/clinchem.2004.046870

    Article  CAS  PubMed  Google Scholar 

  11. Muchtar E, Gertz MA, Kyle RA, Lacy MQ, Dingli D, Leung N, Buadi FK, Hayman SR, Kapoor P, Hwa YL et al (2019) A modern primer on light chain amyloidosis in 592 patients with mass spectrometry-verified typing. Mayo Clin Proc 94:472–483. https://doi.org/10.1016/j.mayocp.2018.08.006

    Article  CAS  PubMed  Google Scholar 

  12. Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, Salvi F, Ciliberti P, Pastorelli F, Biagini E, Coccolo F, Cooke RM, Bacchi-Reggiani L, Sangiorgi D, Ferlini A, Cavo M, Zamagni E, Fonte ML, Palladini G, Salinaro F, Musca F, Obici L, Branzi A, Perlini S (2009) Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation 120(13):1203–1212. https://doi.org/10.1161/CIRCULATIONAHA.108.843334 (Epub 2009 Sep 14 PMID: 19752327)

    Article  CAS  PubMed  Google Scholar 

  13. Rahman JE, Helou EF, Gelzer-Bell R, Thompson RE, Kuo C, Rodriguez ER, Hare JM, Baughman KL, Kasper EK (2004) Noninvasive diagnosis of biopsy-proven cardiac amyloidosis. J Am Coll Cardiol 43(3):410–415. https://doi.org/10.1016/j.jacc.2003.08.043 (PMID: 15013123)

    Article  PubMed  Google Scholar 

  14. Gertz MA, Comenzo R, Falk RH, Fermand JP, Hazenberg BP, Hawkins PN et al (2005) 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 79:319–328

    Article  Google Scholar 

  15. Carroll JD, Gaasch WH, McAdam KP (1982) Amyloid cardiomyopathy: characterization by a distinctive voltage/mass relation. Am J Cardiol 49(1):9–13. https://doi.org/10.1016/0002-9149(82)90270-3 (PMID: 6459025)

    Article  CAS  PubMed  Google Scholar 

  16. Martinez-Naharro A, Treibel TA, Abdel-Gadir A, Bulluck H, Zumbo G, Knight DS, Kotecha T, Francis R, Hutt DF, Rezk T, Rosmini S, Quarta CC, Whelan CJ, Kellman P, Gillmore JD, Moon JC, Hawkins PN, Fontana M (2017) Magnetic resonance in transthyretin cardiac amyloidosis. J Am Coll Cardiol 70(4):466–477. https://doi.org/10.1016/j.jacc.2017.05.053 (PMID: 28728692)

    Article  CAS  PubMed  Google Scholar 

  17. Mörner S, Hellman U, Suhr OB, Kazzam E, Waldenström A (2005) Amyloid heart disease mimicking hypertrophic cardiomyopathy. J Intern Med 258(3):225–230. https://doi.org/10.1111/j.1365-2796.2005.01522.x (PMID: 16115295)

    Article  PubMed  Google Scholar 

  18. Dorbala S, Bokhari S, Miller E, Bullock-Palmer R, Soman P, Thompson R (2019) ASNC practice points: 99mTechnetium-pyrophosphate imaging for transthyretin cardiac amyloidosis (American Society of Nuclear Cardiology website). Available at https://www.asnc.org/Files/Amyloid/ASNC%20Practice%20Point-99mTechnetium-Pyrophosphate.2019.pdf. Accessed February 2019

  19. Habib G, Bucciarelli-Ducci C, Caforio ALP, Cardim N, Charron P, Cosyns B et al (2017) EACVI Scientific Documents Committee. Multimodality imaging in restrictive cardiomyopathies: an EACVI expert consensus document in collaboration with the “Working Group on myocardial and pericardial diseases” of the European Society of Cardiology endorsed by the Indian Academy of Echocardiography. Eur Heart J Cardiovasc Imaging 18:1090–121

  20. Quarta CC, Solomon SD, Uraizee I, Kruger J, Longhi S, Ferlito M et al (2014) Left ventricular structure and function in transthyretin-related vs light-chain cardiac amyloidosis. Circulation 129:1840–1849

    Article  Google Scholar 

  21. Phelan D, Collier P, Thavendiranathan P, Popovic ZB, Hanna M, Plana JC et al (2012) Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart 98:1442–1448

    Article  Google Scholar 

  22. Singh V, Soman P, Malhotra S (2020) Reduced diagnostic accuracy of apical-sparing strain abnormality for cardiac amyloidosis in patients with chronic kidney disease. J Am Soc Echocardiogr 33(7):913–916. https://doi.org/10.1016/j.echo.2020.03.012 (Epub 2020 May 27 PMID: 32473842)

    Article  PubMed  Google Scholar 

  23. Pagourelias ED, Mirea O, Duchenne J, Van Cleemput J, Delforge M, Bogaert J et al (2017) Echo parameters for differential diagnosis in cardiac amyloidosis: a head-to-head comparison of deformation and non-deformation parameters. Circ Cardiovasc Imaging 10:e005588

  24. Cueto-Garcia L, Reeder GS, Kyle RA, Wood DL, Seward JB, Naessens J et al (1985) Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival. J Am Coll Cardiol 6:737–743

    Article  CAS  Google Scholar 

  25. Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER (1981) M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation 63:188–196

    Article  CAS  Google Scholar 

  26. Gonzalez-Lopez E, Gagliardi C, Dominguez F, Quarta CC, de Haro-Del Moral FJ, Milandri A et al (2017) Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths. Eur Heart J 38:1895–1904

    Article  CAS  Google Scholar 

  27. Jurcuţ R, Onciul S, Adam R, Stan C, Coriu D, Rapezzi C, Popescu BA (2020) Multimodality imaging in cardiac amyloidosis: a primer for cardiologists. Eur Heart J Cardiovasc Imaging 21(8):833–844. https://doi.org/10.1093/ehjci/jeaa063 (PMID: 32393965)

    Article  PubMed  Google Scholar 

  28. Maceira AM, Joshi J, Prasad SK, Moon JC, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ (2005) Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 111(2):186–193. https://doi.org/10.1161/01.CIR.0000152819.97857.9D (Epub 2005 Jan 3 PMID: 15630027)

    Article  PubMed  Google Scholar 

  29. Fontana M, Pica S, Reant P, Abdel-Gadir A, Treibel TA, Banypresad SM, Maestrini V, Barcella W, Rosmini S, Bulluck H, Sayed RH et al (2015) Prognostic value of late gadolinium enhancement cardiac magnetic resonance in cardiac amyloidosis. Circulation 132:1570–1579

    Article  CAS  Google Scholar 

  30. Uretsky S (2012) Cardiovascular magnetic resonance imaging in hypertrophic cardiomyopathy. Prog Cardiovasc Dis 54(6):512–516

    Article  Google Scholar 

  31. Zhu Y, Park EA, Lee W, Kim HK, Chu A, Chung JW, Park JH (2015) Extent of late gadolinium enhancement at right ventricular insertion points in patients with hypertrophic cardiomyopathy: relation with diastolic dysfunction. Eur Radiol 25(4):1190–1200. https://doi.org/10.1007/s00330-014-3390-8 (Epub 2015 Jan 18 PMID: 25597022)

    Article  PubMed  Google Scholar 

  32. Kwong RY, Heydari B, Abbasi S, Steel K, Al-Mallah M, Wu H, Falk RH (2015) Characterization of cardiac amyloidosis by atrial late gadolinium enhancement using contrast-enhanced cardiac magnetic resonance imaging and correlation with left atrial conduit and contractile function. Am J Cardiol 116(4):622–9. https://doi.org/10.1016/j.amjcard.2015.05.021. Epub 2015 May 22. PMID: 26076990; PMCID: PMC4769620

  33. Shintani Y, Okada A, Morita Y, Hamatani Y, Amano M, Takahama H, Amaki M, Hasegawa T, Ohta-Ogo K, Kanzaki H, Ishibashi-Ueda H, Yasuda S, Shimazaki C, Yoshinaga T, Yazaki M, Sekijima Y, Izumi C (2019) Monitoring treatment response to tafamidis by serial native T1 and extracellular volume in transthyretin amyloid cardiomyopathy. ESC Heart Fail 6(1):232–236. https://doi.org/10.1002/ehf2.12382. Epub 2018 Nov 27. PMID: 30478886; PMCID: PMC6352892

  34. Martinez-Naharro A, Kotecha T, Norrington K, Boldrini M, Rezk T, Quarta C, Treibel TA, Whelan CJ, Knight DS, Kellman P, Ruberg FL, Gillmore JD, Moon JC, Hawkins PN, Fontana M (2019) Native T1 and extracellular volume in transthyretin amyloidosis. JACC Cardiovasc Imaging 12(5):810–819. https://doi.org/10.1016/j.jcmg.2018.02.006 (Epub 2018 Mar 14 PMID: 29550324)

    Article  PubMed  Google Scholar 

  35. Baggiano A, Boldrini M, Martinez-Naharro A, Kotecha T, Petrie A, Rezk T et al (2020) Noncontrast magnetic resonance for the diagnosis of cardiac amyloidosis. JACC Cardiovasc Imaging 13:69–80

    Article  Google Scholar 

  36. Fontana M, Banypersad SM, Treibel TA, et al (2014) Native T1 mapping in transthyretin amyloidosis. J Am Coll Cardiol Img 7:157–165

  37. Stats MA, Stone JR (2016) Varying levels of small microcalcifications and macrophages in ATTR and AL cardiac amyloidosis: implications for utilizing nuclear medicine studies to subtype amyloidosis. Cardiovasc Pathol 25:413–417

    Article  CAS  Google Scholar 

  38. Gillmore JD, Maurer MS, Falk RH et al (2016) Non-biopsy diagnosis of cardiac transthyretin amyloidosis. Circulation 133:2404–2412

    Article  CAS  Google Scholar 

  39. Castano A, Haq M, Narotsky DL, Goldsmith J, Weinberg RL, Morgenstern R, Pozniakoff T, Ruberg FL, Miller EJ, Berk JL et al (2016) Multicenter study of planar technetium 99m pyrophosphate cardiac imaging: predicting survival for patients with ATTR cardiac amyloidosis. JAMA Cardiol 1:880–889. https://doi.org/10.1001/jamacardio.2016.2839

    Article  PubMed  Google Scholar 

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  1. Heart and Vascular Institute, Henry Ford West Bloomfield Hospital, West Bloomfield, MI, 48322, USA

    Jacqueline Sennott & Karthikeyan Ananthasubramaniam

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  1. Jacqueline Sennott
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Sennott, J., Ananthasubramaniam, K. Multimodality imaging approach to cardiac amyloidosis: part 2. Heart Fail Rev 27, 1515–1530 (2022). https://doi.org/10.1007/s10741-021-10179-6

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