Abstract
Cardiac involvement drives the prognosis and treatment in systemic amyloid. Echocardiography, the mainstay of current cardiac imaging, defines cardiac structure and function. Echocardiography, in conjunction with clinical phenotype, electrocardiogram and biomarkers (brain natriuretic peptide and troponin), provides an assessment of the likelihood and extent of cardiac involvement. Two tests are transforming our understanding of cardiac amyloidosis, bone tracer scanning and cardiovascular magnetic resonance (CMR). CMR provides a “second opinion” on the heart’s structure and systolic function with better accuracy and more precision than echocardiography but is unable to assess diastolic function and is not as widely available. Where CMR adds unique advantages is in evaluating myocardial tissue characterisation. With administration of contrast, the latest type of late gadolinium enhancement imaging (phase-sensitive inversion recovery sequence) is highly sensitive and specific with images virtually pathognomonic for amyloidosis. CMR is also demonstrating that the range of structural and functional changes in cardiac amyloid is broader than traditionally thought. CMR with T1 mapping, a relatively new CMR technique, can measure the amyloid burden and the myocyte response to infiltration (hypertrophy/cell loss) with advantages for tracking change (e.g. the wall thickness can stay the same but the composition can change) over time or during therapy. Such techniques hold great promise for advancing drug development in this arena and providing new prognostic insights. CMR with tissue characterisation is rewriting our understanding of cardiac amyloidosis and may lead to the development of new classification, therapies and prognostic systems.
Similar content being viewed by others
References
Falk RH, Comenzo RL, Skinner M (1997) The systemic amyloidoses. N Engl J Med 337(13):898–909
Meier-Ewert HK, Sanchorawala V, Berk JL, Ruberg FL (2011) Cardiac amyloidosis: evolving approach to diagnosis and management. Curr Treat Options Cardiovasc Med 13(6):528–542. doi:10.1007/s11936-011-0147-4
Audet A, Cote N, Couture C, Bosse Y, Despres JP, Pibarot P, Mathieu P (2012) Amyloid substance within stenotic aortic valves promotes mineralization. Histopathology 61(4):610–619. doi:10.1111/j.1365-2559.2012.04265.x
Rocken C, Peters B, Juenemann G, Saeger W, Klein HU, Huth C, Roessner A, Goette A (2002) Atrial amyloidosis: an arrhythmogenic substrate for persistent atrial fibrillation. Circulation 106(16):2091–2097
Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, Salvi F, Ciliberti P, Pastorelli F, Biagini E, Coccolo F, Cooke RMT, 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
Pozo E, Kanwar A, Deochand R, Castellano J, Naib T, Pazos-Lopez P, Osman K, Cham M, Narula J, Fuster V, Sanz J (2014) Cardiac magnetic resonance evaluation of left ventricular remodelling distribution in cardiac amyloidosis. Heart epub 2014 Jul 10
Bogaert J, Taylor AM (2005) Non-ischemic myocardial disease. In: Bogaert JDS, Taylor AM (eds) Clinical cardiac MRI. Springer, New York, pp 234–237
Quarta CC, Solomon SD, Uraizee I, Kruger J, Longhi S, Ferlito M, Gagliardi C, Milandri A, Rapezzi C, Falk RH (2014) Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. Circulation 129(18):1840–1849. doi:10.1161/CIRCULATIONAHA.113.006242
White SK, Sado D, Fontana M, Banypersad SM, Maestrini V, Flett AS, Piechnik SK, Robson MD, Hausenloy DJ, Sheikh AM, Hawkins PN, Moon JC (2013) T1 mapping for myocardial extracellular volume measurement by CMR: bolus only versus primed infusion technique. JACC Cardiovasc Imaging 6:955–962
Maceira AM, Joshi J, Prasad SK, Moon J, Perugini E, Harding I, Sheppard MN, Poole-Wilson PA, Hawkins PN, Pennell DJ (2005) Cardiovascular magnetic resonance in cardiac amyloidosis. Circulation 111:186–193
Syed IS, Glockner JF, Feng D, Araoz PA, Martinez MW, Edwards WD, Gertz MA, Dispenzieri A, Oh JK, Bellavia D, Tajik AJ, Grogan M (2010) Role of cardiac magnetic resonance imaging in the detection of cardiac amyloidosis. JACC Cardiovasc Imaging 3:155–164
Austin BA, Tang WH, Rodriguez ER, Tan C, Flamm SD, Taylor DO, Starling RC, Desai MY (2009) Delayed hyper-enhancement magnetic resonance imaging provides incremental diagnostic and prognostic utility in suspected cardiac amyloidosis. JACC Cardiovasc Imaging 2:1369–1377
Ruberg FL, Appelbaum E, Davidoff R, Ozonoff A, Kissinger KV, Harrigan C, Skinner M, Manning WJ (2009) Diagnostic and prognostic utility of cardiovascular magnetic resonance imaging in light-chain cardiac amyloidosis. Am J Cardiol 103:544–549
Vogelsberg H, Mahrholdt H, Deluigi CC, Yilmaz A, Kispert EM, Greulich S, Klingel K, Kandolf R, Sechtem U (2008) Cardiovascular magnetic resonance in clinically suspected cardiac amyloidosis: noninvasive imaging compared to endomyocardial biopsy. J Am Coll Cardiol 51:1022–1030
Aquaro GD, Pugliese NR, Perfetto F, Cappelli F, Barison A, Masci PG, Passino C, Emdin M (2014) Myocardial signal intensity decay after gadolinium injection: a fast and effective method for the diagnosis of cardiac amyloidosis. Int J Cardiovasc Imaging 30(6):1105–1115. doi:10.1007/s10554-014-0436-6
Dungu JN, Valencia O, Pinney JH, Gibbs SD, Rowczenio D, Gilbertson JA, Lachmann HJ, Wechalekar A, Gillmore JD, Whelan CJ, Hawkins PN, Anderson LJ (2014) CMR-based differentiation of AL and ATTR cardiac amyloidosis. JACC Cardiovasc Imaging 7:133–142
Perugini E, Guidalotti PL, Salvi F, Cooke RMT, Pettinato C, Riva L, Leone O, Farsad M, Ciliberti P, Bacchi-Reggiani L, Fallani F, Branzi A, Rapezzi C (2005) Non-invasive Etiologic diagnosis of cardiac amyloidosis using 99mTC-3,3-Diphophono-1,2-Propanodicarboxylic acid scintography. J Am Coll Cardiol 46:1076–1084
Kellman P, Arai AE, McVeigh ER, Aletras AH (2002) Phase-sensitive inversion recovery for detecting myocardial infarction using gadolinium-delayed hyperenhancement. Magn Reson Med 47:372–383
Fontana M, White SK, Banypersad SM, Sado DM, Maestrini V, Flett AS, Piechnik SK, Neubauer S, Roberts N, Moon J (2012) Comparison of T1 mapping techniques for ECV quantification. Histological validation and reproducibility of ShMOLLI versus multibreath-hold T1 quantification equilibrium contrast CMR. J Cardiovasc Magn Reson 14:88
Kellman P, Hansen M (2014) T1-mapping in the heart: accuracy and precision. J Cardiovasc Magn Reson 16(1):2–20
Sado DM, Maestrini V, Piechnik SK, Banypersad SM, White SK, Flett AS, Robson MD, Neubauer S, Ariti C, Arai AE, Kellman P, Yamamura J, Schoennagel BP, Shah F, Davis B, Trompeter S, Walker M, Porter J, Moon JC (2014) Noncontrast myocardial T(1) mapping using cardiovascular magnetic resonance for iron overload. J Magn Reson Imaging epub 2014 Aug 8
Sado DM, White SK, Piechnik SK, Banypersad SM, Treibel TA, Captur G, Fontana M, Maestrini V, Flett AS, Robson MD, Lachmann RH, Murphy E, Mehta A, Hughes DA, Neubauer S, Elliott PM, Moon JC (2013) Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping. Circ Cardiovasc Imaging 6:392–398
Thompson RB, Chow K, Khan A, Chan A, Shanks M, Paterson I, Oudit GY (2013) T1 mapping with cardiovascular MRI is highly sensitive for fabry disease independent of hypertrophy and sex. Circ Cardiovasc Imaging 6:637–645
Banypersad SM, Sado DN, Flett AS, Gibbs SD, Pinney JH, Maestrini V, Cox AT, Fontana M, Whelan CJ, Wechalekar AD, Hawkins PN, Moon JC (2013) Quantification of myocardial extracellular volume fraction in systemic AL amyloidosis: an equilibrium contrast cardiovascular magnetic resonance study. Circ Cardiovasc Imaging 6:34–39
Fontana M, Banypersad SM, Treibel TA, Maestrini V, Sado DM, White SK, Pica S, Castelletti S, Piechnik SK, Robson MD, Gilbertson JA, Rowczenio D, Hutt DF, Lachmann HJ, Wechalekar AD, Whelan CJ, Gillmore JD, Hawkins PN, Moon JC (2014) Native T1 mapping in transthyretin amyloidosis. JACC Cardiovasc Imaging 7:157–165
Karamitsos TD, Piechnik SK, Banypersad SM, Fontana M, Ntusi NB, Ferreira VM, Whelan CJ, Myerson SG, Robson MD, Hawkins PN, Neubauer S, Moon JC (2013) Noncontrast T1 mapping for the diagnosis of cardiac amyloidosis. JACC Cardiovasc Imaging 6:488–497
Moon JC, Messroghli DR, Kellman P, Piechnik SK, Robson MD, Ugander M, Gatehouse PD, Arai AE, Friedrich MG, Neubauer S, Schulz-Menger J, Schelbert EB (2013) Society for cardiovascular Magnetic Resonance Imaging; Cardiovascular Magnetic Resonance Working Group of the European Society of Cardiology. Myocardial T1 mapping and extracellular volume quantification: a Society for Cardiovascular Magnetic Resonance (SCMR) and CMR Working Group of the European Society of Cardiology consensus statement. J Cardiovasc Magn Reson 15(1):92
Lee JJ, Liu S, Nacif MS, Ugander M, Han J, Kawel N, Sibley CT, Kellman P, Arai AE, Bluemke DA (2011) Myocardial T1 and extracellular volume fraction mapping at 3 tesla. J Cardiovasc Magn Reson 13:75–85
Roujol S, Weingärtner S, Foppa M, Chow K, Kawaji K, Ngo LH, Kellman P, Manning WJ, Thompson RB, Nezafat R (2014) Accuracy, precision, and reproducibility of four T1 mapping sequences: a head-to-head comparison of MOLLI, ShMOLLI, SASHA, and SAPPHIRE. Radiology 4(4):140296
Fontana M, Banypersad S, Treibel TA, Maestrini V, Sado DN, White SK, Bulluck H, Herrey AS, Hawkins PN, Moon JC (2014) AL and ATTR cardiac amyloid are different: native T1 mapping and ECV detect different biology. J Cardiovasc Magn Reson 16(Suppl 1):P341
Mongeon FP, Jerosch-Herold M, Coelho-Filho OR, Blankstein R, Falk RH, Kwong RY (2012) Quantification of extracellular matrix expansion by CMR in infiltrative heart disease. JACC Cardiovasc Imaging 5(9):897–907. doi:10.1016/j.jcmg.2012.04.006
Quarta CC, Guldalotti PL, Longhi S, Pettinato C, Leone O, Felrini A, Blagini E, Grigioni F, Bacchi-Reggiani ML, Lorenzini M, Milandri A, Branzi A, Rapezzi C (2012) Defining the diagnosis in echocardiographically suspected senile systemic amyloidosis. J Am Coll Cardiol Imaging 5:755–758
Koyama J, Falk RH (2010) Prognostic significance of strain Doppler imaging in light-chain amyloidosis. JACC Cardiovasc Imaging 3:333–342
Palka P, Lange A, Fleming AD, Donnelly JE, Dutka DP, Starkey IR, Shaw TR, Sutherland GR, Fox KA (1997) Differences in myocardial velocity gradient measured throughout the cardiac cycle in patients with hypertrophic cardiomyopathy, athletes and patients with left ventricular hypertrophy due to hypertension. J Am Coll Cardiol 30:760–768
Biagini E, Spirito P, Rocchi G, Ferlito M, Rosmini S, Lai F, Lorenzini M, Terzi F, Bacchi-Reggiani L, Boriani G, Branzi A, Boni L, Rapezzi C (2009) Prognostic implications of the Doppler restrictive filling pattern in hypertrophic cardiomyopathy. Am J Cardiol 104:1727–1731
Kwong RY, Falk RH (2005) Cardioavascular magnetic resonance in cardiac amyloidosis. Circulation 111:122–124
White JA, Kim HW, Shah D, Fine N, Kim KY, Wendell DC, Al-Jaroudi W, Parker M, Patel M, Gwadry-Sridhar F, Judd RM, Kim RJ (2014) CMR imaging with rapid visual T1 assessment predicts mortality in patients suspected of cardiac amyloidosis. JACC Cardiovasc Imaging 7:143–156
Fontana M, Pica S, Reant P, Abdel-Gadir A, Treibel TA, Banypersad S, Maestrini V, Lane T, Lachmann HJ, Whelan CJ, Wechalekar AD, Manisty C, Herrey AS, Gillmore JD, Hawkins PN, Moon JC (2014) Cardiovascular magnetic resonance in cardiac amyloidosis: a 242 patient prospective study. Am Heart Assoc Sci Sess Abstract accepted
Banypersad SM, Fontana M, Maestrini V, Sado DM, Captur G, Petrie A, Piechnik SK, Whelan CJ, Herrey AS, Gillmore JD, Lachmann HJ, Wechalekar AD, Hawkins PN, Moon JC (2014) T1 mapping and survival in systemic light-chain amyloidosis. Eur Heart J. doi:10.1093/eurheartj/ehu444
Alnylam (2013) Phase 2 study to evaluate ALN-TTRSC in patients with transthyretin (TTR) cardiac amyloidosis. http://www.clinicaltrials.gov/ct2/show/NCT01981837?term=alnylam&rank=2
GlaxoSmithKline (2013) A study to evaluate the safety of GSK2398852 when co-administered with GSK2315698 in patients with systemic amyloidosis. http://clinicaltrials.gov/show/NCT01777243
Leone O, Longhi S, Quarta C, Ragazzini T, De Giorgi L, Pasquale F, Potena L, Lovato L, Milandri A, Arpesella G, Rapezzi C (2012) New pathological insights into cardiac amyloidosis: implications for non-invasive diagnosis. Amyloid 19:99–105
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Fontana, M., Chung, R., Hawkins, P.N. et al. Cardiovascular magnetic resonance for amyloidosis. Heart Fail Rev 20, 133–144 (2015). https://doi.org/10.1007/s10741-014-9470-7
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10741-014-9470-7