Skip to main content
Log in

Echocardiographic Findings in Cardiac Amyloidosis: Inside Two-Dimensional, Doppler, and Strain Imaging

  • Echocardiography (JM Gardin and AH Waller, Section Editors)
  • Published:
Current Cardiology Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

The aim is to provide a description of the most important echocardiographic features in systemic amyloidosis.

Recent Findings

Amyloidosis is a heterogeneous group of multisystem disorders, characterized by an extracellular deposition of amyloid fibrils. Several imaging tests are available for the diagnosis; however, echocardiography is the cornerstone of the non-invasive imaging modality for cardiac amyloidosis. So far, little is known about the diagnosis of cardiac amyloidosis through imaging modalities.

Summary

We summarized the most important echocardiographic findings in cardiac amyloidosis. Hence, we offered a systematic report of the diagnostic performance of cardiac amyloidosis using echocardiography.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Primer on the rheumatic diseases. In JohnH. Klippel, John H. Stone, L Eslie J. Crofford, Patience H. White. Patience H. White, editors. 28th chapter. Springer-Verlag New York. 2008

  2. Mazzeo A, Russo M, Di Bella G, Minutoli F, Stancanelli C, Gentile L, et al. Transthyretin-related familial amyloid polyneuropathy (TTR-FAP): a single-center experience in Sicily, an Italian endemic area. J Neuromuscul Dis. 2015;2(s2):S39–48.

    Article  Google Scholar 

  3. Russo M, Mazzeo A, Stancanelli C, Di Leo R, Gentile L, Di Bella G, et al. Transthyretin-related familial amyloidotic polyneuropathy: description of a cohort of patients with Leu64 mutation and late onset. J Peripher Nerv Syst. 2012;17(4):385–90.

    Article  CAS  Google Scholar 

  4. Banypersad SM, Moon JC, Whelan C, Hawkins PN, Wechalekar AD. Updates in cardiac amyloidosis: a review. J Am Heart Assoc. 2012;1:e000364 originally published April 23, 2012.

    Article  Google Scholar 

  5. Flodrova P, Flodr P, Pika T, Vymetal J, Holub D, Dzubak P, et al. Cardiac amyloidosis: from clinical suspicion to morphological diagnosis. Pathology. 2018 Apr;50(3):261–8.

    Article  CAS  Google Scholar 

  6. Knight DS,Zumbo G, Barcella W, Steeden JA, Muthurangu V, Martinez-Naharro A, Treibel TA, Abdel-Gadir, et al. Cardiac structural and functional consequences of amyloid deposition by cardiac magnetic resonance and echocardiography and their prognostic roles. JACC Cardiovasc Imaging. 2018.

  7. Mohty D, Damy T, Cosnay P, Echahidi N, Casset-Senon D, Virot P, et al. Cardiac amyloidosis: updates in diagnosis and management. Arch Cardiovasc Dis. 2013;106(10):528–40.

    Article  Google Scholar 

  8. Kotecha T, Martinez-Naharro A, Treibel TA, Francis R, Nordin S, Abdel-Gadir A, et al. Myocardial edema and prognosis in amyloidosis. J Am Coll Cardiol. 2018;71(25):2919–31.

    Article  Google Scholar 

  9. • Stancanelli C, Gentile L, Di Bella G, Minutoli F, Russo M, Vita G, et al. Phenotypic variability of TTR Val122Ile mutation: a Caucasian patient with axonal neuropathy and normal heart. Neurol Sci. 2017;38(3):525–6 This study shows some phenotypical features of TTR amyloidosis.

    Article  Google Scholar 

  10. •• Rapezzi C, Merlini G, Quarta CC, Riva L, Longhi S, Leone O, et al. Systemic cardiac amyloidoses: disease profiles and clinical courses of the 3 main types. Circulation. 2009;120:1203–12 This study provides a global description of systemic cardiac amyloidosis.

    Article  CAS  Google Scholar 

  11. Mesquita ET, Jorge AJL, Junior CVS, de Andrade TR. Cardiac amyloidosis and its new clinical phenotype: heart failure with preserved ejection fraction. Arq Bras Cardiol. 2017;109(1):71–80.

    PubMed  PubMed Central  Google Scholar 

  12. Ruberg FL. Cardiac amyloidosis: a zebra hiding in plain sight? Circ Cardiovasc Imaging. 2017;10:e006186.

    Article  Google Scholar 

  13. •• Vogelsberg H, Mahrholdt H, Deluigi CC, Yilmaz A, Greulich S, et al. Cardiovascular magnetic resonance in clinically suspected cardiac amyloidosis: noninvasive imaging compared to endomyocardial biopsy. J Am Coll Cardiol. 2008;51(10):1022–30 This study is important because shows the diagnostic power of cardiac magnetic resonance in amyloidosis.

    Article  Google Scholar 

  14. • Di Bella G, Minutoli F, Mazzeo A, Vita G, Oreto G, Carerj S, et al. MRI of cardiac involvement in transthyretin familial amyloid polyneuropathy. AJR Am J Roentgenol. 2010;195:W394–9 This study demonstrates the diagnostic importance of MRI in cardiac amyloidosis.

    Article  Google Scholar 

  15. • Minutoli F, Di Bella G, Mazzeo A, Donato R, Russo M, Scribano E, et al. Comparison between (99m)Tc-diphosphonate imaging and MRI with late gadolinium enhancement in evaluating cardiac involvement in patients with transthyretin familial amyloid polyneuropathy. AJR Am J Roentgenol. 2013;200(3):W256–65 The study provides a view of two diagnostic tools in cardiac amyloidosis.

    Article  Google Scholar 

  16. Maceira AM, Prasad SK, Hawkins PN, Roughton M, Pennell DJ. Cardiovascular magnetic resonance and prognosis in cardiac amyloidosis. J Cardiovasc Magn Reson. 2008;10:54.

    Article  Google Scholar 

  17. Jason N. Dungu, Oswaldo Valencia, Jennifer H. Pinney, Simon D.J. Gibbs, Dorota Rowczenio, Janet A. Gilbertson, Helen J. Lachmann, et al. CMR-Based Differentiation of AL and ATTR Cardiac Amyloidosis. JACC: Cardiovascular Imaging. 2014;7(2):133–142).

  18. Minutoli F, Di Bella G, Sindoni A, Vita G, Baldari S. Effectiveness of skeletal scintigraphy in transthyretin-related amyloidosis. Int J Cardiol. 2013;168(5):4988–9.

    Article  Google Scholar 

  19. •• Bokhari S, Castanõ A, Pozniakoff T, Deslisle S, Latif F, Maurer MS. (99m) Tcpyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidoses. Circ Cardiovasc Imaging. 2013;6:195–201 This study provides the way for differentiating the various types of amyloidosis.

    Article  Google Scholar 

  20. Bokhari S, Castaño A, Pozniakoff T, Deslisle S, Latif F, Maurer MS. 99mTc-Pyrophosphate scintigraphy for differentiating light-chain cardiac amyloidosis from the transthyretin-related familial and senile cardiac amyloidosis. Circ Cardiovasc Imaging. 2013;6(2):195–201.

    Article  Google Scholar 

  21. • Quarta CC, Solomon SD, Uraizee I, Kruger J, Longhi S, Ferlito M, et al. Left ventricular structure and function in transthyretin-related versus light-chain cardiac amyloidosis. Circulation. 2014;129:1840–9 This study provides a differentiation of two types of Cardiac Amyloidosis.

    Article  Google Scholar 

  22. •• Cacciapuoti F. The role of echocardiography in the non-invasive diagnosis of cardiac amyloidosis. J Echocardiogr. 2015;13(3):84–9. https://doi.org/10.1007/s12574-015-0249-1 In this review the most common echocardiographic features of CA are summarised.

    Article  PubMed  Google Scholar 

  23. Siqueira-Filho AG, Cunha CL, Tajik AJ, Seward JB, Schattenberg TT, Giuliani ER. M-mode and two-dimensional echocardiographic features in cardiac amyloidosis. Circulation. 1981;63:188–96.

    Article  CAS  Google Scholar 

  24. •• Di Bella G, Pizzino F, Minutoli F, Zito C, Donato R, Dattilo G, et al. The mosaic of the cardiac amyloidosis diagnosis: role of imaging in subtypes and stages of the disease. Eur Heart J Cardiovasc Imaging. 2014;15(12):1307–15 The review depicts the changes in cardiac imaging among the different stages of the disease.

    Article  Google Scholar 

  25. Koyama J, Davidoff R, Falk RH. Longitudinal myocardial velocity gradient derived frompulsed Doppler tissue imaging in AL amyloidosis: a sensitive indicator of systolic and diastolic dysfunction. J Am Soc Echocardiogr. 2004;17:36–44.

    Article  Google Scholar 

  26. Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation. 2003;107:2446–52.

    Article  Google Scholar 

  27. Bellavia D, Pellikka PA, Abraham TP, Al-Zahrani GB, Dispenzieri A, Oh JK, et al. Evidence of impaired left ventricular systolic function by Doppler myocardial imaging in patients with systemic amyloidosis and no evidence of cardiac involvement by standard two-dimensional and Doppler echocardiography. Am J Cardiol. 2008;101:1039–45.

    Article  Google Scholar 

  28. Di Bella G, Minutoli F, Piaggi P, Casale M, Mazzeo A, Zito C, et al. Usefulness of combining electrocardiographic and echocardiographic findings and brain natriuretic peptide in early detection of cardiac amyloidosis in subjects with transthyretin gene mutation. Am J Cardiol. 2015;116(7):1122–7.

    Article  Google Scholar 

  29. • Carroll JD, Gaasch WH, McAdam KP. Amyloid cardiomyopathy: characterization by a distinctive voltage/mass relation. Am J Cardiol. 1982;49(1):9–13 The review explains the differences in the structure of the left ventricular hypertrophy.

    Article  CAS  Google Scholar 

  30. •• Cacciapuoti F. The role of echocardiography in the non-invasive diagnosis of cardiac amyloidosis. J Echocardiogr. 2015;13(3):84–9 This is a complete review of the diagnostic power of echocardiography in amyloidosis.

    Article  Google Scholar 

  31. •• Koyama J, Ray-Sequin PA, Falk RH. Prognostic significance of ultrasound myocardial tissue characterization in patients with cardiac amyloidosis. Circulation. 2002;106(5):556–61 This study demonstrates that myocardial tissue plays a diagnostic and prognostic role in cardiac amyloidosis.

    Article  Google Scholar 

  32. • Tei C, Dujardin KS, Hodge DO, Kyle RA, Tajik AJ, Seward JB. Doppler index combining systolic and diastolic myocardial performance: clinical value in cardiac amyloidosis. J Am Coll Cardiol. 1996;28(3):658–64 This is one of the most complete study about the diastolic disfunction in cardiac amyloidosis.

    Article  CAS  Google Scholar 

  33. Klein AL, Hatle LK, Burstow DJ, et al. Doppler characterization of left ventricular diastolic function in cardiac amyloidosis. J Am Coll Cardiol. 1989;1(3):1017–26.

    Article  Google Scholar 

  34. •• Koyama J, Ray-Sequin PA, Falk RH. Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation. 2003;107:2446–52 This is a complete view about longitudinal ventricular function in patients with cardiac amyloidosis.

    Article  Google Scholar 

  35. • Di Bella G, Minutoli F, Piaggi P, Casale M, Mazzeo A, Zito C, et al. Quantitative comparison between amyloid deposition detected by (99m)Tc-diphosphonate imaging and myocardial deformation evaluated by strain echocardiography in transthyretin-related cardiac amyloidosis. Circ J. 2016;80(9):1998–2003 This study provides a multiparametric description of the amyloid deposition.

    Article  Google Scholar 

  36. • Di Bella G, Minutoli F, Pingitore A, Zito C, Mazzeo A, Aquaro GD, et al. Endocardial and epicardial deformations in cardiac amyloidosis and hypertrophic cardiomyopathy. Circ J. 2011;75(5):1200–8 The review specifies the different motion of epicardial end endocardial layers.

    Article  Google Scholar 

  37. Bellavia D, Abraham TP, Pellikka PA, Al-Zahrani GB, Dispenzieri A, Oh JK, et al. Detection of left ventricular systolic dysfunction in cardiac amyloidosis with strain rate echocardiography. J Am Soc Echocardiogr. 2007;20(10):1194–202.

    Article  Google Scholar 

  38. Belkin RN, Kupersmith AC, Kalique O, et al. A novel two-di- mensional echocardiographic finding in cardiac amyloidosis. Echocardiography. 2010;27:1171–6.

    Article  Google Scholar 

  39. •• Phelan D, Collier P, Thavendiranathan P, Popović ZB, Hanna M, Plana JC, et al. Relative apical sparing of longitudinal strain using two-dimensional speckle-tracking echocardiography is both sensitive and specific for the diagnosis of cardiac amyloidosis. Heart. 2012;98:1442–8 This is a complete and detailed view about apical sparing in cardiac amyloidosis.

    Article  Google Scholar 

  40. Lee GY, Kim HK, Choi JO, Chang SA, Oh JK, Jeon ES, et al. Visual assessment of relative apical sparing pattern is more useful than quantitative assessment for diagnosing cardiac amyloidosis in borderline or mildly increased left ventricular wall thickness. Circ J. 2015;79(7):1575–84.

    Article  CAS  Google Scholar 

  41. Austin BA, Tang WH, Rodriguez ER, et al. Delayed hyper-enhancement magnetic resonance imaging provides incremental diagnostic and prognostic utility in suspected cardiac amyloidosis. JACC Cardiovasc Imaging. 2009;2:1369e77.

    Article  Google Scholar 

  42. Porciani MC, Lilli A, Perfetto F, Cappelli F, Massimiliano Rao C, Del Pace S, et al. Tissue Doppler and strain imaging: a new tool for early detection of cardiac amyloidosis. Amyloid. 2009;16(2):63–70.

    Article  Google Scholar 

  43. • Plehn JF, Huntington M, Skinner M, Cohen A. Valvular regurgitation in primary amyloidosis: an early sign of cardiac involvement (abstr). Clin Res. 1988;36:308A This study explains the valvular involvement in the early stage of amyloidosis.

    Google Scholar 

  44. Plehn J, Dilsizian V, Skinner M, Cohen AS. Ventricular diastolic abnormalities in primary amyloidosis without obvious cardiac involvement (abstr). Circulation. 1986;74(suppl II):II-1800.

    Google Scholar 

  45. Hongo M, Fujii T, Jirayama J, et al. Radionuclide angiographic assessment of left ventricular diastolic filling in amyloid heart disease: a study of patients with familial amyloid polyneuropathy. J Am Coll Cardiol. 1989;13:4853.

    Article  Google Scholar 

  46. Plehn JF, Facc, Friedman BJ. Diastolic dysfunction in amyloid heart disease: restrictive cardiomyopathy or not? Editorial Comment JACC. 1989;13(I):6.

    Google Scholar 

  47. Di Bella G, Minutoli F, Madaffari A, Mazzeo A, Russo M, Donato R, et al. Left atrial function in cardiac amyloidosis. J Cardiovasc Med (Hagerstown). 2016;17(2):113–21.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Dalia Di Nunzio.

Ethics declarations

Conflict of Interest

Dalia Di Nunzio, Antonino Recupero, Cesare de Gregorio, Concetta Zito, Scipione Carerj, and Gianluca Di Bella declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Echocardiography

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Di Nunzio, D., Recupero, A., de Gregorio, C. et al. Echocardiographic Findings in Cardiac Amyloidosis: Inside Two-Dimensional, Doppler, and Strain Imaging. Curr Cardiol Rep 21, 7 (2019). https://doi.org/10.1007/s11886-019-1094-z

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11886-019-1094-z

Keywords

Navigation