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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 1  |  Issue : 2  |  Page : 163-166

Speckle tracking as an adjunctive echocardiographic technique for differentiating cardiac amyloidosis from hypertrophic cardiomyopathy: A case study


Department of Cardiology, Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, India

Date of Web Publication28-Aug-2017

Correspondence Address:
Sandya Nandakumar
Amrita Institute of Medical Sciences and Research Centre, Ponekkara P.O., Kochi - 682 041, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_59_17

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  Abstract 


Cardiac Amyloidosis (CA) often requires multiple diagnostic modalities with echocardiogram being the most important non-invasive method. Echocardiographic findings of left ventricular hypertrophy (LVH), impaired relaxation and dilated atrias can be seen in others forms of LVH like hypertrophic cardiomyopathy (HCM). Speckle tracking echocardiography (STE), is a relatively new advancement in echocardiography which makes it possible to assess the deformation of the left ventricle and can be utilized for differentiating CA from other forms of LVH. We demonstrate the incremental value provided by STE in distinguishing CA from HCM.

Keywords: Cardiac amyloidosis, hypertrophic cardiomyopathy, speckle tracking


How to cite this article:
Nandakumar S, Chandrasekharan R, Vijayakumar M, Thachathodiyl R. Speckle tracking as an adjunctive echocardiographic technique for differentiating cardiac amyloidosis from hypertrophic cardiomyopathy: A case study. J Indian Acad Echocardiogr Cardiovasc Imaging 2017;1:163-6

How to cite this URL:
Nandakumar S, Chandrasekharan R, Vijayakumar M, Thachathodiyl R. Speckle tracking as an adjunctive echocardiographic technique for differentiating cardiac amyloidosis from hypertrophic cardiomyopathy: A case study. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2017 [cited 2020 Sep 29];1:163-6. Available from: http://www.jiaecho.org/text.asp?2017/1/2/163/213681




  Introduction Top


Infiltration of different types of misfolded precursor proteins results in dysfunction of myocardium in cardiac amyloidosis (CA). The most common presentation of CA is heart failure.[1],[2],[3] Often, the diagnosis is difficult, especially when the involvement is predominantly cardiac. Cardiac involvement varies in different types of amyloidosis; however, there are few types of amyloidosis which predominantly affects the cardiac muscles: (a) light-chain (AL type), (b) transthyretin-related amyloidosis (ATTR) or familial type, and (c) secondary amyloidosis.[3] The deposition of amyloid impairs the myocardial performance leading to both diastolic as well as systolic dysfunction.

Cardiac involvement results in characteristic morphological and functional changes in the myocardium which can be identified by echocardiography.[4] Other pathological states that resemble amyloidosis are hypertrophic cardiomyopathy (HCM), hypertensive heart disease, aortic stenosis, and other condition that increases the afterload of the heart. At times, the differentiation from other forms of left ventricular hypertrophy (LVH) becomes difficult and may require additional new echocardiographic techniques for diagnosis.[5],[6]

Strain imaging is a modality which could be used to differentiate the amyloid deposition from other conditions noninvasively.[5] The longitudinal strain (LS) which is expressed as deformation during systolic shortening of base-to-apex length is observed in apical projections. The muscle shortening is higher in the apical region and in the middle portion compared to the base which can be observed as a strain gradient in the base–apex region.[5] CA profoundly alters all the strain parameters compared with other cause of LVH.[7] In CA, relatively less amyloid deposition occurs at the apex than at the base and mid segment; hence, there is less resistance to deformation resulting in normal strain at the apical segment and reduced strain in mid and basal segments resulting in relative sparing of apical strain.[5] We report a case of CA where we used STE to demonstrate this pattern and how it helps to differentiate it from HCM.


  Case Report Top


A 66-year-old female presented with a history of progressive dyspnea and pedal edema of 8 months' duration. Clinical picture was suggestive of congestive heart failure. ECG was showed low-voltage complex. Serum protein electrophoresis showed increased beta-band.

M mode echocardiogram (Vivid 9 ultrasound system, GE medical, Milwaukee, Wisconsin, USA) showed smallish left ventricular (LV) cavity, thickening of myocardium with reduced systolic thickening, and significantly reduced amplitude of excursion of tricuspid annulus of 5 mm.

2D echocardiogram revealed granular speckled appearance of the myocardium, biatrial enlargement, thickened papillary muscles, thickening of valves, and thickened interatrial septum [Figure 1]. The right ventricular free wall was also thickened [Figure 2]. Small circumferential pericardial effusion was also noted.
Figure 1: Biatrial enlargement and left ventricular hypertrophy in cardiac amyloidosis

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Figure 2: Right ventricular free wall hypertrophy

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3D echocardiographic volume analysis revealed reduced end-diastolic volume (42 ml), end-systolic volume (26 ml), and decreased stroke volume (16 ml) with an ejection fraction (EF) of 38% [Figure 3]. Thickening of mitral valve, aortic valve, and tricuspid valve was more evident on 3D echocardiography [Figure 4].
Figure 3: Three-dimensional volume analysis of cardiac amyloidosis showing reduced volumes and ejection fraction

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Figure 4: Three-dimensional imaging showing thickened mitral valve and tricuspid valve

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Mitral Doppler showed an E/A ratio of 2.64, deceleration time of 141 ms, and isovolumic relaxation time of 63 ms. Pulmonary vein Doppler showed systolic fraction of 10% and S<<D. These findings were consistent with Grade III diastolic dysfunction.

Mitral E velocity/tissue Doppler e velocity ratio of 35 indicated increased LV diastolic pressure and hence elevated pulmonary venous pressure. S' at inferoseptum was measured as 2 cm/s and anterolateral S'2 cm/s indicating severely impaired longitudinal systolic function [Figure 5].
Figure 5: Tissue Doppler showing significant reduction in S and e' velocities of lateral annulus

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LS measurements were performed using automated functional imaging advanced analysis technologies, GE medical, at a frame rate of 62 frames/s. In our patient, there was reduced global LS (GLS) with typical apical sparing pattern [Figure 6]. The polar map shows markedly reduced GLS with good LS in the apical region.
Figure 6: Strain analysis showing apical sparing in cardiac amyloidosis

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Using the same ultrasound platform, echocardiographic study of a patient with apical HCM was performed for comparison, which revealed hypertrophied apex and reduction in the LS at the apex [Figure 7] and [Table 1].
Figure 7: Two-dimensional echocardiography images of the apical hypertrophic cardiomyopathy showing hypertrophy of the apex

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Table 1: Comparison of CA and HCM

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  Discussion Top


The definite diagnosis of CA is arrived by an endomyocardial biopsy or by noncardiac positive biopsy with typical classical cardiovascular magnetic resonance (CMR) imaging or electrocardiographic findings, clinical history, and conventional echocardiographic findings.[3] CMR with classical late gadolinium enhancement is utilized as a diagnostic modality in many patients unless there is a contraindication for CMR.[8]

Many of the contemporary echocardiographic parameters are helpful in arriving at a diagnosis of CA along with symptoms and electrocardiographic changes, and this includes LV wall thickening, biatrial enlargement, and restrictive filling pattern. Speckled appearance of myocardium is suggestive of CA. Strain and strain-rate imaging can identify the myocardial dysfunction in early stages of CA, but it is not specific. GLS permits quantification of LV function, with greater sensitivity than EF. Greater sensitivity of GLS over EF has resulted in the suggestion that it is useful as an earlier marker of change for many cardiomyopathies. CA is characterized by regional variations in LS from base to apex. In our patient, the LS was reduced with apical sparing as seen in polar map.

CMR of this patient showed typical features of CA with diffuse enhancement and whitish appearance of the normally dark myocardium. Less resistance to deformation because of less extracellular deposition along with the process of dynamic reciprocity and increased myocardial contraction results in relative apical sparing.[9]

GLS is affected earlier than radial and circumferential strain.[10] Amyloidosis affects epicardial strain earlier and without segmental variation compared to endocardial strain. In comparison to HCM, the apical sparing was so evident in our CA patient. Apical HCM showed regional variation in strain with severely impaired apical strain [Figure 8] due to abnormalities in myocardial mechanics that are related to the abnormal myocardial hypertrophy at the apex.[10],[11]
Figure 8: Strain analysis showing impaired apical strain in apical hypertrophic cardiomyopathy

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  Conclusion Top


STE is helpful to detect the regional variation in strain and can be used as a tool to differentiate CA from other conditions leading to LVH.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Falk RH, Comenzo RL, Skinner M. The systemic amyloidoses. N Engl J Med 1997;337:898-909.  Back to cited text no. 1
    
2.
Merlini G, Bellotti V. Molecular mechanisms of amyloidosis. N Engl J Med 2003;349:583-96.  Back to cited text no. 2
    
3.
Koyama J, Ikeda S, Ikeda U. Echocardiographic assessment of the cardiac amyloidoses. Circ J 2015;79:721-34.  Back to cited text no. 3
    
4.
Fitzgerald BT, Bashford J, Scalia GM. Regression of the anatomic cardiac features of amyloid light chain cardiac amyloidosis accompanied by normalization of global longitudinal strain. Cardiovascular Imaging Case Reports 2017;1:46-7.  Back to cited text no. 4
    
5.
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.  Back to cited text no. 5
    
6.
Tsang W, Lang RM. Echocardiographic evaluation of cardiac amyloid. Curr Cardiol Rep 2010;12:272-6.  Back to cited text no. 6
    
7.
Sun JP, Stewart WJ, Yang XS, Donnell RO, Leon AR, Felner JM, et al. Differentiation of hypertrophic cardiomyopathy and cardiac amyloidosis from other causes of ventricular wall thickening by two-dimensional strain imaging echocardiography. Am J Cardiol 2009;103:411-5.  Back to cited text no. 7
    
8.
Tuzovic M, Yang EH, Baas AS, Depasquale EC, Deng MC, Cruz D, et al. Cardiac amyloidosis: Diagnosis and treatment strategies. Curr Oncol Rep 2017;19:46.  Back to cited text no. 8
    
9.
Williams LK, Forero J, Calleja A, Delgado D, Crean AM, Rakowski H, et al. “Apical sparing” Longitudinal strain pattern with cardiac MRI correlates with LGE distribution in cardiac Amyloidosis. Circulation 2015;132:A18015.  Back to cited text no. 9
    
10.
Bhatti S, Vallurupalli S, Ambach S, Magier AZ, Hakeem A, Mazur W. Determination of strain pattern in patients with cardiac amyloidosis secondary to multiple myeloma: A feature tracking study. J Cardiovasc Magn Reson 2016;18 Suppl 1:P273.  Back to cited text no. 10
    
11.
Liu D, Hu K, Nordbeck P, Ertl G, Störk S, Weidemann F, et al. Longitudinal strain bull's eye plot patterns in patients with cardiomyopathy and concentric left ventricular hypertrophy. Eur J Med Res 2016;21:21.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
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