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Echocardiography and etiology of Hypertrophic cardiomyopathy

“Application of a Parametric Display of Two-Dimensional Speckle-Tracking Longitudinal Strain to Improve the Etiologic Diagnosis of Mild to Moderate Left Ventricular Hypertrophy”

Echocardiography has a major role in the diagnostic work-up of a patient with left ventricular hypertrophy (LVH). However the distinction of hypertrophic cardiomyopathy (HCM) or cardiac amyloidosis (CA) from hypertensive heart disease (HHD) may be difficult, especially in the context of moderate LVH. The aim of this study was to determine the impact of parametric (polar) maps of regional longitudinal strain on identification of the etiology of mild to moderate left ventricular hypertrophy.
The authors analyzed twenty-four consecutive echocardiographic studies with mild to moderate LVH (eight with CA, eight with HCM, and eight with hypertensive heart disease), adequate images to assess longitudinal strain and absence of electrocardiographic criteria for low voltage or LVH or a pseudoinfarct pattern. Twenty level 3-trained readers provided the most likely of three diagnoses (CA, HCM, or HHD) and scored their confidence in making the diagnosis from two-dimensional images and diastolic parameters. A teaching exercise was provided on the interpretation of longitudinal strain in these cohorts, and interpretation was repeated with the addition of the strain polar map.
Baseline concordance among the readers was poor (κ = 0.28) and improved with the addition of strain data (κ = 0.57). Accuracy was improved with the addition of polar maps for the entire study cohort (P < 0.001), with 22% of cases reclassified correctly. The largest improvements in sensitivity (from 40% to 86%, P < 0.001), specificity (from 84% to 95%, P < 0.001), and accuracy (from 70% to 92%, P < 0.001) were seen for CA. The strain polar map significantly improved reader confidence in making the correct diagnosis overall (P < 0.001).
The authors concluded that regional variations in strain are easily recognizable, accurate, and reproducible means of differentiating causes of LVH. The detection of LVH etiology may be a useful clinical application for strain.
Myocardial Disease


Despite an important role in differentiating the causes of LVH (1), standard echocardiography techniques are not able to distinguish the etiology in a large number of cases, especially at the early phase of the diseases when the typical features are not necessarily present. Assessment of myocardial deformation or strain using 2D speckel-tracking has emerged as a potential means to differentiate hypertrophic cardiomyopathy from hypertensive heart disease, athlete’s heart, and also cardiac amyloidosis (2-5).
    The authors used an interesting method in order to evaluate the real impact of Two-Dimensional Speckle-Tracking Longitudinal Strain in that setting. They include only patients with moderate LVH (14±1mm), without obvious ECG abnormalities in favor of a given diagnosis. The assessment of the diagnosis (HCM or CA or HHD) was performed by 20 echocardiographic readers unaware of the cases. During a first session they classified the documents without data on Two-Dimensional Speckle-Tracking Longitudinal Strain but with detailed conventional echocardiography and also tissue Doppler evaluation at lateral and medial mitral annulus. During a second session they classified the patients with the help of Two-Dimensional Speckle-Tracking Longitudinal Strain. The readers also evaluated the confidence with correctness of the diagnosis according to a semi quantitative scale. The readers previously had a teaching session about the interpretation of Longitudinal Strain (LS) in the context of LVH and the representation of typical pattern of strain polar maps (eye bull representation of LS): relative apical sparing of LS with reduced LS at the basal and middle levels of LV (cardiac amyloidosis), reduced LS at the site of greatest hypertrophy (hypertrophic cardiomyopathy).

Conclusion:

A limitation of the study is the small sample size of the population (3 groups of 8 patients). A second point to underline is that no single echographic parameter will provide the magic key but requires to be interpreted in the context of various components of diagnostic work-up (including personal history and symptoms, ECG, basic biology etc) as emphazised by a recent position paper of the WG (1).
However the study demonstrates that the addition of a strain polar map to standard echocardiographic parameters in this cohort significantly improves reader concordance, diagnostic accuracy, and confidence in making the correct diagnosis. The typical representation of strain polar map is an easy tool that should help us to appropriately diagnose the etiology of LVH and therefore will facilitate the appropriate management of the patients.

References


1.    Rapezzi C et al. Diagnostic work-up in cardiomyopathies: bridging the gap between clinical phenotypes and final diagnosis. A position statement from the ESC Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013 May;34(19):1448-58.
2.    Popovic ZB, et al. Association between regional ventricular function and myocardial fibrosis in hypertrophic cardiomyopathy assessed by speckle tracking echocardiography and delayed hyperenhancement magnetic resonance imaging. J Am Soc Echocardiogr 2008;21:1299-305.
3.    Phelan D, 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.
4.    Liu D, et al. Impact of regional left ventricular function on outcome for patients with AL amyloidosis. PLoS One 2013;8:e56923.
5.    Serri K, et al. Global and regional myocardial function quantification by two dimensional strain: application in hypertrophic cardiomyopathy. J Am Coll Cardiol 2006;47:1175-81.

 






Notes to editor


Presented by: Dr Philippe Charron, Referral center for cardiac hereditary diseases, Pitié-Salpêtrière Hospital and Paris 6 University, Paris, France. 

Phelan D, Thavendiranathan P, Popovic Z, Collier P, Griffin B, Thomas JD, Marwick TH.
J Am Soc Echocardiogr. 2014 May 26. doi: 10.1016/j.echo.2014.04.015. [Epub ahead of print]
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.