Review
Echocardiographic Evaluation of Systolic and Diastolic Function in Patients With Cardiac Amyloidosis

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The typical appearance of cardiac amyloidosis using standard echocardiographic techniques is usually a late finding only in patients with relatively advanced stages of the disease. Early noninvasive identification of cardiac amyloidosis is of growing clinical importance. Newer echocardiographic techniques, including tissue Doppler imaging and deformation imaging (strain rate imaging and 2-dimensional speckle tracking), are powerful tools for quantifying regional myocardial motion and deformation. Using these advanced techniques, early functional impairment in cardiac amyloidosis may be detectable when the results of standard echocardiography are still normal or inconclusive. This review provides a comprehensive overview of the different echocardiographic approaches for the assessment of systolic and diastolic function in patients with cardiac amyloidosis. Special attention is paid to regional myocardial function assessed by tissue Doppler imaging, strain rate imaging, and 2-dimensional speckle-tracking imaging.

Section snippets

Standard Echocardiographic Findings

Conventional M-mode and 2D echocardiographic features associated with CA include concentric left ventricular (LV) and right ventricular (RV) thickening, normal LV cavity size, dilated atria, and pericardial effusion.1 The myocardial texture often features a distinct “granular sparkling” for diagnosing CA with sensitivity of 87% and specificity of 81% (Figure 1).2 The LV ejection fraction and fractional shortening in CA remain normal or nearly normal up to the late stage of the disease.

How to Assess Regional Myocardial Function

TDI provides quantification of regional myocardial function by the assessment of myocardial segmental motion and deformation. Tissue Doppler velocity can be acquired through pulsed Doppler, M-mode Doppler, or 2D color Doppler.5 The main limitation of tissue velocity data is the influence of cardiac overall motion and adjacent myocardial tethering. Deformation imaging can overcome these issues. Myocardial deformation can be demonstrated by strain rate and strain parameters derived from TDI as

Left Ventricular Systolic Dysfunction

LV global systolic function remains normal until the late stage of the disease in patients with CA.12, 13, 14 However, despite a normal LV ejection fraction and fractional shortening, longitudinal systolic function may be altered very early during disease progression.15 Thus, newer imaging techniques for the assessment of longitudinal function, such as TDI, SRI, and STI, have been suggested for the echocardiographic evaluation of patients with CA.6, 16, 17, 18, 19, 20, 21, 22, 23, 24 The recent

Doppler echocardiography

Diastolic dysfunction is a predominant feature of CA and can be evaluated using standard and Doppler echocardiography. In 1989, Klein et al35 were the first to describe the characteristics of LV filling pattern in patients with CA by transmitral inflow and pulmonary venous flow velocities using Doppler echocardiography. It thus became apparent that patients with CA exhibit various types of diastolic filling abnormalities. In early disease, wall thickness was only mildly increased, and an

Right Ventricular Dysfunction

It has been reported that RV dysfunction is common in CA and indicates poorer outcomes.50, 51 Echocardiographic evaluation of RV dysfunction is challenging because of the right ventricle's complicated geometry. In the search for integrated measures of myocardial performance, the Tei index has been proposed; it is thought to reflect an evaluation of systolic and diastolic function in combination.

In amyloid light-chain amyloidosis, patients with RV dysfunction defined by tricuspid annular plane

Implications for Cardiac Amyloidosis Management

The management of CA includes treatment of the heart failure and treatment of the underlying disease. As is known, patients with amyloid light-chain amyloidosis with cardiac involvement tend to respond poorly to conventional heart failure therapies.54 The main treatment option for heart failure in amyloid light-chain amyloidosis is the use of diuretics. In contrast, chemotherapy and stem cell transplantation can attenuate amyloid formation and lead to regression of deposits, preservation of

Conclusions

Longitudinal myocardial dysfunction can be documented in an early stage of patients with CA and might appear before congestive heart failure occurs and sometimes even in subjects with normal results on standard echocardiography. Preserved radial function can compensate the loss of longitudinal myocardial function, and therefore cardiac output is maintained at this stage. Typically, strain and strain rate in the basal and middle segments are primarily reduced in patients with CA, while

References (57)

  • D. Bellavia 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)
  • D. Bellavia et al.

    Detection of left ventricular systolic dysfunction in cardiac amyloidosis with strain rate echocardiography

    J Am Soc Echocardiogr

    (2007)
  • D. Bellavia et al.

    Independent predictors of survival in primary systemic (AL) amyloidosis, including cardiac biomarkers and left ventricular strain imaging: an observational cohort study

    J Am Soc Echocardiogr

    (2010)
  • J.P. Sun 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)
  • J.S. Schroeder et al.

    Cardiac amyloidosisDiagnosis by transvenous endomyocardial biopsy

    Am J Med

    (1975)
  • A.L. Klein et al.

    Doppler characterization of left ventricular diastolic function in cardiac amyloidosis

    J Am Coll Cardiol

    (1989)
  • C.P. Appleton et al.

    Relation of transmitral flow velocity patterns to left ventricular diastolic function: new insights from a combined hemodynamic and Doppler echocardiographic study

    J Am Coll Cardiol

    (1988)
  • J.E. Rahman et al.

    Noninvasive diagnosis of biopsy-proven cardiac amyloidosis

    J Am Coll Cardiol

    (2004)
  • A.L. Klein et al.

    Serial Doppler echocardiographic follow-up of left ventricular diastolic function in cardiac amyloidosis

    J Am Coll Cardiol

    (1990)
  • M.J. Garcia et al.

    Differentiation of constrictive pericarditis from restrictive cardiomyopathy: assessment of left ventricular diastolic velocities in longitudinal axis by Doppler tissue imaging

    J Am Coll Cardiol

    (1996)
  • G.B. Al-Zahrani et al.

    Doppler myocardial imaging compared to standard two-dimensional and Doppler echocardiography for assessment of diastolic function in patients with systemic amyloidosis

    J Am Soc Echocardiogr

    (2009)
  • B.H. Lorell et al.

    Cardiac hypertrophy: the consequences for diastole

    J Am Coll Cardiol

    (1987)
  • D.L. Brutsaert et al.

    Analysis of relaxation in the evaluation of ventricular function of the heart

    Prog Cardiovasc Dis

    (1985)
  • A.L. Klein et al.

    Comprehensive Doppler assessment of right ventricular diastolic function in cardiac amyloidosis

    J Am Coll Cardiol

    (1990)
  • A.R. Patel et al.

    Right ventricular dilation in primary amyloidosis: an independent predictor of survival

    Am J Cardiol

    (1997)
  • J.B. Selvanayagam et al.

    Evaluation and management of the cardiac amyloidosis

    J Am Coll Cardiol

    (2007)
  • R.L. Comenzo et al.

    Dose-intensive melphalan with blood stem-cell support for the treatment of AL (amyloid light-chain) amyloidosis: survival and responses in 25 patients

    Blood

    (1998)
  • A.G. Siqueira-Filho et al.

    M-mode and two-dimensional echocardiographic features in cardiac amyloidosis

    Circulation

    (1981)
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    Drs. Liu and Niemann contributed equally to this article.

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