Two‐Dimensional Echocardiography in Myocardial Amyloidosis

Abstract

Two-dimensional echocardiography is the best means of identifying early cardiac amyloid infiltration and gauging its subsequent progression. The early asymptomatic phase is characterized on echocardiography by a mild-to-moderate increase in left ventricular and/or right ventricular wall thicknesses. The distinctive combination of low electrocardiography voltage and increase in left ventricular mass on the echocardiogram, both compatible with substantial amyloid infiltration, is valuable in diagnosis and appears to indicate the severity of the disease. Other ancillary but common findings are left atrial dilatation, a small pericardial effusion, thickening of cardiac valves, papillary muscles, and interatrial septum. Finally, there is a peculiar texture of myocardial walls, with highly refractile areas that are typical, although not specific, of myocardial amyloidosis and can also be quantitatively described by digital image analysis techniques. The echocardiographic appearance of amyloidosis can closely mimic several other diseases. Asymmetric hypertrophy of the septum due to amyloid deposition may occur, simulating hypertrophic cardiomyopathy. The granular sparkling of myocardial walls is also found in myocarditis with severe fibrosis, and it is quite common in hypertrophic cardiomyopathy, as well as in other infiltrative diseases of the myocardium. It is not uncommon that the echocardiographic examination represents a turning point in the work-up of the patient, briskly orienting the clinician towards the correct diagnostic pathway. However, the likelihood of the cardiologist-echocardiographer to successfully and prospectively identify myocardial amyloidosis is substantially higher if all the clinical and electrocardiographic information is reviewed at the time of the echocardiographic examination.

Noninvasive diagnosis of biopsy-proven cardiac amyloidosis

“…Valvular dysfunction from amyloid deposition may also occur. Cardiac amyloidosis is associated with a variable but generally poor prognosis (1), and early recognition may improve the outcome.…”

Left atrial size—Another Differentiator for Cardiac Amyloidosis

“…The overall picture of thickened walls with a granular "sparkled" or "speckled" appearance, with normal ventricular cavity size, and pericardial effusions are the characteristic findings [1][2][3]. The combination of lowvoltage QRS on electrocardiography with thickened walls on echocardiogram has been shown to be useful to discriminate amyloidosis from hypertension [7]. All of these require multiple analytic processes to arrive at a possible diagnosis.…”

Exertional dyspnoea and syncope

“…1 2 On echocardiography, cardiac amyloidosis is characterised by the presence of a non-dilated thickened left ventricle with evidence of diastolic dysfunction and impaired long axis contraction ( fig 1). [8][9][10] The left ventricular ejection fraction is preserved until late in the disease. 7 Dilation of the right ventricle is seen in severe disease and indicates poor prognosis.…”