ReviewEchocardiographic Evaluation of Systolic and Diastolic Function in Patients With Cardiac Amyloidosis
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
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Drs. Liu and Niemann contributed equally to this article.