Diagnosis of cardiac involvement in systemic amyloidosis by state-of-the-art echocardiography: where are we now?

Introduction: Echocardiography is the first-line imaging modality used to characterize heart compromise in systemic infiltrative diseases and to evaluate response to therapy. Cardiac amyloidosis results in progressive heart failure due to restriction of ventricular filling. However, in early stages, typical findings may not be evident, even when symptoms and signs of heart failure with preserved ejection fraction are already present. Identification of early compromise is now made possible with the advent of new echocardiography techniques as two-dimensional strain imaging.Areas covered: Diagnostic and prognostic parameters that can be assessed by echocardiography are discussed, including M-mode, two-dimensional and Doppler echocardiography, as well as by new tools as tissue Doppler imaging and strain imaging.Expert opinion: Systemic amyloidosis is a rare disorder resulting from the production of misfolded proteins, primarily light-chain (AL) immunoglobulins and transthyretin (ATTR). The prognosis of affected subjects depends mainly on the presence and the degree of cardiac involvement. Early diagnosis and treatment are mandatory in order to improve survival. We present here a proposal for structured diagnosis, an algorithm based peak systolic longitudinal left ventricular strain, combined with parameters including typical myocardial texture, increased ventricular wall thickness and the presence of some degree of left ventricular diastolic dysfunction.