Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Abstract

Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy resulting from deposition of misfolded immunoglobulin light chains (AL-CA) or transthyretin (ATTR-CA) proteins in the myocardium. Survival varies between the different subtypes of amyloidosis and degree of cardiac involvement, but accurate diagnosis is essential to ensure initiation of therapeutic interventions that may slow or potentially prevent morbidity and mortality in these patients. As there are now effective treatment options for CA, identifying underlying disease pathogenesis is crucial and can be guided by multimodality imaging techniques such as echocardiography, magnetic resonance imaging, and nuclear scanning modalities. However, as use of cardiac imaging is becoming more widespread, understanding optimal applications and potential shortcomings is increasingly important. Additionally, certain imaging modalities can provide prognostic information and may affect treatment planning. In patients whom imaging remains non-diagnostic, tissue biopsy, specifically endomyocardial biopsy, continues to play an essential role and can facilitate accurate and timely diagnosis such that appropriate treatment can be started. In this review, we examine the multimodality imaging approach to the diagnosis of CA with particular emphasis on the prognostic utility and limitations of each imaging modality. We also discuss how imaging can guide the decision to pursue tissue biopsy for timely diagnosis of CA.

Case Report and Literature Review of Cardiac Amyloidosis: A Not-So-Rare Cause of Heart Failure

“…For the remaining patients that do not fulfill all these criteria, it will be required an endomyocardial biopsy positive for amyloid deposits or extracardiac biopsy positive for amyloid, in addition to echocardiogram or CMR suggestive of cardiac amyloidosis [10]. As mentioned, non-invasive evaluation is the new backbone for the diagnosis of cardiac amyloidosis [13][14][15]. However no single imaging modality by itself is enough for the diagnosis of cardiac amyloidosis [13][14][15].…”

“…As mentioned, non-invasive evaluation is the new backbone for the diagnosis of cardiac amyloidosis [13][14][15]. However no single imaging modality by itself is enough for the diagnosis of cardiac amyloidosis [13][14][15]. It is necessary an approach with multiple imaging modalities, including echocardiography, CMR and nuclear techniques, in addition to clinical features [13][14][15].…”

“…However no single imaging modality by itself is enough for the diagnosis of cardiac amyloidosis [13][14][15]. It is necessary an approach with multiple imaging modalities, including echocardiography, CMR and nuclear techniques, in addition to clinical features [13][14][15]. Multimodality imaging, mainly echocardiography and CMR, is also important for the prognosis and follow-up of these patients [13][14][15].…”

World Heart Federation Consensus on Transthyretin Amyloidosis Cardiomyopathy (ATTR-CM)

“…These may be of particular importance in early disease, when the echo changes are subtle and less specific [107,108]. Scores help to objectify the echo parameters and a more widespread use could decrease delays in diagnosis and treatment [111].…”

“…At present, there is no established value in repeating scans to evaluate disease burden in patients with ATTR-CM. However, the potential benefits of repeating scans have not been extensively studies in the context of current ATTR-CM treatment approaches [111].…”