Multi-modality imaging of cardiac amyloidosis: Contemporary update

Abstract

Cardiac amyloidosis is a heterogeneous and challenging diagnostic disease with poor prognosis that is now being altered by introduction of new therapies. Echocardiography remains the first-line imaging tool, and when disease is suspected on echocardiography, cardiac magnetic resonance imaging and nuclear imaging play critical roles in the non-invasive diagnosis and evaluation of cardiac amyloidosis. Advances in multi-modality cardiac imaging allowing earlier diagnosis and initiation of novel therapies have significantly improved the outcomes in these patients. Cardiac imaging also plays important roles in the risk stratification of patients presenting with cardiac amyloidosis. In the current review, we provide a clinical and imaging focused update, and importantly outline the imaging protocols, diagnostic and prognostic utility of multimodality cardiac imaging in the assessment of cardiac amyloidosis.

Cardiac Magnetic Resonance and Amyloidosis: How can it Assist Clinical Reasoning?

“…Different heart diseases can cause increased thickness in the ventricular walls, and these conditions are often difficult to differentiate clinically. 1,2,6,9 CMR can assist in the characterization of the etiology by identifying cases that are secondary to hypertension, cases of hypertrophic cardiomyopathy, Fabry disease, and CA. In this scenario, it is important to use all the tools that the exam provides, especially because both LE patterns and T1 mapping values vary according to etiology, and they can provide information that is essential to diagnosis.…”

“…In the event that CA is confirmed, CMR results will make it possible to proceed to define the type of alteration and establish appropriate treatment as quickly as possible. 1,2,6,9…”

“…Due to the reduced intra-and interobserver variability and the superior capacity for myocardial characterization, CMR can be useful for defining diagnosis in cases where there are doubts after other forms of analysis, such as echocardiography, have been carried out. 1,2,10…”

“…Cardiac magnetic resonance (CMR) can contribute to confirmation of diagnosis and, more recently, to the screening of family members of carriers of hereditary forms of CA. 1,2 In this article, we will review the contributions of CMR in relation to clinical suspicion or screening for CA, initiating with a review of the information that the exam offers and, subsequently, discussing clinical scenarios in which CMR can be most useful.…”

Nuclear Imaging for the Diagnosis of Cardiac Amyloidosis in 2021

“…Nuclear imaging as an alternative diagnostic approach has become more popular among clinicians who suspect cardiac amyloidosis in their patients. In addition, compared to other imaging modalities such as echocardiogram and cardiac magnetic resonance imaging (CMR), nuclear imaging is the only available non-invasive method that can accurately distinguish ATTR amyloidosis from AL cardiac amyloidosis within the appropriate clinical settings [25]. Nuclear imaging has been viewed as an essential part of the diagnosis of cardiac amyloidosis and has been included in the multi-society consensus statement published in 2019 [26,27].…”