Author + information
- Received January 18, 2019
- Revision received May 28, 2019
- Accepted June 3, 2019
- Published online November 4, 2019.
- Marianna Fontana, MD, PhDa,b,∗ (, )
- Andrej Ćorović, MA, MB BChira,c,
- Paul Scully, MBBS, MResb,d and
- James C. Moon, MDb,d
- aNational Amyloidosis Centre, University College London, London, United Kingdom
- bInstitute of Cardiovascular Sciences, University College London, London, United Kingdom
- cAddenbrooke’s Hospital, Cambridge, United Kingdom
- dBarts Heart Centre, St. Bartholomew’s Hospital, London, United Kingdom
- ↵∗Address for correspondence:
Dr. Marianna Fontana, Cardiac MR UCL Cardiac CMR Service, University College London (Royal Free Campus), National Amyloidosis Centre, University College London (Royal Free Campus), Rowland Hill Street, London NW3 2PF, United Kingdom.
• Cardiac amyloidosis is an underdiagnosed cause of heart failure, significantly more common than previously thought; with specific treatments now available, diagnosing cardiac amyloidosis is more important than ever.
• Noninvasive imaging methods, including echocardiography, bone scintigraphy, and cardiac magnetic resonance, play a key role in the diagnostic algorithm.
• Noninvasive imaging could also be used to track disease burden, as well as response to therapy.
Cardiac involvement drives prognosis and treatment choices in cardiac amyloidosis. Echocardiography is the first-line examination for patients presenting with heart failure, and it is the imaging modality that most often raises the suspicion of cardiac amyloidosis. Echocardiography can provide an assessment of the likelihood of cardiac amyloid infiltration versus other hypertrophic phenocopies and can assess the severity of cardiac involvement. Visualizing myocardial amyloid infiltration is challenging and, until recently, was restricted to the domain of the pathologist. Two tests are transforming this: cardiac magnetic resonance (CMR) imaging and bone scintigraphy. After the administration of contrast, CMR is highly sensitive and specific for the 2 main types of ventricular myocardial amyloidosis, light chain amyloidosis (AL) and transthyretin amyloidosis (ATTR). CMR structural and functional assessment combined with tissue characterization can redefine cardiac involvement by tracking different disease processes, ranging from amyloid infiltration, to the myocardial response associated with amyloid deposition, through the visualization and quantification of myocardial edema and myocyte response. Bone scintigraphy (paired with exclusion of serum free light chains) is emerging as the technique of choice for distinguishing ATTR from light chain cardiac amyloidosis and other cardiomyopathies; it has transformed the diagnostic pathway for ATTR, allowing noninvasive diagnosis of ATTR without the need for a tissue biopsy in the majority of patients. CMR with tissue characterization and bone scintigraphy are rewriting disease understanding, classification, and definition, and leading to a change in patient care.
Dr. Fontana is supported by a British Heart Foundation Intermediate Clinical Research Fellowship (FS/18/21/33447). Dr. Scully is supported by a British Heart Foundation Clinical Research Training Fellowship (FS/16/31/32185). All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Received January 18, 2019.
- Revision received May 28, 2019.
- Accepted June 3, 2019.
- 2019 American College of Cardiology Foundation
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