Author + information
- Received December 12, 2018
- Revision received June 21, 2019
- Accepted July 28, 2019
- Published online June 1, 2020.
- aDivision of Nuclear Medicine, Department of Radiology, Brigham and Women’s Hospital, Boston, Massachusetts
- bCV Imaging Program, Cardiovascular Division, Brigham and Women’s Hospital, Boston, Massachusetts
- cCardiac Amyloidosis Program, Division of Cardiology, Department of Medicine, Brigham and Women’s Hospital, Boston, Massachusetts
- ↵∗Address for correspondence:
Dr. Sharmila Dorbala, Cardiac Amyloidosis Program, Division of Nuclear Medicine, Department of Radiology, Cardiovascular Division, Department of Medicine, Brigham and Women’s Hospital and Harvard Medical School, 75 Francis Street, Boston, Massachusetts 02115.
• Cardiac amyloidosis is substantially underdiagnosed and AL amyloidosis, if untreated, is rapidly fatal. Emerging therapies for cardiac amyloidosis increase the urgency for developing noninvasive imaging for early detection and for tracking therapeutic response.
• Classic imaging features on echocardiography and cardiac magnetic resonance, although typical for cardiac amyloidosis, are not specific enough to distinguish light chain amyloidosis from transthyretin amyloidosis.
• Myocardial bone-avid radiotracer uptake is highly specific for transthyretin cardiac amyloidosis when plasma cell dyscrasia has been excluded; it is now replacing the need for biopsy in many patients.
• Detection of early cardiac amyloidosis, quantitation of its burden, and assessment of response to therapy are important next steps for imaging to advance the evaluation and management of cardiac amyloidosis.
Cardiac amyloidosis (CA) is one of the most rapidly progressive forms of heart disease, with a median survival from diagnosis, if untreated, ranging from <6 months for light chain amyloidosis to 3 to 5 years for transthyretin amyloidosis. Early diagnosis and accurate typing of CA are necessary for optimal management of these patients. Emerging novel disease modifying therapies increase the urgency to diagnose CA at an early stage and identify patients who may benefit from these life-saving therapies. The goal of this review is to provide a practical approach to echocardiography, cardiac magnetic resonance, and radionuclide imaging in patients with known or suspected CA.
- amyloid tracers
- cardiac amyloidosis
- cardiac magnetic resonance
- radionuclide imaging
Drs. Dorbala and Falk are supported by a National Institutes of Health RO1 grant (RO1 HL 130563). Dr. Dorbala is supported by an American Heart Association Grant (AHA 16 CSA 2888 0004); has received consulting fees from GE Health Care, Proclara, and Abdominal Aortic Aneurysm; and has received consulting fees and grant support from Pfizer. Dr. Falk has received consulting fees from Ionis Pharmaceuticals and Alnylam Pharmaceuticals; and has received research funding from GlaxoSmithKline. Dr. Cuddy has reported that she has no relationships relevant to the contents of this paper to disclose.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC: Cardiovascular Imaging author instructions page.
- Received December 12, 2018.
- Revision received June 21, 2019.
- Accepted July 28, 2019.
- 2020 American College of Cardiology Foundation
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