Author + information
- Received February 5, 2020
- Revision received April 2, 2020
- Accepted April 2, 2020
- Published online June 1, 2020.
- Mazen Hanna, MDa@mazhanna,
- Frederick L. Ruberg, MDb,
- Mathew S. Maurer, MDc,
- Angela Dispenzieri, MDd,e,
- Sharmila Dorbala, MDf,
- Rodney H. Falk, MDf,
- James Hoffman, MDg,
- Wael Jaber, MDa,
- Prem Soman, MD, PhDh,
- Ronald M. Witteles, MDi and
- Martha Grogan, MDj,∗ (, )@marthagrogan1
- aDepartment of Cardiovascular Medicine, Amyloidosis Center, Cleveland Clinic, Cleveland, Ohio
- bSection of Cardiovascular Medicine, Department of Medicine and Amyloidosis Center, Boston University School of Medicine, Boston Medical Center, Boston, Massachusetts
- cDivision of Cardiology, Department of Medicine, Columbia University Medical Center, New York, New York
- dDivision of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota
- eDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
- fDivision of Cardiology, and Amyloidosis Program, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts
- gDivision of Hematology, University of Miami, Miami, Florida
- hDivision of Cardiology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
- iStanford Amyloid Center, Stanford University School of Medicine, Stanford, California
- jDepartment of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota
- ↵∗Address for correspondence:
Dr. Martha Grogan, Department of Cardiovascular Diseases, Mayo Clinic, 200 SW First Street, Rochester, Minnesota 55905.
• Technetium-labeled cardiac scintigraphy may be used to diagnose transthyretin cardiac amyloidosis.
• Light chain amyloidosis must be excluded; biopsy is required if a monoclonal protein is present.
• SPECT imaging is essential to confirm myocardial uptake.
• Ongoing studies will determine the utility of cardiac scintigraphy in less selected populations.
Technetium-labeled cardiac scintigraphy (i.e., Tc-PYP scan) has been repurposed for the diagnosis of transthyretin amyloid cardiomyopathy (ATTR-CM). Validated in cohorts of patients with heart failure and echocardiographic and/or cardiac magnetic resonance imaging findings suggestive of cardiac amyloidosis, cardiac scintigraphy can confirm the diagnosis of ATTR-CM only when combined with blood and urine testing to exclude a monoclonal protein. Multisocietal guidelines support the nonbiopsy diagnosis of ATTR-CM using cardiac scintigraphy, yet emphasize its use in the appropriate clinical context and the crucial need to rule out light chain amyloid cardiomyopathy. Although increased awareness of ATTR-CM and the advent of effective therapy have led to rapid adoption of diagnostic scintigraphy, there is heterogeneity in adherence to consensus guidelines. This perspective outlines clinical scenarios wherein findings on technetium-labeled cardiac scintigraphy have been misinterpreted, reviews causes of false-negative and false-positive results, and provides strategies to avoid costly and potentially fatal misdiagnoses.
- diagnostic testing
- Technetium-labeled bone scintigraphy
Dr. Hanna has served on Advisory Boards for Alnylam, Pfizer, Eidos Therapeutics, and Akcea Therapeutics. Dr. Ruberg is supported by National Institutes of Health (NIH) grant R01HL139671; and has received research grants from Pfizer, Eidos Therapeutics, and Akcea Therapeutics. Dr. Maurer is supported by NIH grants R01HL139671, R21AG058348, and K24AG036778; has received consulting income from Pfizer, GlaxoSmithKline, Eidos Therapeutics, Prothena, Akcea Therapeutics, Ionis Pharmaceuticals, and Alnylam; and his institution has received clinical trial funding from Pfizer, Prothena, Eidos Therapeutics, and Alnylam. Dr. Dizpenzieri has received research support from Celgene, Takeda, Janssen, Pfizer, and Alnylam; and has received consulting fees from Intellia Therapeutics and Akcea Therapeutics. Dr. Dorbala is supported by NIH grant 1 RO1 HL 130563 and American Heart Association grant AHA19SRG34950011; has received research grants from Pfizer and GE; has been a consultant for GE; and has served on Advisory Boards for Proclara, Pfizer, and GE. Dr. Falk has received consulting fees from Alnylam, Akcea Therapeutics, Ionis Therapeutics, and Eidos Therapeutics; and has received research grants from Akcea Therapeutics and Eidos Therapeutics. Dr. Soman has received grant funding to his institution from Pfizer; and has served on Advisory Boards for Pfizer, Alnylam, Ionis Therapeutics, and Eidos Therapeutics. Dr. Witteles has been a consultant for and served on Advisory Boards for Pfizer and Alnylam. Dr. Grogan has received research support from Alnylam, Eidos Therapeutics, Pfizer, and Prothena; and has received consulting fees and honoraria (paid to Mayo Clinic) from Akcea Therapeutics, Alnylam, Eidos Therapeutics, Pfizer, and Prothena. All other authors have reported that they have 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 author instructions page.
- Received February 5, 2020.
- Revision received April 2, 2020.
- Accepted April 2, 2020.
- 2020 American College of Cardiology Foundation
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