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Multimodality imaging of cardiac amyloidosis
  1. Dominik C Benz1,
  2. Sharmila Dorbala2
  1. 1 Department of Nuclear Medicine, University Hospital Zurich, Zurich, Switzerland
  2. 2 Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, Brigham and Women's Hospital, Boston, Massachusetts, USA
  1. Correspondence to Dr Sharmila Dorbala, Brigham and Women's Hospital, Boston, MA 02115, USA; sdorbala{at}

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Learning objectives

  • To discuss clinical features of cardiac amyloidosis (CA).

  • To list typical echocardiography and cardiac magnetic resonance imaging features of CA.

  • To outline multisocietal criteria for diagnosis of transthyretin and light chain CA.

  • To define the central role of bone-avid tracer cardiac single-photon emission computed tomography (SPECT) to diagnose transthyretin CA without a biopsy.

  • To provide an algorithmic approach to diagnosis of CA non-invasively using bone-avid tracer cardiac SPECT.


Systemic amyloidosis is characterised by misfolding of proteins which deposit as insoluble amyloid fibrils in various organs, including the heart. Most cases of cardiac amyloidosis (CA) result from misfolding of immunoglobulin light chains produced by a clonal plasma cell disorder (AL amyloidosis), or transthyretin (TTR) protein produced predominantly in the liver (ATTR amyloidosis). ATTR amyloidosis frequently results from age-related misfolding of the wild-type TTR (ATTRwt), and less commonly, from misfolding of a variant TTR from an autosomal dominant mutation of the TTR gene (ATTRv). The misfolded proteins deposit as insoluble amyloid fibrils in the myocardial interstitial space, disrupting its architecture and causing a prototypical restrictive cardiomyopathy with high mortality. Without treatment, median survival from diagnosis ranges from <6 months for AL-CA to 3–5 years for ATTR-CA.1 Diagnosis is often delayed because of low disease awareness, leading to worse clinical outcomes.2 Fortunately, highly effective treatments are available for amyloidosis. Daratumumab, a CD38 monoclonal antibody directed against plasma cells, when added to standard of care therapy with CyBORD (cyclophosphamide, bortezomib and dexamethasone), substantially improves survival in AL amyloidosis compared with CyBORD alone; daratumumab was approved for treatment of AL amyloidosis in 2022.3 Novel therapies that stabilise (tafamidis, acoramidis) or silence (inotersen, patisiran, vutrisiran) ATTR protein have improved survival and heart failure hospitalisations compared with placebo.4 Currently, tafamidis is the only approved therapy for ATTR-CA. Remarkably, a recent report described three …

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  • X @DorbalaSharmila

  • Contributors DCB prepared the first draft of the manuscript and SD edited the manuscript and added figures and tables.

  • Funding This work was supported by the National Institutes of Health grant K24 HL 157648 (SD) and the Swiss National Science Foundation grant P400PM_199305 (DCB).

  • Competing interests SD received consulting fees from Pfizer, GE Health Care, AstraZeneca, Novo Nordisk. SD received Investigator-initiated grants to her institution from Pfizer, GE Health Care, Attralus, Philips, Siemens.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Author note References which include a * are considered to be key references.