Role of imaging in the diagnosis and management of patients with cardiac amyloidosis: State of the art review and focus on emerging nuclear techniques

Wael A. Aljaroudi, Milind Y. Desai, W. H.Wilson Tang, Dermot Phelan, Manuel D. Cerqueira, Wael A. Jaber

Research output: Contribution to journalReview articlepeer-review

46 Scopus citations

Abstract

Amyloidosis is an infiltrative disease characterized by deposition of amyloid fibrils within the extracellular tissue of one or multiple organs. Involvement of the heart, cardiac amyloidosis, is recognized as a common cause of restrictive cardiomyopathy and heart failure. The two major types of cardiac amyloidosis are cardiac amyloid light-chain (AL) and transthyretin-related cardiac amyloidosis (ATTR, mutant and wild types) (Nat Rev Cardiol 2010;7:398-408). While early recognition of cardiac amyloidosis is of major clinical importance, so is the ability to differentiate between subtypes. Indeed, both prognosis and therapeutic options vary drastically depending on the subtype. While endomyocardial biopsy with immunostaining is considered the gold standard, advances in imaging provide an attractive non-invasive alternative. Currently, electrocardiography, echocardiography, and cardiac magnetic resonance imaging are all used in the evaluation of cardiac amyloidosis with varying diagnostic and prognostic accuracy. Yet, none of these modalities can effectively differentiate the cardiac amyloid subtypes. Recent data with 99mTc-phosphate derivatives, previously used as bone seeking radioactive tracers, have shown promising results; these radiotracers selectively bind ATTR, but not AL subtype, and can differentiate subtypes with high diagnostic accuracy. This review will initially present the non-radionuclide imaging techniques and then focus on the radionuclide imaging techniques, particularly 99mTc-DPD and 99mTc-PYP, mechanism of action, performance and interpretation of the study, diagnostic accuracy, prognostic value, future clinical perspective, and outlook.

Original languageEnglish (US)
Pages (from-to)271-283
Number of pages13
JournalJournal of Nuclear Cardiology
Volume21
Issue number2
DOIs
StatePublished - Apr 2014
Externally publishedYes

Keywords

  • Cardiac amyloidosis
  • noninvasive multimodality imaging
  • transthyretin amyloid

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

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