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[en] The majority of individuals with cardiac amyloidosis have myocardial amyloid deposits formed from misfolded light chain (AL) or transthyretin (TTR) proteins. Diagnosis of amyloidosis and differentiation between the types is important for prognosis, therapy, and genetic counseling. Cardiac ATTR amyloidosis, the focus of this practice points document, is an under diagnosed cause of heart failure. Amyloid derived from wild-type TTR results in a restrictive cardiomyopathy, most commonly presenting in men in their early 70's onwards, but occasionally seen as young as age 60. Although almost 1 in 4 males > 80 years have some TTR-derived amyloid deposits at autopsy, the clinical significance of a mild degree of deposition is unknown - generally clinical manifestations of heart failure occur once enough amyloid has been deposited to cause LV wall thickening. Approximately 3-4% among US African Americans have a common inherited mutation of the TTR gene (Val122Ile), which produces a restrictive cardiomyopathy in a minority, but may contribute to heart failure in a higher proportion. Cardiac amyloidosis should be suspected in individuals with heart failure and thickened ventricles with grade 2 or greater diastolic dysfunction on echocardiography or typical findings on cardiac magnetic resonance imaging (CMR; diffuse late gadolinium enhancement, ECV expansion or characteristic T-1 relaxation times); diagnosis is confirmed by endomyocardial biopsy and typing of amyloid fibrils as needed. Several studies confirm the high sensitivity and specificity of ^99mTc-bone compound scintigraphy [^99mTc-3,3-diphosphono-1,2- propanedicarboxylic acid (DPD) or PYP for cardiac ATTR amyloidosis; recent studies highlight the value of DPD and/or PYP in differentiating cardiac ATTR from AL amyloidosis. A distinct advantage of ^99mTc-PYP imaging, even when echocardiography and CMR are diagnostic for cardiac amyloidosis, is its ability to specifically identify ATTR cardiac amyloidosis non-invasively and thereby guide patient management. The purpose of this document is to identify the critical components involved in performing ^99mTechnetium-pyrophosphate (^99mTc-PYP) imaging for the evaluation of cardiac transthyretin amyloidosis (ATTR)

[en] This document is the second of 2 companion appropriate use criteria (AUC) documents developed by the American College of Cardiology, American Association for Thoracic Surgery, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, Heart Rhythm Society, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, Society for Cardiovascular Magnetic Resonance, and Society of Thoracic Surgeons. The first document (J Am Coll Cardiol 2017;70:1647-1672) addresses the evaluation and use of multimodality imaging in the diagnosis and management of valvular heart disease, whereas this document addresses this topic with regard to structural (nonvalvular) heart disease. While dealing with different subjects, the 2 documents do share a common structure and feature some clinical overlap. The goal of the companion AUC documents is to provide a comprehensive resource for multimodality imaging in the context of structural and valvular heart disease, encompassing multiple imaging modalities.

Using standardized methodology, the clinical scenarios (indications) were developed by a diverse writing group to represent patient presentations encountered in everyday practice and included common applications and anticipated uses. Where appropriate, the scenarios were developed on the basis of the most current American College of Cardiology/American Heart Association Clinical Practice Guidelines.

A separate, independent rating panel scored the 102 clinical scenarios in this document on a scale of 1 to 9. Scores of 7 to 9 indicate that a modality is considered appropriate for the clinical scenario presented. Midrange scores of 4 to 6 indicate that a modality may be appropriate for the clinical scenario, and scores of 1 to 3 indicate that a modality is considered rarely appropriate for the clinical scenario.

The primary objective of the AUC is to provide a framework for the assessment of these scenarios by practices that will improve and standardize physician decision making. AUC publications reflect an ongoing effort by the American College of Cardiology to critically and systematically create, review, and categorize clinical situations in which diagnostic tests and procedures are utilized by physicians caring for patients with cardiovascular diseases. The process is based on the current understanding of the technical capabilities of the imaging modalities examined.

No abstract available