Intractable Rare Dis Res. 2017;6(4):291-294. (DOI: 10.5582/irdr.2017.01065)

In vivo quantification of amyloid burden in TTR-related cardiac amyloidosis.

Kollikowski AM, Kahles F, Kintsler S, Hamada S, Reith S, Knuchel R, Rocken C, Mottaghy FM, Marx N, Burgmaier M


Cardiac transthyretin-related (ATTR) amyloidosis is a severe cardiomyopathy for which therapeutic approaches are currently under development. Because non-invasive imaging techniques such as cardiac magnetic resonance imaging and echocardiography are non-specific, the diagnosis of ATTR amyloidosis is still based on myocardial biopsy. Thus, diagnosis of ATTR amyloidosis is difficult in patients refusing myocardial biopsy. Furthermore, myocardial biopsy does not allow 3D-mapping and quantification of myocardial ATTR amyloid. In this report we describe a 99mTc-DPD-based molecular imaging technique for non-invasive single-step diagnosis, three-dimensional mapping and semiquantification of cardiac ATTR amyloidosis in a patient with suspected amyloid heart disease who initially rejected myocardial biopsy. This report underlines the clinical value of SPECT-based nuclear medicine imaging to enable non-invasive diagnosis of cardiac ATTR amyloidosis, particularly in patients rejecting biopsy.

KEYWORDS: Cardiac ATTR amyloidosis, nuclear cardiac imaging, quantification, amyloid burden, myocardial biopsy, cardiac magnetic resonance imaging

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