Positron emission tomography (PET) utilizing Pittsburgh compound B (PIB) for detection of amyloid heart deposits in hereditary transthyretin amyloidosis (ATTR)

Abstract

PIB-PET, in contrast to DPD scintigraphy, has the potential to specifically identify cardiac amyloid depositions irrespective of amyloid fibril composition. The heart appears to be a target organ for amyloid deposition in ATTR amyloidosis.

Reduced left atrial myocardial deformation irrespective of cavity size: a potential cause for atrial arrhythmia in hereditary transthyretin amyloidosis

“…The absence of cardiac biopsy in these patients weakens the explanation of atrial infiltration, although the findings are consistent. However, a previous study from our group has shown the presence of amyloid infiltration in the heart of hereditary ATTR patients irrespectively of fibril type [18]. Our findings in hereditary ATTR cannot be automatically transferred to other types of amyloidosis.…”

“…Despite LV involvement caused by amyloid depositions in the ATTR amyloidosis group [18], and irrespective of LV wall thickness, there was no significant difference in LA size when compared to the control group. This is an unexpected finding, since the LA, described as the "Cinderella of the left heart", is commonly enlarged in any form of LV disease [19,20].…”

Cardiomyopathies

“…61 Marked elevations in T1 and extracellular volume 15 may be useful for diagnosis and monitoring response to therapy. 62 Encouraging results of treatment have been reported by Pilebro et al 63 using the tracer C-Pittsburgh compound B, which has been shown to detect cardiac amyloid by positron emission tomography (PET). 63 A definitive diagnosis of this condition still requires histological verification.…”

PET-CMR in heart failure - synergistic or redundant imaging?

“…These phenotypes have been associated with differences in amyloid fibril composition – type A (full length TTR and fragments) and type B (full length TTR) [55]. Pilebro et al investigated the role of 11 C–Pittsburgh compound B (PIB) to differentiate these subtypes of ATTR, and showed that global LV 11 C–PIB was higher in patients with Type B V30 M ATTR [56]. …”