Transthyretin derived amyloid deposits in the atrium and the aortic valve: insights from multimodality evaluations and mid-term follow up

Abstract

Background Recent studies have reported atrial involvement and coexistence of aortic stenosis in transthyretin (ATTR) cardiac amyloidosis (CA). However, pathological reports of extraventricular ATTR amyloid deposits in atrial structures or heart valves are limited, and the clinical implications of ATTR amyloid deposits outside the ventricles are not fully elucidated. Case presentation We report 3 cases of extraventricular ATTR amyloid deposits confirmed in surgically resected aortic valves and left atrial structures, all of which were unlikely to have significant ATTR amyloidosis infiltrating the ventricles as determined by multimodality evaluation including 99mtechnetium-pyrophosphate scintigraphy, cardiac magnetic resonance, endomyocardial biopsy and their mid-term clinical course up to 5 years. These findings suggested that these were extraventricular ATTR amyloid deposits localized in the aortic valve and the left atrium. Conclusions While long-term observation is required to fully clarify whether these extraventricular ATTR amyloid deposits are truly localized outside the ventricles or are early stages of ATTR-CA infiltrating the ventricles, our 3 cases with multimodality evaluations and mid-term follow up suggest the existence of extraventricular ATTR amyloid deposits localized in the aortic valve and left atrial structures.

Distinctive Deposition Patterns of Sporadic Transthyretin-Derived Amyloidosis in the Atria: A Forensic Autopsy–Based Study

“…In recent years, the evaluation of ATTR deposition in the atria has been given increased attention [8][9][10][11]. We previously found that ATTR deposition patterns within the atrial septum (AS) differed on each side [12].…”

“…2 factor-derived amyloid (AANF) is commonly found in the atria of nearly all old-aged patients with a left-predominant deposition [13][14][15], the deposition pattern of ATTR in the left and right atrial regions remain uninvestigated. Furthermore, several studies have reported the potential colocalisation of ATTR and AANF [8,11,12,16]. However, the relationship between ATTR and AANF is yet to be elucidated.…”