New pathological insights into cardiac amyloidosis: implications for non-invasive diagnosis

Abstract

In amyloidotic cardiomyopathy, amyloid deposition is highly heterogeneous. Different patterns of infiltration are identifiable, including diffuse, mainly segmental and mainly subendocardial. Awareness of this variability can help the interpretation of ECGs, echocardiograms and magnetic resonance imaging.

Cardiac Amyloid Load

“…However, whether the amyloid burden obtained from a visual field of an EMB represents whole cardiac amyloid load precisely remains debatable. A comparable software-based method for quantification of amyloid deposits has been reported previously in whole hearts obtained from heart transplantation or autopsies (34). Unfortunately, we , patients with a 20% to 40% amyloid load had a significantly longer survival than those patients with smaller or larger loads.…”

Myocardial amyloid infiltration: a less than expected homogeneous process

“…Notably, the variability in LVH is in line with the variable distribution of amyloidotic myocardial infiltration documented by pathological studies 6. Specifically, the occurrence of segmental distribution of amyloid infiltration—leading to increased thickness—has been demonstrated at the histological level 6…”

Cardiac magnetic resonance evaluation of left ventricular remodelling distribution in cardiac amyloidosis

“…Another important finding in our study is that the presence of asymmetry was much higher (69%). Interestingly, a recent histological study of CA26 described a segmental form with predominance in the septum. As mentioned above and demonstrated in figure 4, this pattern resembles that seen in asymmetric hypertrophic cardiomyopathy, so it is imperative for imagers and clinicians to be aware that asymmetric LVH is still consistent with CA, particularly in the appropriate clinical scenario.…”