Transthyretin Amyloid Cardiomyopathy

Regression of cardiac amyloid load documented by cardiovascular magnetic resonance in a patient with hereditary amyloidosis

“…in cardiac phenotype severity as assessed by CMR within the last 5 years. In principle, hATTR cardiomyopathy may indeed manifest with a slow clinical progression-but almost never shows a substantial regression of cardiac imaging findings as was documented in this case [3].…”

“…Interestingly, the previous thickening of the atrial septum had almost completely disappeared (Figs. 3,4).…”

“…Hereditary ATTR-associated cardiomyopathy usually manifests with a hypertrophic/restrictive phenotype, conduction disease and arrhythmias leading to heart failure, reduced quality of life and finally to death [4]. A non-invasive diagnosis of cardiac amyloidosis is possible either by multi-parametric CMR or repurposed bone scintigraphy [3,5].…”

“…Tafamidis, a TTR stabilizer, was (2019; c, d). Reduced, rather unchanged global and regional longitudinal strain values are measured at both time points shown to slow the progression of ATTR polyneuropathy and was approved for its treatment in numerous countries [3]. Moreover, in the ATTR-ACT trial that included hATTR and wtATTR cardiomyopathy patients, tafamidis was associated with lower all-cause mortality and rates of cardiovascular hospitalizations [13].…”

Cardiac changes associated with vascular aging

“…Cardiac MRI and technetium scintigraphy can aid in the diagnosis. Although the prognosis for ATTRwt has traditionally been poor, novel therapeutic agents such as tafamidis, which stabilizes transthyretin, preventing its dissociation into amyloid, and patisiran, which interferes with amyloid production, can improve prognosis …”