Heart Fail Rev. 2026 Nov 9;31(1):120. doi: 10.1007/s10741-026-10693-5.

ABSTRACT

Cardiac amyloidosis, historically characterized as a restrictive cardiomyopathy, is now understood as a multi-compartmental disorder. Atrial amyloid infiltration-whether ANP-derived isolated atrial amyloidosis secondary to atrial stretch, or primary atrial ATTR infiltration-produces an atrial cardiomyopathy that frequently manifests as atrial fibrillation and a thrombogenic substrate even in sinus rhythm. Cardiac amyloidosis exhibits a high prevalence in heart failure, particularly among elderly patients presenting with preserved ejection fraction. Furthermore, TTR amyloid frequently coexists with degenerative aortic stenosis, and amyloid deposits are also found in the coronary microcirculation and the aorta. Given the current availability of efficacious treatments for amyloidosis, the recognition of these diverse clinical manifestations is paramount for achieving accurate diagnosis, appropriate risk stratification, and timely therapeutic intervention to enhance cardiovascular outcomes. This review frames amyloid cardiovascular disease as a multisystem process affecting the myocardium, atria, valves, and vasculature-often with extracardiac red flags-rather than solely as an infiltrative ventricular cardiomyopathy. Actionable implications currently concentrate on ventricular ATTR/AL cardiomyopathy and concomitant aortic stenosis; coronary and aortic medial deposits remain mechanistically important research targets rather than independently treatable lesions.

PMID:43002279 | DOI:10.1007/s10741-026-10693-5