Recent evidence suggests cardiac amyloidosis (CA) is a mostly underdiagnosed condition, particularly in the transthyretin-mediated form, and is a frequent cause of heart failure with preserved ejection fraction (HFpEF) in the elderly. New paradigms about CA also involve the development of disease-modifying specific therapies. This article summarizes these new concepts.
Keywords
Heart Failure; Restrictive Cardiomyopathy; Amyloidosis; Cardiovascular Imaging; Cardiovascular Disease
| Clinical history and physical examination |
| HFpEF, particularly in elderly men (age > 65 years) |
| Angiotensin-converting enzyme inhibitor or beta-blocker intolerance |
| Bilateral carpal tunnel syndrome |
| Lumbar canal stenosis |
| Biceps tendon rupture |
| Unexplained peripheral neuropathy, particularly when associated with autonomic dysfunction |
| Cardiac imaging |
| Scintigraphy showing anomalous grade 2-3 increased cardiac uptake of pyrophosphate-Tc99m |
| Infiltrative phenotype on echocardiogram, with biventricular hypertrophy, pericardial effusion, valve thickening, and interatrial septum thickening |
| Longitudinal strain rate reduction that spares the apical region (“apical sparing” pattern) |
| Restrictive abnormality of ventricular filling with right ventricular wall thickening |
| CMR showing late gadolinium enhancement with diffuse subendocardial or transmural pattern, increased ECV |
| Combined clues |
| Heart failure with unexplained left ventricular wall thickening and a non-dilated ventricular cavity |
| Concentric left ventricular hypertrophy with reduced or non-increased QRS amplitude |
| Reduced left ventricular systolic function despite normal global ejection fraction |
| Aortic stenosis with right ventricular wall thickening, particularly with a paradoxical low flow-low gradient pattern |
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