Table 1

Comparison of causes, clinical presentation and clinical outcome of the main types of cardiac amyloidosis

Amyloidosis typeClinical presentationClinical outcome
AL Heart failure, often with multiorgan involvement
  especially hepatomegaly, peripheral and autonomic    neuropathy, nephrotic syndrome, macroglossia and periorbital purpura
With treatment and haematological response, patients’ outcomes vary with organ involvement; patients with advanced cardiac involvement may experience sudden cardiac death despite haematological response
Without treatment, patients with cardiac involvement live <1 year
ATTRm Heart failure with peripheral or autonomic neuropathy; only 50% positive family historyCardiomyopathy is slowly progressive, and patients may not present with symptoms until ventricular wall thickening causes functional deficits in cardiac function
Without treatment, patients with ATTR cardiac amyloidosis can survive from years to decades
ATTRwt Heart failure with carpal tunnel syndrome, biceps tendon rupture and spinal stenosis
 Atrial fibrillation is a  common feature, often several years before heart failure
  • AL, amyloid light chain; ATTRm, hereditary transthyretin amyloidosis; ATTRwt, wild-type transthyretin amyloidosis; LV, left ventricular.