Table 1.

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

Amyloidosis type	Clinical presentation	Clinical 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 history	Cardiomyopathy 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.