Valve disease in cardiac amyloidosis: an echocardiographic score

Cardiac amyloidosis (CA) may affect all cardiac structures, including the valves. From 423 patients undergoing a diagnostic workup for CA we selected 2 samples of 20 patients with amyloid transthyretin (ATTR-) or light-chain (AL-) CA, and age- and sex-matched controls. We chose 31 echocardiographic items related to the mitral, aortic and tricuspid valves, giving a value of 1 to each abnormal item. Patients with ATTR–CA displayed more often a shortened/hidden and restricted posterior mitral valve leaflet (PMVL), thickened mitral chordae tendineae and aortic stenosis than those with AL–CA, and less frequent PMVL calcification than matched controls. Score values were 15.8 (13.6–17.4) in ATTR–CA, 11.0 (9.3–14.9) in AL–CA, 12.8 (11.1–14.4) in ATTR–CA controls, and 11.0 (9.1–13.0) in AL–CA controls (p = 0.004 for ATTR- vs. AL–CA, 0.009 for ATTR–CA vs. their controls, and 0.461 for AL–CA vs. controls). Area under the curve values to diagnose ATTR–CA were 0.782 in patients with ATTR–CA or matched controls, and 0.773 in patients with LV hypertrophy. Patients with ATTR–CA have a prominent impairment of mitral valve structure and function, and higher score values. The valve score may help identify patients with ATTR–CA among patients with CA or unexplained hypertrophy.