Key result
Patients with ATTR-CA demonstrated significantly higher echocardiographic valve score values (median 15.8) compared to patients with AL-CA (median 11.0) and matched controls (median 12.8).
Why the study?
Cardiac amyloidosis may affect all cardiac structures, including the valves.
Does a 31-item echocardiographic valve score help differentiate ATTR cardiac amyloidosis from AL cardiac amyloidosis and unexplained hypertrophy?
Case-Control (n=80)
Single-blind
No
Does a 31-item echocardiographic valve score help differentiate ATTR cardiac amyloidosis from AL cardiac amyloidosis and unexplained hypertrophy?
Absolute Event Rate: 15.8% vs 11%
p-value: p=0.004
A novel 31-item echocardiographic valve score can effectively identify ATTR cardiac amyloidosis and differentiate it from AL amyloidosis and unexplained left ventricular hypertrophy.
Background Cardiac amyloidosis (CA) may affect all cardiac structures, including the valves. Methods 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. Results 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. Conclusions Patients with ATTR-CA have a prominent impairment of mitral valve structure and function, and higher score values. The valve score is quite effective in identifying patients with ATTR-CA among patients with CA or unexplained hypertrophy.
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Aimo et al. (2022) conducted a case-control in Cardiac amyloidosis (n=80). ATTR-CA vs. AL-CA and matched controls was evaluated on Echocardiographic valve score (p=0.004). Patients with ATTR-CA demonstrated significantly higher echocardiographic valve score values (median 15.8) compared to patients with AL-CA (median 11.0) and matched controls (median 12.8).
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