Key result
ATTR-CA linked to ~5-point higher echocardiographic valve score vs AL-CA.
Why the study?
Cardiac amyloidosis may affect all cardiac structures, including the heart valves.
Does a novel echocardiographic valve score differentiate ATTR-CA from AL-CA and unexplained hypertrophy in patients evaluated for cardiac amyloidosis?
Case-Control (n=80)
Blinded to final diagnosis (for echocardiographic operators)
No
Does a novel echocardiographic valve score differentiate ATTR-CA from AL-CA and unexplained hypertrophy in patients evaluated for cardiac amyloidosis?
Absolute Event Rate: 15.8% vs 11%
p-value: p=0.004
A comprehensive echocardiographic valve score reveals that ATTR-CA involves significantly greater structural and functional mitral valve impairment than AL-CA, offering a promising tool to aid in differential diagnosis.
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. Funding Acknowledgement Type of funding sources: None.
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Aimo et al. (2022) conducted a case-control in Cardiac Amyloidosis (n=80). Amyloid transthyretin cardiac amyloidosis (ATTR-CA) vs. Amyloid light-chain cardiac amyloidosis (AL-CA) and matched controls was evaluated on Echocardiographic valve score (p=0.004). Patients with ATTR-CA had a significantly higher median echocardiographic valve score (15.8) compared to patients with AL-CA (11.0, p=0.004) and matched controls (12.8, p=0.009).
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