Valve disease in cardiac amyloidosis: an echocardiographic score

Abstract

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.

Early detection of subclinical cardiac amyloidosis: the importance of increasing physician awareness and routine imaging assessment

“…The condition primarily affects the heart but can also impact the nervous system, kidneys, gastrointestinal tract, eyes, and musculoskeletal system, presenting with a variety of clinical symptoms. Cardiac involvement in ATTR includes myocardial stiffness leading primarily to diastolic dysfunction, heart failure, valvular involvement, angina, conduction abnormalities, arrhythmias potentially resulting in sudden cardiac death [1,2]. Therefore, cardiac involvement is the main driver of morbidity and mortality [2].…”