Key result
The "cherry on top" strain sign identifies 100% of patients with concomitant CA and AS.
Why the study?
Concomitant cardiac amyloidosis and aortic stenosis may be mistaken for isolated aortic stenosis, which can impact treatment strategy and prognosis.
Does strain echocardiography (specifically the 'cherry on top' sign and RALS) accurately predict concomitant cardiac amyloidosis in patients with severe aortic stenosis?
Cross-Sectional (n=42)
No
Does strain echocardiography (specifically the 'cherry on top' sign and RALS) accurately predict concomitant cardiac amyloidosis in patients with severe aortic stenosis?
Strain echocardiography, particularly the 'cherry on top' sign and elevated RALS, provides high sensitivity for detecting concomitant cardiac amyloidosis in patients with severe aortic stenosis.
May flag possible amyloidosis in aortic stenosis; cross-sectional data leave open prospective validation before clinical adoption.
BACKGROUND: Concomitant cardiac amyloidosis (CA) and aortic stenosis (AS) may be mistaken for isolated AS, potentially impacting the treatment strategy and patient's prognosis. Therefore, it is crucial to distinguish between these conditions, as failure to promptly diagnose CA may lead to considerable complications. The aim of this study is to investigate the diagnostic value of strain predictors in patients with concomitant CA and AS compared to isolated AS. METHODS: mTc-DPD scintigraphy. Those showing Perugini grade 2 or 3 tracer uptakes without evidence of monoclonal gammopathy were diagnosed with CA and underwent speckle-tracking echocardiography. Furthermore, strain analysis was performed to evaluate myocardial deformation, with a focus on detecting apical sparing and reduction in bull's eye mapping, resulting in the characteristic "cherry on top" sign. RESULTS: Eight patients were diagnosed with CA, representing 19.0% of those suspected of concomitant CA and 7.8% of the overall cohort with severe AS. AF arrhythmia was significantly more frequent in these patients compared to those with isolated AS. Echocardiography findings revealed that E/E' ratio and RALS were significantly higher in patients with concomitant CA, while GLS and mean basal LS were significantly lower in this group. The "cherry on top" sign was detected in 19 patients (45.2%), present in 100% of those with concomitant CA and AS, versus 32.4% in isolated AS cases (P = 0.04). This sign demonstrated a sensitivity of 100% and a specificity of 67.6% for predicting concomitant CA and AS. CONCLUSIONS: In conclusion, the "cherry on top" sign was significantly more prevalent in patients with concomitant CA and AS, compared to those with isolated AS, demonstrating a sensitivity of 100% and a specificity of 67.6% for predicting concomitant CA. Moreover, RALS and E/E' ratios were significantly higher in patients with concomitant CA, whereas GLS and mean basal LS were significantly lower in this group.
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Jafarisis et al. (2024) conducted a cross-sectional in Concomitant cardiac amyloidosis and severe aortic stenosis (n=42). Strain echocardiography ("cherry on top" sign) vs. Isolated severe aortic stenosis was evaluated on Presence of "cherry on top" sign (p=0.04). The "cherry on top" sign on strain echocardiography was present in 100% of patients with concomitant cardiac amyloidosis and aortic stenosis compared to 32.4% in isolated aortic stenosis cases.
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