Correlation between nac and columbia scores and speckle tracking echocardiography: towards better phenotyping of cardiac amyloidosis

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Abstract Background The National Amyloidosis Centre (NAC) and the Columbia Classifications are used to categorize the severity and progression of cardiac amyloidosis. Recent studies indicated that myocardial involvement initially occurs at the atrial level, preceding the manifestation of standard echocardiographic alterations. Understanding how the currently approved clinical classifications correlate with other parameters is pivotal to better define the disease burden at the onset of its manifestation. Purpose To verify the correlation between the two Classifications and other parameters for amyloidotic burden estimation, with a focus on advanced echocardiography techniques (Global Longitudinal Strain, GLS; Peak Atrial Longitudinal Strain, PALS; Peak Atrial Contraction Strain, PACS). Methods A population of 96 patients (age 76.9; 50-91, SD ± 9.2) affected by cardiac amyloidosis was selected: 58 Transthyretin-related Amyloidosis (ATTR), 38 Light-Chain Amyloidosis (AL). Each patient was classified according to both NAC and Columbia systems. Then, NAC and Columbia classifications were correlated with various parameters. The Pearson's correlation was applied to clinical and echocardiographic measurements, such us functional capacity (measured by the 6-min walk test; 6MWT), standard echocardiographic techniques and advanced echocardiographic techniques, including atrial strain (PALS and PACS) and left ventricular strain (GLS). Results The NAC classification shows a positive correlation with the NYHA class (NAC-NYHA, r²=0.281, p£0.005; Columbia-NYHA, r²=0.648, p£0.001), indicating a worsening of the perceived symptoms (NYHA) by patients with increasing NAC class. Both the classifications show a positive correlation with the GLS (NAC-GLS, r²=0.245, p£0.005; Columbia-GLS, r²=0.384, p£0.001), indicating a deterioration of GLS which increases at the worsening of NAC and Columbia classes. Both systems also show a negative correlation with Left Ventricular Ejection Fraction (LVEF; NAC-LVEF, r²=-0.368, p£0.001; Columbia-LVEF, r²=-0.379, p£0.001) and Peak Atrial Longitudinal Strain (PALS; NAC-PALS, r²= -0.255, p£0.005; Columbia-PALS, r²=-0.398, p£0.001), demonstrating a reduction in LVEF and atrial strain with the increasing of NAC and Columbia systems. The latter shows also an inverse correlation with the 6-Min Walk tTest (6MWT; r²-0.472, p£0.001) and a positive correlation with the presence of B-lines on chest ultrasound (r²=0.360, p£0.05). Conclusions The integration between NAC and Columbia values with PALS and GLS can be used to personalize and monitor the therapy over time. The early atrial involvement could be pivotal for developing a new classification that integrates the parameters considered here. Such a classification could assist clinicians in appropriately titrating diuretic therapy, determining the optimal timing for initiating treatment with disease modifier treatments before significant cardiac involvement occurs.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.