Automated extracellular volume fraction measurement for diagnosis and prognostication in patients with light-chain cardiac amyloidosis

自动化细胞外容积分数测量用于轻链型心脏淀粉样变性患者的诊断和预后

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Abstract

AIMS: T1 mapping on cardiac magnetic resonance (CMR) imaging is useful for diagnosis and prognostication in patients with light-chain cardiac amyloidosis (AL-CA). We conducted this study to evaluate the performance of T1 mapping parameters, derived from artificial intelligence (AI)-automated segmentation, for detection of cardiac amyloidosis (CA) in patients with left ventricular hypertrophy (LVH) and their prognostic values in patients with AL-CA. METHODS AND RESULTS: A total of 300 consecutive patients who underwent CMR for differential diagnosis of LVH were analyzed. CA was confirmed in 50 patients (39 with AL-CA and 11 with transthyretin amyloidosis), hypertrophic cardiomyopathy in 198, hypertensive heart disease in 47, and Fabry disease in 5. A semi-automated deep learning algorithm (Myomics-Q) was used for the analysis of the CMR images. The optimal cutoff extracellular volume fraction (ECV) for the differentiation of CA from other etiologies was 33.6% (diagnostic accuracy 85.6%). The automated ECV measurement showed a significant prognostic value for a composite of cardiovascular death and heart failure hospitalization in patients with AL-CA (revised Mayo stage III or IV) (adjusted hazard ratio 4.247 for ECV ≥40%, 95% confidence interval 1.215-14.851, p-value = 0.024). Incorporation of automated ECV measurement into the revised Mayo staging system resulted in better risk stratification (integrated discrimination index 27.9%, p = 0.013; categorical net reclassification index 13.8%, p = 0.007). CONCLUSIONS: T1 mapping on CMR imaging, derived from AI-automated segmentation, not only allows for improved diagnosis of CA from other etiologies of LVH, but also provides significant prognostic value in patients with AL-CA.

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