Abstract

Systemic amyloidosis is an uncommon disorder in which amyloid fibrils deposit extracellularly. Three-dimensional speckle-tracking echocardiography (3DSTE) is a novel method able to assess left ventricular (LV) global longitudinal strain (GLS). Our aim was to evaluate the prognostic impact of 3DSTE-derived LV-GLS in patients with cardiac amyloidosis (CA). A total of 35 patients suffering from light-chain (AL) CA or transthyretin (TTR) CA were selected, but 7 patients had to be excluded due to insufficient image quality or were lost for follow-up. With AL-CA 23 cases, while for TTR-CA 5 patients were diagnosed. Complete two-dimensional Doppler and 3DSTE were performed in all subjects. The median follow-up was 201 days (ranging from 36 to 632 days) during which cardiovascular event was detected in 17 CA patients, including 8 cardiac deaths. Six patients were diagnosed with acute heart failure, two patients needed invasive interventions (percutaneous coronary intervention with stent-implantation, implantable cardioverter defibrillator implantation) and in one patient new higher grade atrioventricular block was registered. Using ROC analysis, 3DSTE-derived LV-GLS ≥11.8% (absolute value) was found to be a significant predictor for cardiovascular event-free survival (sensitivity 65%, specificity 64%, area under the curve 0.71, p=.05). Lower LV ejection fraction was confirmed in patients with LV-GLS <11.8% as compared to cases with LV-GLS ≥11.8%. In case of a cardiovascular event, LV-GLS was lower as compared to that of subjects with no events. Multivariable regression analysis confirmed that LV-GLS and LV end-diastolic diameter were independent predictors of cardiovascular survival. 3DSTE-derived LV-GLS is an independent predictor for future cardiovascular events in CA patients.

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