Echocardiographic risk stratification in light chain and transthyretin amyloidosis: a meta-analysis.

Abstract

Aims: The role of echocardiography in amyloidosis prognostication remains undefined in international guidelines. This meta-analysis aims to evaluate associations between echocardiography-derived measurements and clinical outcomes in light chain (AL) and transthyretin (ATTR) amyloidosis.

Methods and results: MEDLINE, Embase, Cochrane Library, and Google Scholar were systematically searched through July 2024 for studies reporting associations between echocardiographic variables [left ventricular global longitudinal strain (LV-GLS), LV ejection fraction (LVEF), tricuspid annular plane systolic excursion (TAPSE), interventricular septum diameter (IVSd), LV mass index (LVMi) and E/e' ratios] and adverse events in AL or ATTR amyloidosis. Prespecified demographic items and clinical outcomes were extracted by two blinded, independent reviewers. The prespecified primary outcome was all-cause mortality. Random-effect models were applied to pool hazard ratios (HR). 94 studies comprising 16158 patients (n = 4788 AL, n = 8241 ATTR, n = 3129 mixed aetiologies) were included. Median follow-up was 22.3 (IQR, 16.9-31.4) months. Higher all-cause mortality risk (HR, 1.10: 95%CI, 1.08-1.12; P < 0.001) was observed per 1% LV-GLS decrement, consistent across AL and ATTR subgroups. Lower all-cause mortality risk was seen with increasing LVEF (per 1%) and TAPSE (per 1 mm) in the overall population (HR_LVEF, 0.98; 95%CI, 0.98-0.98; P < 0.001; and HR_TAPSE, 0.94; 95%CI, 0.93-0.95; P < 0.001) and in AL and ATTR subgroups. Higher E/e' ratios (per 1 unit) were associated with all-cause mortality (HR, 1.02; 95%CI, 1.02-1.03; P < 0.001), consistent across AL and ATTR subtypes. No reliable associations between structural parameters (IVSd, LVMi) and clinical outcomes were found.

Conclusion: Echocardiographic measures of biventricular deformation, systolic and diastolic function, were consistently associated with mortality in amyloidosis, while structural parameters were not. Echocardiography may have an important role in the initial risk stratification of cardiac amyloidosis.