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

https://doi.org/10.1007/s00392-025-02625-4

David Koeckerling (Heidelberg)1, R. Reddy (London)2, C. Eichhorn (Basel)3, V. Braun (Mannheim)4, J. Howard (London)2, Y. Ahmad (San Francisco)5, F. aus dem Siepen (Heidelberg)1, B. Meder (Heidelberg)1, N. Frey (Heidelberg)1, D. Mereles (Heidelberg)1

1Universitätsklinikum Heidelberg Klinik für Innere Med. III, Kardiologie, Angiologie u. Pneumologie Heidelberg, Deutschland; 2Imperial College London National Heart & Lung Institute London, Großbritannien; 3Universitätsspital Basel Klinik für Innere Medizin Basel, Schweiz; 4Medizinische Fakultät Mannheim der Universität Heidelberg Abteilung für Herz- Kreislaufforschung Mannheim, Deutschland; 5University of California Division of Cardiology San Francisco, USA

 

Background: Novel therapies decelerate the progression of cardiac amyloidosis, making precise prognostication essential for guiding therapeutic decision making. However, the role of echocardiography in risk stratification of light chain (AL) or transthyretin (ATTR) amyloidosis remains largely undefined.

Methods: MEDLINE, Embase, Cochrane Library and Google Scholar were systematically searched from inception through July 2024 for non-randomised diagnostic studies reporting associations between echocardiographic variables and adverse events in patients with AL or ATTR amyloidosis. Prespecified demographic items, echocardiographic variables, and clinical outcomes were extracted by two blinded, independent reviewers. Selected parameters were left ventricular global longitudinal strain (LV-GLS), right ventricular free wall strain (RV-FWS), LV ejection fraction (LVEF), tricuspid annular plane systolic excursion (TAPSE), interventricular septum diameter (IVSd), LV mass index (LVMi) and the E/e’ ratio. The prespecified primary outcome was all-cause mortality, prespecified secondary outcomes were cardiovascular mortality and major adverse cardiac events (MACE). Random-effects models using the method of Hartung, Knapp, Sidik and Jonkman were applied to pool hazard ratios (HR).

Results: 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. In the overall population, 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. Decreasing RV-FWS (per 1%) was associated with all-cause mortality (HR 1.11, 95%CI 1.03-1.20, p=0.009) in the overall population. Lower all-cause mortality risk was observed with increasing LVEF (per 1%) and TAPSE (per 1mm) in the overall population (HRLVEF 0.98, 95%CI 0.98-0.98, p<0.001; HRTAPSE 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 subgroups. LV-GLS, RV-FWS, LVEF, TAPSE and E/e’ ratios were also associated with secondary endpoints. No consistent associations between structural parameters (IVSd, LVMi) and clinical outcomes were found.

Conclusion: Echocardiographic measures of biventricular myocardial deformation, systolic and diastolic function, were consistently associated with adverse events in AL and ATTR amyloidosis. Conversely, echocardiographic assessment of structural alterations (IVSd, LVMi) does not appear to substantially enhance risk prediction. Echocardiography may have an important role in the initial risk stratification of cardiac amyloidosis.

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