Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis.

Autor:	Cohen OC; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Ismael A; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Pawarova B; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Manwani R; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Ravichandran S; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Law S; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Foard D; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Petrie A; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Ward S; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Douglas B; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Martinez-Naharro A; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Chacko L; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Quarta CC; Alexion Pharmaceuticals Inc., Stockley Park,3 Furzeground Way, Hayes, Uxbridge UB11 1EZ, UK., Mahmood S; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Sachchithanantham S; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Lachmann HJ; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Hawkins PN; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Gillmore JD; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Fontana M; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Falk RH; Brigham and Women's Hospital, 75 Francis Street, Boston, MA 02115, USA., Whelan CJ; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK., Wechalekar AD; National Amyloidosis Centre, University College London, Rowland Hill Street, London NW3 2PF, UK.
Jazyk:	angličtina
Zdroj:	European heart journal [Eur Heart J] 2022 Jan 31; Vol. 43 (4), pp. 333-341.
DOI:	10.1093/eurheartj/ehab507
Abstrakt:	Aims: Cardiac involvement, a major determinant of prognosis in AL (light-chain immunoglobulin) amyloidosis, is characterized by an impairment of longitudinal strain (LS%). We sought to evaluate the utility of LS% in a prospectively observed series of patients. Methods and Results: A total of 915 serial newly diagnosed AL patients with comprehensive baseline assessments, inclusive of echocardiography, were included. A total of 628/915 (68.6%) patients had cardiac involvement. The LS% worsened with advancing cardiac stage with mean -21.1%, -17.1%, -12.9%, and -12.1% for stages I, II, IIIa, and IIIb, respectively (P < 0.0001). There was a highly significant worsening of overall survival (OS) with worsening LS% quartile: LS% ≤-16.2%: 80 months, -16.1% to -12.2%: 36 [95% confidence interval (CI) 20.9-51.1] months, -12.1% to -9.1%: 22 (95% CI 9.1-34.9) months, and ≥-9.0%: 5 (95% CI 3.2-6.8) months (P < 0.0001). Improvement in LS% was seen at 12 months in patients achieving a haematological complete response (CR) (median improvement from -13.8% to -14.9% in those with CR and difference between involved and uninvolved light chain <10 mg/L). Strain improvement was associated with improved OS (median not reached at 53 months vs. 72 months in patients without strain improvement, P = 0.007). Patients achieving an LS% improvement and a standard N-terminal pro-brain natriuretic peptide-based cardiac response survived longer than those achieving a biomarker-based cardiac response alone (P < 0.0001). Conclusion: Baseline LS% is a functional marker that correlates with worsening cardiac involvement and is predictive of survival. Baseline LS% and an absolute improvement in LS% are useful additional measures of prognosis and response to therapy in cardiac AL amyloidosis, respectively. (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.)
Databáze:	MEDLINE
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