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

Abstract

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.

Graded Cardiac Response Criteria for Patients With Systemic Light Chain Amyloidosis

“…Therefore, other methods for cardiac response assessment have been explored. These include echocardiographic strain measurement, 15,26,27 extracellular volume measurement using cardiac magnetic resonance, 28 and functional assessment such as the 6minute walk test, 29,30 targeted therapies as well as other large studies assessing natriuretic peptide-based cardiac response.…”

A Risk Score to Diagnose Cardiac Involvement and Provide Prognosis Information in Patients at Risk of Cardiac Light-Chain Amyloidosis

“…In addition to the diagnosis of AL CA, prognosis information is also paramount to patients as well as their clinicians. The prognostic value of PE ( 24 , 42 ), LQRSV ( 31 ), impaired strain ( 43 , 44 ) and LVMWT ( 45 ) has been previously described alone. Nonetheless, to our knowledge, the joint prognostic value has not yet been discussed.…”

Stroke volume and myocardial contraction fraction in transthyretin amyloidosis cardiomyopathy: A systematic review

“…Recent studies have shown the predictive value of several echocardiographic features, such as LVEF, average strain rate, E/e' , TAPSE, and SVi (10,11). In 2011, Bhuiyan et al (14) wanted to evaluate the end-diastolic pressure-volume relation and other pressure-volume indices in patients with TTR-CA to determine how these indices change over time and whether abnormal pressurevolume relations and indices of pump function were associated with reduced survival.…”

“…Indeed, the disease progression is accompanied by a progressive impairment of systolic left ventricular function and a decrease in left ventricular diastolic volume, leading to a decline in stroke volume (SV) not necessarily associated with a decreased of LVEF. Recent studies have shown the predictive value of staging system based on biomarkers and several echocardiographic measurements of central cardiac function, but only few studies focused on SV (6)(7)(8)(9)(10)(11).…”