Dataset Information

Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis.

ABSTRACT:

Background

The differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis.

Methods

Seventy-four subjects with AL amyloidosis and presence of late gadolinium enhancement (LGE) pattern typical for cardiac amyloidosis were analyzed. Long axis strain (LAS) and myocardial contraction fraction (MCF), as well as morphological and functional markers, were measured. The primary endpoint was death, while death and heart transplantation served as a composite secondary endpoint.

Results

After a median follow-up of 41 months, 29 out of 74 patients died and 10 received a heart transplant. Left ventricular (LV) functional parameters were reduced in patients, who met the composite endpoint (LV ejection fraction 51% vs. 61%, LAS - 6.9% vs - 10%, GLS - 12% vs - 15% and MCF 42% vs. 69%; p < 0.001 for all). In unadjusted univariate analysis, LAS (HR = 1.05, p < 0.001) and MCF (HR = 0.96, p < 0.001) were associated with reduced transplant-free survival. Kaplan-Meier analyses showed a significantly lower event-free survival in patients with reduced MCF. MCF and LAS performed best to identify high risk patients for secondary endpoint (Log-rank test p < 0.001) in a combined model. Using sequential Cox regression analysis, the addition of LAS and MCF to LV ejection fraction led to a significant increase in the predictive power of the model (χ² (df = 1) = 28.2, p < 0.001).

Conclusions

LAS and MCF as routinely available and robust CMR-derived parameters predict outcome in LGE positive AL amyloidosis. Patients with impaired LV function in combination with reduced LAS and MCF are at the highest risk for death and heart transplantation.

SUBMITTER: Arenja N

PROVIDER: S-EPMC6704553 | biostudies-literature | 2019 Aug

REPOSITORIES: biostudies-literature

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Publications

Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis.

Arenja Nisha N Andre Florian F Riffel Johannes H JH Siepen Fabian Aus dem FAD Hegenbart Ute U Schönland Stefan S Kristen Arnt V AV Katus Hugo A HA Buss Sebastian J SJ

Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance 20190822 1

<h4>Background</h4>The differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis.<h4>Methods</h4>Seventy-four subjects with AL amyloidosis and presence of late gadolinium enhancement (LGE) pattern typical for cardiac amyloidosis were analyzed. Long axis strain (LAS) and myocardial contraction fraction (MCF), as well as morphological and functional markers, were measured. Th ...[more]

PMID: 31434577

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Project description:BackgroundRecent evidence underscores the importance of cardiovascular magnetic resonance (CMR) in light chain amyloidosis (AL amyloidosis). We aimed to comprehensively assess the prognostic significance of CMR parametric mapping in AL amyloidosis.MethodsThis prospective study consecutively included AL amyloidosis patients who underwent CMR imaging before therapy. The statistical analyses included T2, extracellular volume, and native T1 as variates under investigation, adjusted for well-established prognostic markers. The outcome was death from any cause.ResultsIn total, 195 patients (age, 57.2 ± 9.1 years; male/female, 123/72) were recruited. At the median follow-up time (19 months), the survival probability was approximately 67.2% (131/195). T >44 ms, extracellular volume fraction (ECV) >47%, and native T1 >1468 ms were significantly prognostic (all, P < 0.05) but non-significant after adjustment for N-terminal pro-B-type natriuretic peptide (all, P > 0.05) in AL amyloidosis. T2 >44 ms was independently prognostic after correcting for left ventricle (LV) late gadolinium enhancement, LV ejection fraction, LV longitudinal strain, and therapeutic response (all, P < 0.05). In patients achieving deep hematologic response, T2 >44 ms (hazard ratios [HR] 6.611, 95% confidence interval [CI] 1.723-25.361, P = 0.006) was significantly prognostic for mortality after adjustment for cardiac response. Accordingly, T2 >44 ms was significantly associated with mortality (HR 5.734, 95% CI 1.189-27.656, P = 0.030) and remained independently prognostic after correcting for LV late gadolinium enhancement and LV longitudinal strain (both, P < 0.05) in patients who achieved both deep hematologic response and cardiac response.ConclusionThis study highlights that T2 is a valuable independent predictor of mortality in an AL amyloidosis population, additive to common CMR risk factors. Moreover, myocardial edema assessment identified patients in need of adjunctive therapies, which is of particular prognostic significance in patients with deep therapeutic response.

Project description:BackgroundApical sparing of left ventricular (LV) strain can occur in light-chain cardiac amyloidosis (AL-CA). We employed indicators of the strain ratio of the apex to base (RAB) and the relative apical sparing of strain (RAS) on the basis of LV global and segmental strain to distinguish AL-CA from hypertrophic cardiomyopathy (HCM).MethodsIn all, 36 AL-CA patients, 37 HCM patients, and 36 healthy controls underwent 3.0 T cardiac magnetic resonance (CMR) examination. We compared LV strain parameters from CMR tissue tracking (CMR-TT), including global and segmental peak radial strain (PRS), peak circumferential strain (PCS), and peak longitudinal strain (PLS); the peak systolic strain rate in radial, circumferential, and longitudinal directions (PSSR_R, PSSR_C, PSSR_L); and the peak diastolic strain rate in radial, circumferential, and longitudinal directions (PDSR_R, PDSR_C, PDSR_L). We also assessed the values of RAB and RAS. Differences in all groups were compared using an independent t-test and a nonparametric rank sum test.ResultsIn the comparison of global strain parameters, all the peak strain, systolic, and diastolic peak strain rates of the AL-CA group significantly decreased compared with those of the HCM and healthy control groups (all P<0.001). The values of PSSR in all directions were lower in the AL-CA than in the HCM patients (PSSR_R, P<0.001; PSSR_C, P=0.004; PSSR_L, P=0.010) . In the analysis of segmental strain parameters, all peak strains in the basal segment showed significant differences between the AL-CA and HCM groups (all P<0.001). Some strain rate parameters in the basal segment were also noted to be significantly different (PSSR_R, P<0.001; PSSR_L, P<0.001; PDSR_R, P=0.015; PDSR_C, P=0.020). Both the RAB and RAS of peak strain in all directions showed significant differences between the AL-CA and HCM groups (all P<0.001). The RAB of the radial and circumferential PSSR showed statistical differences between the 2 groups (P<0.001 and P=0.001). The RAS in the radial direction of both the PSSR and PDSR was statistically different (P=0.003 and P=0.012).ConclusionsThe CMR-TT technique can be used to quantitatively compare global and segmental strain differences between AL-CA and HCM. In addition, RAB and RAS are reliable parameters for assessing the apical sparing pattern and thus, for distinguishing AL-CA from HCM.

Project description:BackgroundWhile patients with cardiac transthyretin amyloidosis are easily diagnosed with bone scintigraphy, the detection of cardiac light chain (AL) amyloidosis is challenging. Cardiac magnetic resonance (CMR) analyses play an essential role in the differential diagnosis of cardiomyopathies; however, limited data are available from cardiac AL-Amyloidosis. Hence, the purpose of the present study was to analyze the potential role of CMR in the detection of cardiac AL-amyloidosis.MethodsWe included 35 patients with proved cardiac AL-amyloidosis and two control groups constituted by 330 patients with hypertrophic cardiomyopathy (HCM) and 70 patients with arterial hypertension (HT), who underwent CMR examination. The phenotype and degree of left ventricular (LV) hypertrophy and the amount and pattern of late gadolinium enhancement (LGE) were evaluated. In addition, global and regional LV strain parameters were also analyzed using feature-tracking techniques. Sensitivity and specificity of several CMR parameters were analyzed in diagnosing cardiac AL-amyloidosis.ResultsThe sensitivity and specificity of diffuse septal subendocardial LGE in diagnosing cardiac AL-amyloidosis was 88% and 100%, respectively. Likewise, the sensitivity and specificity of septal myocardial nulling prior to blood pool was 71% and 100%, respectively. In addition, a LV end-diastolic septal wall thickness ≥ 15 mm had an optimal diagnostic performance to differentiate cardiac AL-amyloidosis from HT (sensitivity 91%, specificity 89%). On the other hand, a reduced global LV longitudinal strain (< 15%) plus apical sparing (apex-to-base longitudinal strain > 2) had a very low sensitivity (6%) in detecting AL-Amyloidosis, but with very high specificity (100%).ConclusionsThe findings from this study suggest that CMR could have an optimal diagnostic performance in the diagnosis of cardiac AL-amyloidosis. Hence, further larger studies are warranted to validate the findings from this study.

Project description:Background Cardiac amyloidosis (CA) is a disease of interstitial myocardial infiltration, usually by light chains or transthyretin. We used diffusion tensor cardiovascular magnetic resonance (DT-CMR) to noninvasively assess the effects of amyloid infiltration on the cardiac microstructure. Methods DT-CMR was performed at diastole and systole in 20 CA, 11 hypertrophic cardiomyopathy, and 10 control subjects with calculation of mean diffusivity, fractional anisotropy, and sheetlet orientation (secondary eigenvector angle). Results Mean diffusivity was elevated and fractional anisotropy reduced in CA compared with both controls and hypertrophic cardiomyopathy (P<0.001). In CA, mean diffusivity was correlated with extracellular volume (r=0.68, P=0.004), and fractional anisotropy was inversely correlated with circumferential strain (r=-0.65, P=0.02). In CA, diastolic secondary eigenvector angle was elevated, and secondary eigenvector angle mobility was reduced compared with controls (both P<0.001). Diastolic secondary eigenvector angle was correlated with amyloid burden measured by extracellular volume in transthyretin, but not light chain amyloidosis. Conclusions DT-CMR can characterize the microstructural effects of amyloid infiltration and is a contrast-free method to identify the location and extent of the expanded disorganized myocardium. The diffusion biomarkers mean diffusivity and fractional anisotropy effectively discriminate CA from hypertrophic cardiomyopathy. DT-CMR demonstrated that failure of sheetlet relaxation in diastole correlated with extracellular volume in transthyretin, but not light chain amyloidosis. This indicates that different mechanisms may be responsible for impaired contractility in CA, with an amyloid burden effect in transthyretin, but an idiosyncratic effect in light chain amyloidosis. Consequently, DT-CMR offers a contrast-free tool to identify novel pathophysiology, improve diagnostics, and monitor disease through noninvasive microstructural assessment.

Project description:BackgroundThe prognostic significance of left atrial (LA) dysfunction in cardiac amyloidosis is being increasingly acknowledged, but its importance in stage II light-chain cardiac amyloidosis (AL-CA) remains unclear. This study aimed to determine the prognostic value of LA strain in stage II AL-CA.MethodsPatients with stage II AL-CA who underwent cardiac magnetic resonance at Peking Union Medical College Hospital between January 1, 2015, and October 31, 2021, were consecutively enrolled in this cohort study. Telephone and clinical follow-ups were conducted monthly. The primary endpoints were all-cause mortality, and the secondary endpoints were the combination of all-cause mortality and cardiac progression. Cox regression and Kaplan-Meier survival analyses were performed to identify associations between variables and outcomes.ResultsWe included 131 patients with stage II AL-CA [61% male; median age 59 years; interquartile range (IQR), 51-65 years]. Among these patients, 37 (28%) reached the primary endpoint (all-cause mortality), and 52 (40%) reached the secondary endpoint (median follow-up 28 months; IQR, 10-50 months). Serum biomarker N-terminal pro-B-type natriuretic peptide and LA strain were significantly associated with the outcomes (all P values <0.05). In the multivariable models, reduced LA booster strain remained associated with a higher risk of all-cause mortality [hazard ratio (HR) 0.64, 95% confidence interval (CI): 0.42-0.96; P=0.031] and the secondary endpoint (HR 0.68, 95% CI: 0.49-0.93; P=0.015). The metrics of cardiac function that showed superior accuracy in predicting the risk of mortality were LA reservoir strain [concordance index (C-index) 0.72; 95% CI: 0.64-0.80] and LA booster strain (C-index 0.71; 95% CI: 0.64-0.80). The addition of LA booster strain to established prognostic predictors improved the discrimination and goodness of fit (P<0.001).ConclusionsLA booster strain is an independent prognostic indicator in stage II AL-CA and is superior to metrics of LV function. LA strain has potential clinical value as an early prognostic marker and may aid in identifying underdiagnosed patients with poor prognosis.

Dataset Information

Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis.

Background

Methods

Results

Conclusions

Publications

Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis.

Similar Datasets

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