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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Independent Prognostic Value of Stroke Volume Index in Patients With Immunoglobulin Light Chain Amyloidosis
Paolo Milani1,2, Angela Dispenzieri3, Christopher G Scott4
1Division of Hematology, Department of Internal Medicine (P.M., A.D., M.A.G., M.Q.L., F.K.B., S.K., S.R.H., N.L., D.D., J.A.L. Y.L., P.K., R.S.G., Y.L.H., S.R.Z., R.A.K., S.V.R.).
Insights
Stroke volume index (SVI) and left ventricular strain are key echocardiographic predictors of survival in AL amyloidosis. SVI is a practical measure for predicting outcomes in these patients, independent of biomarker staging.
Area of Science:
- Cardiology
- Medical Imaging
- Oncology
Background:
- Cardiac involvement significantly impacts prognosis in AL amyloidosis.
- Echocardiography is crucial for diagnosing AL amyloidosis and assessing cardiac function.
- Prognostic stratification in AL amyloidosis relies heavily on cardiac assessment.
Purpose of the Study:
- To evaluate the prognostic value of echocardiographic variables in AL amyloidosis patients.
- To explore the role of stroke volume (SV) and left ventricular strain in predicting survival.
- To compare echocardiographic predictors with established biomarker staging systems.
Main Methods:
- Analysis of echocardiographic data from 754 AL amyloidosis patients.
- Inclusion of Doppler-derived stroke volume (SV) measurements within 30 days of diagnosis.
- Assessment of reproducibility of SV, myocardial contraction fraction, and left ventricular strain in a separate cohort.
Main Results:
- SV index <33 mL/min, myocardial contraction fraction <34%, and cardiac index <2.4 L/min/m² were highly predictive of survival.
- Left ventricular strain of -14% also demonstrated significant prognostic value.
- These echocardiographic parameters were independent of systolic blood pressure, Mayo staging system, and ejection fraction.
Conclusions:
- SV index and left ventricular strain show comparable prognostic performance in AL amyloidosis.
- SV index is a readily available and reliable echocardiographic measure for predicting survival.
- Integrating SV index into predictive models can enhance prognostic accuracy for AL amyloidosis patients.
Background:
Heart involvement is the most important prognostic determinant in AL amyloidosis patients. Echocardiography is a cornerstone for the diagnosis and provides important prognostic information.
Methods And Results:
We studied 754 patients with AL amyloidosis who underwent echocardiographic assessment at the Mayo Clinic, including a Doppler-derived measurement of stroke volume (SV) within 30 days of their diagnosis to explore the prognostic role of echocardiographic variables in the context of a well-established soluble cardiac biomarker staging system. Reproducibility of SV, myocardial contraction fraction, and left ventricular strain was assessed in a separate, yet comparable, study cohort of 150 patients from the Pavia Amyloidosis Center. The echocardiographic measures most predictive for overall survival were SV index <33 mL/min, myocardial contraction fraction <34%, and cardiac index <2.4 L/min/m2 with respective hazard ratios (95% confidence intervals) of 2.95 (2.37-3.66), 2.36 (1.96-2.85), and 2.32 (1.91-2.80). For the subset that had left ventricular strain performed, the prognostic cut point was -14% (hazard ratios, 2.70; 95% confidence intervals, 1.84-3.96). Each parameter was independent of systolic blood pressure, Mayo staging system (NT-proBNP [N-terminal pro-B-type natriuretic peptide] and troponin), and ejection fraction on multivariable analysis. Simple predictive models for survival, including biomarker staging along with SV index or left ventricular strain, were generated.
Conclusions:
SV index prognostic performance was similar to left ventricular strain in predicting survival in AL amyloidosis, independently of biomarker staging. Because SV index is routinely calculated and widely available, it could serve as the preferred echocardiographic measure to predict outcomes in AL amyloidosis patients.
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