A 4-parameter risk score (pericardial effusion, low QRS voltage, impaired global radial strain, increased wall thickness) accurately diagnosed cardiac light-chain amyloidosis with an AUC of 0.961.
Cohort (n=63)
No
Does a 4-parameter risk score accurately diagnose cardiac involvement and predict overall survival in patients at risk of cardiac light-chain amyloidosis?
A simple 4-parameter risk score using ECG, echocardiography/CMR wall thickness, and strain can accurately identify cardiac involvement and predict survival in patients at risk for AL amyloidosis, which is especially useful when contrast CMR is contraindicated.
Effect estimate: AUC 0.961 (95% CI 0.924-0.997)
Background: Cardiac light-chain amyloidosis (AL CA) portends poor prognosis. Contrast cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) imaging is an important tool in recognizing AL CA. But contraindications to contrast CMR would significantly restrict its clinical application value. Our study aims to construct a convenient risk score to help identify cardiac involvement in patients at risk of AL CA. Moreover, we also investigate whether this risk score could provide prognosis information. Materials and Methods: Sixty-three patients at risk of AL CA were retrospectively included in our study. Basic clinical characters, lab results, 12-lead electrocardiogram data, and cardiac magnetic resonance image data were collected. AL CA was diagnosed according to typical CA LGE pattern. Logistic analysis was used to figure out predictive parameters of AL CA and their β coefficients, further constructing the risk score. Receiver operating characteristics (ROC) curve was used to find the cut-off point best distinguishing AL CA+ from AL CA-patients. Bootstrapping was used for internal validation. All patients were divided into high-risk and low-risk group according to the diagnostic cut-off point, and followed up for survival information. Kaplan-Meier plots and log-rank test were performed to analyze if this score had prognostic value. Results: The risk score finally consisted of 4 parameters: pericardial effusion (PE) (1 point), low electrocardiographic QRS voltages (LQRSV) (1 point), CMR-derived impaired global radial strain (GRS) (13 mm) (2 points). Total score ranged from 0 to 5 points. A cut-off point of 1.5 showed highest accuracy in diagnosing AL CA with an AUC of 0.961 (95% CI: 0.924-0.997, sensitivity: 90.6%, specificity: 83.9%). Kaplan-Meier plots and log-rank test showed that the high-risk group had significantly poor overall survival rates. Conclusion: In patients at risk of AL CA, a risk score incorporating the presence of PE, LQRSV, and CMR-derived impaired GRS and increased LVMWT is predictive of a diagnosis of AL CA by LGE criteria. This risk score may be helpful especially when contrast CMR is not available or contraindicated, and further studies should be considered to validate this score.
Wu et al. (Wed,) conducted a cohort in Cardiac light-chain amyloidosis (n=63). 4-parameter risk score was evaluated on Diagnosis of AL CA (AUC 0.961, 95% CI 0.924-0.997). A 4-parameter risk score (pericardial effusion, low QRS voltage, impaired global radial strain, increased wall thickness) accurately diagnosed cardiac light-chain amyloidosis with an AUC of 0.961.