Echocardiography combined with Mayo staging enhances the identification of early mortality risk in patients with light-chain cardiac amyloidosis
Light-chain cardiac amyloidosis(AL-CA)is a fatal cardiomyopathy caused by the deposition of monoclonal immunoglobulin light-chain in the cardiac fibers,conduction system,valves,and other parts of the heart.Both Mayo 2004 and 2012 staging systems are widely used to assess the prognosis of the AL-CA.Nonetheless,prognostic outcomes can significantly vary among patients within the same stage.Patients in the highest-risk categories,Mayo 2004 stage Ⅲb and Mayo 2012 stage Ⅳ,exhibit extremely poor responses to conventional treatment regimens,resulting in high early mortality rates.Considering new drugs have been developed for these high-risk patients,thus a more detailed stratification of high-risk patients is crucial for pinpointing those at extreme risk.Echocardiography is one of the most commonly used tests for diagnosing and evaluating AL-CA.Although myocardial strain and other echocardiographic parameters are predictive for patient outcomes,they are not routinely measured in clinical practices.To investigate whether conventional echocardiographic measurements can enhance the identification of high-risk patients with early mortality in AL-CA on the basis of existing risk stratification,we have continuously enrolled 76 patients(with an average age of 59±10 years)diagnosed with AL-CA at Peking Union Medical College Hospital during May 2020 to December 2022,two-thirds of which are male(i.e.,51 of 76).Their clinical data,echocardiography,and follow-up results were collected.Both univariate and multivariate logistic regression analyses were used to identify risk factors for early mortality,and risk factor thresholds were calculated using receiver operating characteristic(ROC)curves.Endpoint events were assessed using the Kaplan-Meier survival analysis.The diagnostic efficacy of the model was evaluated using the area under the ROC curve and the integrated discrimination improvement(IDI).The follow-up duration varies from 5 to 20 months,with a median value of 10 months.Early mortality,defined as death within 6 months,occurred in 20 patients(26.3％).In comparison,both Mayo 2004 stage Ⅲb and Mayo 2012 stage Ⅳ yielded higher were 6-month mortality rates of 56.5％ and 41.9％,respectively.Significant differences between patients who died within 6 months and those who survived beyond 6 months were observed in various parameters,such as cTnl,NT-proBNP,Mayo 2004 staging,Mayo 2012 staging,left atrial diameter,average left ventricular wall thickness,left ventricular ejection fraction(LVEF),E/e'ratio,TAPSE,inferior vena cava width,and(E/e')/LVEF.Nonetheless,no significant difference exists among NYHA heart function classification,serum free light chain levels,and treatment regimen.Univariate regression analysis revealed that left atrial diameter,average left ventricular wall thickness,LVEF,LVEF＜50％ patient proportion,E/e',TAPSE,inferior vena cava width,Mayo 2004 stage Ⅲb,and Mayo 2012 stage Ⅳ are predictive factors for 6-month mortality in AL-CA patients.ROC curve analysis established a threshold value of 0.5 for(E/e')/LVEF in predicting endpoint events in AL-CA patients.Through incorporating significant echocardiographic indicators into multivariate regression analysis along with the Mayo 2004 stage Ⅲb as a constant parameter,it shows that left atrial diameter,LVEF,E/e'ratio,inferior vena cava width,and(E/e')/LVEF≥0.5 could jointly predict 6-month mortality in AL-CA patients with the Mayo 2004 stage Ⅲb.When compared to the Mayo 2004 stage Ⅲb alone,(E/e')/LVEF≥0.5 combined with the Mayo 2004 stage Ⅲb had an AUC of 0.731 and 0.824,respectively,in the ROC curve.The IDI yielded a value of 0.113(95％CI:0.013-0.213,P=0.026),representing an 11.3％ enhancement in the predictive value of early mortality in AL-CA patients.Therefore,we suggest that routine echocardiography derived E/e'to LVEF ratio can offer added prognostic value for early mortality beyond Mayo staging.
NETL
NSTL
万方数据
维普
