Prognostic Value of Right Ventricular Dysfunction in Patients WithALAmyloidosis: Comparison of Different Techniques by Cardiac Magnetic Resonance

Background Right ventricular (RV) dysfunction is common in patients with amyloid light-chain (AL) amyloidosis. While cardiac MRI is the reference standard tool for RV assessment, there are a number of measures of RV function that can be evaluated and it is yet unknown which of these results in the highest prognostic performance in AL amyloidosis. Purpose To examine the prognostic value of various measures of RV function in a bid to find which best predicts outcome in AL amyloidosis. Study Type Single-center, prospective. Subjects In all, 129 patients (mean age, 58 +/- 11 years; 61.2% men) with biopsy-proven AL amyloidosis. Field Strength/Sequence 3.0T / balanced steady-state free-precession cine. Assessment RV ejection fraction (EF), RV fractional area change (FAC), RV long axis strain (LAS), RV free wall longitudinal strain (FWS), RV global longitudinal strain (GLS), and tricuspid annular plane systolic excursion (TAPSE). Statistical Tests Mann-WhitneyU-tests, Student'st-tests, receiver-operating characteristic curves, Kaplan-Meier curves, Cox proportional hazards regression models, and C-statistics. Results During the median follow-up period of 38.0 months (interquartile range, 18.5-58.0 months), all-cause mortality occurred in 95 patients (73.6%). The RVEF, RVGLS, TAPSE, RVFAC, and RVFWS were significant predictors of outcome in univariate Cox regression (allP < 0.001). After adjusting for New York Heart Association (NYHA) class, Mayo staging 2004, left ventricular (LV) EF, and LV mass index, RVFWS (HR [hazard ratio] =1.074; 95% CI [confidence interval]: 1.041-1.108;P < 0.001) was an independent predictor of all-cause mortality and had a higher C-statistic (0.753) compared to the model including RVEF (C-statistic = 0.724,P= 0.034), the model including RVFAC (C-statistic = 0.723,P= 0.033), and the model including RVGLS (C-statistic =0.733,P= 0.011). Data Conclusion RV dysfunction appears to be an independent determinant of outcome in patients with AL amyloidosis. RVFWS is a better predictor of all-cause mortality than RVEF, RVFAC, or RVGLS. Evidence Level 2 Technical Efficacy Stage 5