Abstract 17312: Non-invasive Predictors of Cardiac Outcomes in Adults With Ebstein Anomaly
Aim: To evaluate the value of modern non-invasive diagnostic testing in prediction of cardiac events in Ebstein anomaly.
Methods: 105 consecutive adults [aged 39.4±15.8 (18-83) years, 52 male] with unrepaired Ebstein anomaly underwent protocolised cardiovascular magnetic resonance (CMR). During follow-up, cardiac events including death, sustained tachyarrhythmia, congestive cardiac failure, and transplantation were recorded and patients were censored when they underwent surgical repair.
Results: At latest follow-up (4.18+/-2.9 years), 5/105 (0.5%) in persistent atrial fibrillation (AF), 27/105 (25.7%) patients had presented with new episodes of sustained AT and 2/105 (1.9%) had sustained VT. Cumulative mid-term freedom from AT at 6 months, 1 year, 3 years, 5 years and 7 years was 94.9%, 92.7%, 82.9%, 76.0% and 67.5%, respectively. On univariate Cox proportional hazard analysis, maximum right atrial (RA) indexed volume (p<0.0001), maximum RA indexed area from4 chamber cine view (p<0.005), tricuspid regurgitant fraction (TR%RF) (p<0.005), right ventricular (RV) end-diastolic indexed volume (p<0.05), % apical septal leaflet displacement/left ventricle (LV) length from 4 chamber view (p<0.01), RV/LV volume ratio (p<0.05) were important predictors of AT during follow-up. On multivariate Cox proportional hazard analysis, there were 3 independent predictors of AT in follow up: maximum RA indexed volume [hazard ratio (HR) 1.01, 95% confidence interval (CI) 1.002-1.02,p=0.02], % apical septal displacement/LV length from 4 chamber view (HR1.04, 95%CI 1.01-1.06,p=0.006), and TR%RF (HR1.03, 95%CI 1.001-1.07,p=0.045). A composite score including 1 point for each independent predictor (maximum RA indexed volume >130 ml/m2, %apical displacement / LV length from 4 chamber view> 65%, TR%RF >40%), was found highly predictive of AT (area under curve 0.80, 95% CI 0.71 - 0.87, p<0.0001).
Conclusions: Right-sided volume overload (RA volume and/or TR), severity of Ebstein anomaly (degree of apical displacement) were significantly associated with AT during follow-up in adults with unrepaired Ebstein anomaly. The new composite score described here can be incorporated into the noninvasive, periodic assessment of adults with Ebstein anomaly.
- © 2013 by American Heart Association, Inc.