P1616Prognostic value of right atrial strain in pediatric patients with ebstein anomaly. A 44 month follow up study

Abstract

Background: Patients with Ebstein's anomaly (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. Purpose(s): To assess the value of different echocardiographic indices of right ventricle (RV) and atrium (RA), including myocardial deformation parameters, for predicting outcome in a pediatric population with EA. Method(s): A total of 26 consecutive pediatric EA patients (age 2107+/-2403 days, age range 1-6570 days, 61.5% males) underwent echocardiography and were followed for a mean follow-up of 44.1+/-38.6 months (range 1-138 months) for clinical outcomes (MACE: sustained ventricular tachycardia, heart failure hospital admission and death). Baseline data including demographics, ECG and CXR cardio-thoracic ratio were all obtained from medical records at first hospital admission. First echocardiogram available for each patient was analyzed, by operator blinded to clinical data, to extrapolate all standard and advanced parameters, including global longitudinal strain (GLS) by speckle tracking of both RA and RV. Result(s): MACE occurred in 7 (26.9%) patients; they were younger (211.14+/-550.7 vs 2805.6+/-2450.6 days; p=0.011) and had more often a resting oxygen saturation<90% (80% vs 14.3%; p=0.003). QRS duration and cardio-thoracic ratio were similar in both groups. Among the studied echocardiographic parameters, a more severe grade of displacement of tricuspid valve (67.8+/-16 mm/m2 vs 41.1+/-23.6; p=0.023), a lower E/A ratio (0.7+/-0.13 vs 1.2+/-0.34) and lower RA GLS (19.5+/-10.8 vs 33.2+/-11.9; p=0.013) were detected in MACE group. TAPSE, RV fractional area change and RV GLS were similar in both groups. At univariate analysis, variables associated with MACE were: saturation<90% (CI 0.003 to 0.512; p=0.013), TV displacement/BSA (CI 1.001 to 1.124; p=0.045) and RA GLS (CI 0.760 to 0.987; p=0.031). The ROC analysis showed that RA strain <20.1% measured on admission echo had the largest area under the curve (AUC: 0.850) with a 94.7% sensitivity and a 85.7% specificity in predicting MACE. Conclusion(s): Behind classic clinical and instrumental predictors of adverse out-comes, RA strain carries prognostic information regarding major cardiac complications in pediatric EA patients. For this reason, a complete and early echocardiographic evaluation, including myocardial deformation study, should be included in the evaluation and risk-stratification of children with this congenital disorder.