Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly.

Abstract Background Accurate risk stratification of patients with Ebstein's anomaly (EA) is crucial. Aim of the study was to assess the prognostic value of echocardiography, including 2D speckle tracking (STE) derived myocardial deformation indices, for predicting outcome in pediatric and young adult unrepaired EA patients. Methods Fifty consecutive EA patients (1 day–18 years, 52% males) underwent echocardiography and were followed for a mean follow-up of 60 ± 41 months for clinical outcome (ventricular tachyarrhythmia, heart failure, need for surgery and/or death). Clinical and instrumental features of EA patients with stable disease were compared with those of EA patients with progressive disease. Results Twenty-four (48%) EA patients had progressive disease. A more severe grade of tricuspid valve (TV) displacement [59.7 mm/m2 (IQR 27.5–83) vs 28.4 mm/m2 (IQR 17.5–47); p = 0.002], a lower functional right ventricle (RV) fractional area change (FAC) (29.2 ± 7.7% vs 36.7 ± 9.6%; p = 0.004), a higher Celermajer index [0.8 (IQR 0.7–0.98) vs 0.55 (IQR 0.4–0.7); p = 0.000], a lower functional RV-longitudinal strain (−10.2 ± 6.2% vs −16.2 ± 7.3%; p = 0.003) and a lower right atrium peak systolic strain (RA-PALS) (25.2 ± 13.5% vs 36.3 ± 12.5%; p = 0.004) were detected in progressive disease group compared to stable one, respectively. Functional RV-FAC and RA-PALS were independent predictors of progressive disease at multivariate analysis. Conclusion Our study demonstrated for the first time the prognostic role of RV-FAC and RA-PALS in a long-term follow-up of EA young patients. A complete echocardiographic evaluation should be regular part in the evaluation and risk-stratification of EA children. Highlights • Accurate risk stratification of Ebstein's anomaly (EA) patients is crucial. • Echocardiographic parameters may help in predicting outcome in EA patients, • Speckle tracking echo should be a regular part in the evaluation of EA children, [ABSTRACT FROM AUTHOR]