Related Experiment Videos
Prognostic value of echocardiographic parameters in pediatric patients with Ebstein's anomaly
Costantina Prota1, Giovanni Di Salvo2, Jolanda Sabatino2
1Paediatric Cardiology, Royal Brompton Hospital, London, United Kingdom; Heart Department, University Hospital San Giovanni di Dio e Ruggi d'Aragona, Salerno, Italy.
Insights
Echocardiography, including functional right ventricle (RV) fractional area change (FAC) and right atrium peak systolic strain (RA-PALS), can predict disease progression in young Ebstein's anomaly (EA) patients. These measures aid in risk stratification for better patient management.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- Accurate risk stratification is crucial for managing Ebstein's anomaly (EA).
- Echocardiography plays a key role in assessing EA patients.
- Prognostic value of advanced echocardiographic techniques like 2D speckle tracking (STE) needs further evaluation.
Purpose of the Study:
- To assess the prognostic value of echocardiography, including STE-derived myocardial deformation indices.
- To predict clinical outcomes in pediatric and young adult patients with unrepaired EA.
- To compare clinical and instrumental features between stable and progressive EA disease.
Main Methods:
- Fifty consecutive EA patients (1 day-18 years) underwent echocardiography.
- Patients were followed for a mean of 60 months for clinical outcomes.
- Clinical and instrumental features were compared between stable and progressive disease groups.
Main Results:
- 48% of EA patients experienced disease progression.
- Progressive disease was associated with severe tricuspid valve displacement, lower functional right ventricle (RV) fractional area change (FAC), higher Celermajer index, lower RV-longitudinal strain, and lower right atrium peak systolic strain (RA-PALS).
- Functional RV-FAC and RA-PALS were independent predictors of progressive disease.
Conclusions:
- Functional RV-FAC and RA-PALS demonstrate prognostic value in young EA patients.
- Complete echocardiographic evaluation, including these parameters, is recommended for EA risk stratification.
- This study highlights the utility of echocardiography in long-term management of EA.
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.