Related Experiment Videos
Paediatric Ebstein's anomaly: how clinical presentation predicts mortality.
Lianne M Geerdink1,2, Tammo Delhaas3, Willem A Helbing4
1Department of Paediatric Cardiology, Amalia Children's Hospital, Radboud University Medical Centre, Nijmegen, The Netherlands.
Archives of Disease in Childhood
|March 24, 2018
Summary
Prognosis for Ebstein's anomaly in children is challenging. Heart Failure Class 4 and a ventricular septal defect at diagnosis significantly increase the risk of childhood death.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects
- Clinical Outcomes Research
Background:
- Ebstein's anomaly poses challenges in predicting childhood prognosis.
- Identifying early prognostic factors is crucial for managing affected children.
Purpose of the Study:
- To investigate factors at diagnosis associated with mortality in pediatric patients with Ebstein's anomaly.
- To improve the forecasting of prognosis for children diagnosed with Ebstein's anomaly.
Main Methods:
- A cohort study included 176 pediatric patients (0-18 years) diagnosed with Ebstein's anomaly in the Netherlands (1980-2014).
- Kaplan-Meier survival analysis and Cox proportional hazard models were used to identify risk factors for death.
Main Results:
- 18% of patients died before age 18; 1-year survival was 84%.
- Key risk factors for mortality included Heart Failure Class 4, neonatal diagnosis, severe tricuspid regurgitation, right ventricular outflow tract obstruction, and patent ductus arteriosus.
- Multivariable analysis identified Heart Failure Class 4 and a ventricular septal defect as the strongest predictors of death.
Conclusions:
- Ebstein's anomaly patients presenting with Heart Failure Class 4 and a ventricular septal defect face a high risk of mortality during childhood.
- Early identification of these risk factors can aid in risk stratification and management strategies.