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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.
Insights
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.
Background:
Forecasting the prognosis of a child when diagnosed with Ebstein's anomaly is difficult. We, therefore, studied which factors at the time of diagnosis are associated with death during childhood.
Methods:
All consecutive patients (0-18 years) diagnosed with Ebstein's anomaly in the Netherlands between 1980 and 2014 were included. Survival curves were obtained using the Kaplan-Meier method. By using the Cox proportional hazard model, we analysed the factors (at diagnosis) that were associated with death.
Results:
We included 176 patients. Thirty-one patients (18%) died before the age of 18 years. The 1-year survival was 84% and remained stable at 82% from 35 months after diagnosis and onwards. Modified Ross Heart Failure Class 4 at the time of diagnosis was the most important risk factor for death during childhood (HR 12.5, 95% CI 4.4 to 35.9). Furthermore, diagnosis in the neonatal period (HR 4.2, 95% CI 1.5 to 12.0), severe tricuspid valve regurgitation (HR 2.4, 95% CI 1.2 to 5.0), severe right ventricular outflow tract obstruction (HR 3.7, 95% CI 1.8 to 7.7) and a patent ductus arteriosus (HR 2.8, 95% CI 1.3 to 6.0) at the time of diagnosis were univariately associated with death. Multivariable analysis showed that presentation with Heart Failure Class 4 and a ventricular septal defect is the strongest predictor of death in childhood and adolescence.
Conclusion:
Patients with Ebstein's anomaly presenting with Heart Failure Class 4 and a ventricular septal defect have a high risk of death during childhood.