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.
- ebstein’s anomaly
- risk factors
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Contributors LMG conceptualised and designed the study, collected study data of all seven sites, carried out the analyses, drafted the initial manuscript and revised the final manuscript as submitted. TD, WAH, IMK, GJdMS, HtH and LR collected data of each one site, critically reviewed the manuscript and approved the final manuscript as submitted. PGMP carried out analyses, revised the initial manuscript and approved the final manuscript as submitted. CLdK conceptualised and designed the study, critically reviewed the manuscript and approved the final manuscript as submitted. LK conceptualised and designed the study, collected study data of all seven sites, drafted the initial manuscript and revised the final manuscript as submitted. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
Presented at The results of this study have been presented at the 50th meeting of the Association for European Paediatric and Congenital Cardiology, June 1–4, Rome, Italy.