G23(P) A 10 year experience of neonates with truncus arteriosus

Abstract

Results In the last 10 years, 11 babies had a documented diagnosis of Ebstein's anomaly. Ten of these were antenatally diagnosed. Four were born prematurely (<37 weeks). Eight babies required resuscitation at birth and nine babies required intubation within the first 24 hours of life. Many of the babies had other significant co-morbidities including: hydrops fetalis, seizure activity, ventriculomegaly, trisomy 21 and persistent pulmonary hypertension (PPHN). Two babies also underwent therapeutic hypothermia. All of the babies born prematurely died within the neonatal period. Of the seven term babies with Ebstein's anomaly, three were discharged home, two were transferred to the Regional Paediatric Cardiology unit and two died on the neonatal unit. The two babies that were transferred the Regional Paediatric Cardiology unit subsequently died. The three surviving infants continue to be followed up. One of these patients is now seven years old and has not required any surgical intervention. Conclusion In our experience, Ebstein's anomaly was uniformly fatal in babies born prematurely. Furthermore, in term babies the survival was 43%. The overall survival of Ebstein's anomaly presenting in the neonatal period in our cohort was 27%. Although this is an outcome of a small cohort of neonates with a rare congenital heart disease, it is important that parents are counselled appropriately antenatally so that they are adequately prepared for the potentially poor outlook of babies born with Ebstein's anomaly in the neonatal period.