Neonatal Ebstein Anomaly: A 30-year Institutional Review

This study aimed to review our 30-year, single-center experience of neonates admitted with Ebstein anomaly. Between January 1985 and August 2015, 80 neonates with Ebstein anomaly were managed. The primary outcome measures were early and late survival, freedom from reoperation, and functional status. Pulmonary atresia or critical stenosis occurred in 18 neonates. Twenty-seven (34%) patients required intervention: 13 systemic-to-pulmonary shunts, 5 balloon pulmonary valvotomy, 3 relief of right ventricular outflow tract obstruction, 3 Starnes procedures, and 3 other procedures. Sixty-nine (86%) of the neonates survived to hospital discharge. Overall 15-year survival estimate was 67% (SE = 6.5), with a superior prognosis for those able to be managed medically (15-year survival of 79%, SE = 7.0) compared with those in whom surgical or catheter intervention was undertaken (15-year survival of 45%, SE = 11.2, P = 0.005). For early survivors of neonatal surgery, freedom from reoperation at 10 years was 16% (SE = 8.5). For long-term survivors, 96% were classified as New York Heart Association Class I or II. Neonates with Ebstein anomaly who can be managed without intervention have a good prognosis. Substantial mortality risk remains in those who require intervention, especially those complicated by pulmonary atresia.