Clinical Features of Symptomatic Neonates with Ebstein's Anomaly

Cho, Hee Jin; Lee, In Sil; Ko, Jae Kon

Abstract

PURPOSE
Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. METHODS: Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. RESULTS: Ten (67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. O2 saturation and pH were lower and cardiothoracic (CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia (P< 0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but O2 saturation was lower in functional atresia group (P< 0.05). 13 patients (87%) were managed with PGE1. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period (mean 37 months). 3 patients (20%) died and none of patients without pulmonary atresia died. CONCLUSION: We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.