Outcome in cyanotic neonates with Ebstein's anomaly

Clinical, angiographic, radiographic, and echocardiographic data on 46 neonates with Ebstein's anomaly presenting with cyanosis between 1954 and 1996 were reviewed to determine possible risk factors for mortality. Most patients (67%) presented at birth with 3 cases diagnosed in utero. Mean systemic oxygen saturation was 62+/-12%. An atrial septal defect > or = 4 mm was noted in 20 patients (44%). The patent right ventricle to pulmonary artery connection was present in 10 (22%), and pulmonary atresia was functional in 25 (54%) and anatomic in 11 patients (24%). Fifteen patients (35%) underwent surgical interventions. Total mortality was 70% (vs 14% in acyanotic patients diagnosed during the same time period; p <0.0001) and was related to low cardiac output and hypoxia in 20 patients (62%), postoperative complications in 8 (25%), and sudden death in 4 (13%). Kaplan-Meier survival estimates were 61% at age 1 week (95% confidence interval [CI], 47% to 75%), 48% at age 1 month (95% CI, 34% to 62%), and 36% at both 1 and 5 years of age (95% CI, 22% to 50%). Mortality improved from 81% in 1954 to 1985 to 47% in 1986 to 1996 (p = 0.04). Significant independent predictors of mortality included an atrial septal defect > or = 4 mm (odds ratio [OR] 2.39; p = 0.04), reduced left ventricular function (OR 4.10; p = 0.002), and functional or anatomic pulmonary atresia (OR 2.44, p = 0.003; and OR 5.97, p = 0.004, respectively). An echocardiographic ratio of the combined right atrial and atrialized right ventricular area to the area of the functional right ventricle and left heart >1.0 was 100% predictive of mortality.