OUTCOME IN CYANOTIC NEONATES WITH EBSTEINS-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 mortali ty. Most patients (67%) presented at birth with 3 cases diagnosed in u tero. Mean systemic oxygen saturation was 62 +/- 12%, An atrial septal defect greater than or equal to 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 anat omic in 11 patients (24%). Fifteen patients (35%) underwent surgical i nterventions, Total mortality was 70% (vs 14% in acyanotic patients di agnosed during the same time period; p <0.0001) and was related to low cardiac output and hypoxia in 20 patients (62%), postoperative compli cations in 8 (25%), and sudden death in 4 (13%). Kaplan-Meier survival estimates were 61% at age 1 week (95% confidence interval [CI], 47% t o 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 1 954 to 1985 to 47% in 1986 to 1996 (p = 0.04), Significant independent predictors of mortality included an atrial septal defect greater than or equal to 4 mm (odds ratio [OR] 2.39; p = 0.04), reduced left ventr icular function (OR 4.10; p = 0.002), and functional or anatomic pulmo nary atresia (OR 2.44, p = 0.003; and OR 5.97, p = 0.004, respectively ), An echocardiographic ratio of the combined right atrial and atriali zed right ventricular area to the area of the functional right ventric le and left heart >1.0 was 100% predictive of mortality, (C) 1998 by E xcerpta Medica, Inc.