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.