Outcome of Neonates with Ebstein’s Anomaly in the Current Era

Abstract

Ebstein's anomaly is frequently detected before birth, with prenatal detection accounting for the majority of cases in the current population. This study aimed to identify the outcome variables among these infants. The medical records of 59 patients with neonatal Ebstein's anomaly managed at the Asan Medical Center between January, 2001 and June, 2012 were investigated retrospectively. In 46 cases, the diagnosis was made prenatally. Surgical/interventional procedures were performed for 27 of the analyzed patients. Biventricular repair was successful for 12 patients but not for 9 patients with pulmonary atresia. The median follow-up period was 1.96 years (range 0.0-10.4 years). The overall mortality rate was 23.7 % (14/59). Of the 14 deaths, 5 occurred within several hours after birth. The 1- and 5-year survival rates were 78.6 and 76.3 %, respectively. Univariate analysis identified several variables related to the time to death: fetal distress (p = 0.002), prematurity (p = 0.036), low birth weight (p = 0.003), diameter of the atrial septal defect (p = 0.022), and pulmonary stenosis/atresia (p = 0.001). Neither the Carpentier classification (p = 0.175) nor the Celermajer index (p = 0.958) was a significant variable. According to the multivariate analysis, fetal distress (p = 0.004) and pulmonary atresia/stenosis (p < 0.001) were significant determinants of outcome. In conclusion, fetal distress and pulmonary atresia/stenosis are significant predictors of mortality in the current population of patients with neonatal Ebstein's anomaly. A close cooperation of associated clinicians is required for an improvement in outcome. To establish a better surgical strategy for patients with Ebstein's anomaly and pulmonary atresia, studies of larger populations are required.

Paediatric Ebstein’s anomaly: how clinical presentation predicts mortality

“…McElhinney et al 8 described 49 live-born neonates of whom 35 (71%) survived to hospital discharge and beyond 1 month of age. Yu et al 9 examined 59 neonates and showed a 1- and 5 year survival rate of 79% and 76%, respectively. More recently, Freud et al 15 studied perinatal mortality in fetuses with Ebstein’s anomaly or tricuspid valve dysplasia in the current era and found an overall perinatal mortality of 45%.…”

An unusual ultrasonographic manifestation of a fetal Ebstein anomaly

“…A recent study from our center has described the management of 59 neonates with an Ebstein anomaly that had been treated in our division of pediatric cardiology between 2001 and 2012 [7]. In contrast to earlier reports that the majority of neonatal Ebstein anomalies are not diagnosed in the prenatal period [9,10], 46 of the patients (78%) at our center had been prenatally diagnosed with this condition [7]. Two babies in this series died immediately after birth, and among the remaining 57 neonates with an Ebstein anomaly, there were only 2 type-D cases (3.4%).…”

“…Type A is designated as an adherence of the septal and posterior leaflets without a restriction of the functional right ventricle volume; type B means that the right ventricle has atrialized with a normal anterior leaflet; type C refers to cases with a stenotic anterior leaflet; and type D cases manifest with an almost complete atrialization of the ventricle, with the exception of a small infundibular component. Although the precise prevalence of each type of Ebstein anomaly has not been investigated, it is assumed that type D accounts for 3.5% to 8% of cases [7,12] and also that the prenatal diagnosis of type-D is rarer. In type-D cases, all of the leaflet tissue, even the anterior parts, is attached to the right ventricular wall.…”

“…The prenatal diagnosis of an Ebstein anomaly will become more important in the future because the outcomes of surgical and conservative management have improved a great deal in the current era [4,7]. The prognosis for a fetus diagnosed with Ebstein anomaly has been extremely poor in the past with a reported mortality rate as high as 81% [9].…”

“…Fetal echocardiography is a useful tool for the diagnosis of congenital heart disease [2,4,7,8]. However as Ebstein anomalies show a wide spectrum of ultrasonographic manifestations, it can be difficult to make a prenatal diagnosis [2,3,5].…”