Outcomes of neonatal Ebstein's anomaly without right ventricular forward flow

In neonates with Ebstein's anomaly and absent right ventricular forward flow, pulmonary valve morphology is normal or abnormal. Although initial postnatal presentations of these 2 conditions are similar, clinical courses and therapeutic strategies for each category differ greatly.

Among 29 neonates with Ebstein's anomaly without right ventricular forward flow on initial postnatal echocardiography, 16 had a normal pulmonary valve and 13 had an abnormal pulmonary valve.

During the postnatal follow-up of the normal pulmonary valve group, right ventricular forward flow commenced approximately 10 days after birth (1-15 days). The ductus arteriosus was surgically ligated in 3 neonates to facilitate right ventricular forward flow. Biventricular or 1 1/2 ventricular physiology was eventually achieved in 14 patients in the normal pulmonary valve group (14/16, 88%) and 2 patients in the abnormal pulmonary valve group (2/13, 15.3%). With respect to the preoperative echocardiographic findings, the normal pulmonary valve group had a significantly larger pulmonary valve annulus (8.2 ± 1.4 mm in the normal pulmonary valve group and 6.4 ± 1.8 mm in the abnormal pulmonary valve group, P = .002) and smaller cardiothoracic ratio (0.79 ± 0.05 in the normal pulmonary valve group and 0.85 ± 0.07 in the abnormal pulmonary valve group, P = .03). Mild to moderate pulmonary regurgitation was present in all patients (16/16, 100%) in the normal pulmonary valve group, but 3 patients (3/13, 23%) in the abnormal pulmonary valve group also had pulmonary regurgitation. On logistic regression analysis, only pulmonary valve annulus size remained as an indicator of a normal pulmonary valve (P = .03).

In patients with Ebstein's anomaly and absent right ventricular forward flow, large pulmonary valve annulus size indicated a normal pulmonary valve. Patients with a normal pulmonary valve showed better survival and had a higher probability of achieving biventricular hemodynamics.