Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up

Background. Ebstein’s anomaly in the severely symptomatic neonate is usually fatal. Until recently, successful repair has not been reported and various palliative operations have been associated with prohibitive mortality. Recently, we published our initial results with biventricular repair in 3 severely symptomatic neonates. We now update our experience with emphasis on the evolution of our surgical technique and the medium-term follow-up of these patients.

Methods. Since 1994, 8 severely symptomatic neonates and young infants underwent biventricular repair by one surgeon. Six had Ebstein’s anomaly and 2 had physiologically similar pathology with severe tricuspid valve dysplasia, cyanosis, and gross cardiomegaly. One Ebstein patient (2 months old) had undergone a Starnes operation elsewhere. Weight of the patients at operation ranged from 2.1 to 6.4 kg (mean 2.7 kg). Five patients had either anatomical (n = 3) or functional (n = 2) pulmonary atresia. Severe (4/4) tricuspid regurgitation was present in all except 1 (Starnes operation), and cardiothoracic ratio exceeded 0.85 in all patients. Echocardiography severity scores were >1.5 in 6 (grade 4/4) and 1.3 in 1 (grade 3/4). Repair consisted of tricuspid valve repair, reduction atrioplasty, relief of right ventricular outflow tract obstruction, partial closure of atrial septal defect, and correction of all associated cardiac defects. Technique of tricuspid valve repair evolved over time: 3 had Danielson-type repairs, 3 had DeVega-type repairs, and 2 had complex repairs.

Results. One patient died in hospital: a 2.1 kg patient with tricuspid dysplasia, anatomical pulmonary atresia, and hypoplastic pulmonary arteries. The other 7 patients are all in functional class I and in sinus rhythm. Although 3 patients had symptomatic tachyarrhythmias before surgery, no child has experienced SVT after discharge. At recent echocardiography 4 patients had mild tricuspid regurgitation, and 2 had mild-moderate (2/4) tricuspid regurgitation. Three patients are now 7 years old, 2 are almost 2 years old, and the remaining 2 patients are 1 year old.

Conclusions. Surgical repair of the severely symptomatic neonate with Ebstein’s anomaly is feasible and safe. The repair appears durable and with good medium-term outcome.