Repair of neonates and young infants with Ebstein's anomaly and related disorders.

Background: Severely symptomatic neonates and young infants with Ebstein's anomaly usually die without surgical intervention. The relative risks and benefits of single-ventricle palliation versus a two-ventricle repair are uncertain. In a recent series, 69% early survival with single-ventricle palliation was reported in 16 neonates with Ebstein's anomaly. Our institutional bias has been to do a two-ventricle repair in all such patients.

Methods: We reviewed our entire surgical experience with a two-ventricle repair in the severely symptomatic neonate (n = 22) and young infant (n = 5). The indications for operation were ventilator dependence, severe cardiac failure, prostaglandin-dependent circulation, and gross cardiomegaly.

Results: Between 1994 and 2006, 27 consecutive patients with Ebstein's anomaly underwent operation. Associated comorbidities included anatomic or functional pulmonary atresia (n = 18), ventricular septal defects (n = 3), small left ventricle (n = 3), hypoplastic branch pulmonary arteries (n = 3), previous cardiac surgery (n = 4), significant intracranial hemorrhage (n = 3), hepatic necrosis and renal insufficiency (n = 3), and malignant tachyarrhythmias (n = 4). Operations consisted of tricuspid valve repair (n = 23) or valve replacement (n = 2), Blalock-Taussig shunt only (n = 1), and bilateral pulmonary arterioplasty with bidirectional Glenn (n = 1). Hospital survival was 74%, and there have been no late deaths during a median follow-up period of 5.4 years (range, 0.2 to 12 years). Three patients required tricuspid valve replacement during the follow-up period. Late arrhythmia requiring medication is present in 1 patient. All patients are currently in New York Heart Association functional class I.

Conclusions: Two-ventricle repair currently has similar early survival compared with single-ventricle palliation. The advantages of a better physiologic repair can be anticipated for a longer follow-up period.