PII: S0003-4975(00)01393-X

Abstract

Surgical treatment outcome of symptomatic neonates suffering from Ebstein’s anomaly is rather disappointing, regardless the type of management be it medical therapy or operation. Doctor Knott-Craig’s approach, based upon three cases only, provides an alternative, which includes aggressive medical preparation and surgical repair with two ventricles. This innovative surgical technique associates subtotal closure of the atrial septal defect, extensive resection of the right atrium, and vertical plication of the atrialized chamber. Good results have been observed with a 5 years maximum follow-up. In many centers, such patients would be treated with medical therapy and the outcome would be poor results. The indication issue for a temporary bicavo-pulmonary shunt in neonates with Ebstein’s anomaly is not yet solved. Unloading the right ventricle has the theoretical advantage of improving pulmonary artery flow in case of pulmonary artery atresia. This procedure could be performed without cardiopulmonary bypass. It could delay a hazardous intra-cardiac repair, despite the three successful cases presented by the authors. Undoubtedly, an operative treatment should be carried out in symptomatic neonates because medical treatment has no chance of success. However, the recommendation for a repair indication in case of an asymptomatic neonate could be discussed. The decreasing of fetal pulmonary artery resistances during the first 6 months of life offers a potential clinical improvement. Surgical treatment could then be indicated at a later stage under better conditions.