Transcatheter ductal stenting in critical neonatal Ebstein's anomaly

Abstract

Symptomatic neonatal Ebstein's anomaly shows a very poor outcome, most frequently due to duct-dependent pulmonary circulation caused by functional pulmonary valve atresia. In this setting, percutaneous ductal stenting using high-flexibility coronary stents might be a highly cost-effective alternative to systemic-to-pulmonary shunt. Three newborns (age 4.7 +/- 2.9 days; weight 3.0 +/- 0.3 kg) with critical, duct-dependent tricuspid valve Ebstein's anomaly and functional pulmonary atresia unresponsive to prostaglandin infusion and multidrug vasodilator therapy, underwent duct stabilization with high-flexibility, open-cell stents as an alternative to surgical palliation. Their echocardiographic picture predicted a very high mortality rate. In two cases, the ductus arteriosus was recanalized using a combined pharmacological (local prostaglandin infusion) and mechanical (hydrophilic coronary guide-wire manipulation) approach. Stent implantation increased duct diameter from 0.5 +/- 0.7 to 3.2 +/- 0.2 mm (P < 0.0001), resulting in a significant rise in oxygen saturation from 67 +/- 9 to 92 +/- 4% (P < 0.00001). After the procedure, the patients were quickly weaned from mechanical ventilation and discharged after 16 +/- 7 days. Uneventful spontaneous ductal closure ensued in two patients in a few months, and percutaneous oxygen saturation was 87 +/- 3% at the last follow-up session (12 +/- 10 months). In critical newborns with severe, duct-dependent Ebstein's anomaly unresponsive to medical therapy, ductal stenting could be a reliable, safe and cost-effective alternative to either long-term prostaglandin treatment or surgical palliation, in view of lower-risk corrective surgery at older age. This option may also be attempted successfully within a few days of spontaneous ductal closure using a combined pharmacological and mechanical approach.