Patent ductus arteriosus 'stenting' as a life-saving approach in severe neonatal Ebstein's anomaly.

A critical 1-day-old male neonate was referred to cardiac evaluation because of deep cyanosis due to a severe tricuspid valve Ebstein's anomaly with large atrial right-to-left shunt and duct-dependent pulmonary circulation. Ductus arteriosus re-opening by prostaglandin infusion resulted in significant clinical improvement but, after a few hours, it irreversibly closed, and pulmonary vasodilator treatment with inhaled nitric oxide and oral sildenafil did not significantly increase the oxygen saturation. Therefore, it was decided to proceed to ductal recanalization and stenting as an alternative to the surgical shunt. After the procedure, oxygen saturation was raised to over 90%, allowing the baby to be weaned from mechanical ventilation. At 9-month follow-up, he was asymptomatic and showed a systemic saturation over 90% despite complete closure of the stented ductus. In conclusion, ductus arteriosus stenting might be considered to be a reliable and life-saving therapeutic option in severe forms of Ebstein's anomaly as a temporary support to a multidrug vasoactive therapy.