Percutaneous treatment of pulmonary atresia with intact ventricular septum: pulmonary valve perforation using radiofrequency energy and arterial duct stenting.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare congenital heart disease with a poor prognosis. It displays great anatomical diversity with varying degrees of right ventricular (RV) hypoplasia, which determine the therapeutic approach. Ideally, the goal is to establish a biventricular circulation. This may be achieved through surgical or percutaneous techniques. Pulmonary valve perforation using radiofrequency energy is indicated in patients with membranous atresia, moderate RV hypoplasia (bipartite or tripartite) and non-RV dependent coronary circulation. Arterial duct stenting is occasionally necessary to ensure adequate pulmonary flow. It is thus possible to treat some patients using exclusively percutaneous techniques. We report the first known case in Portugal of a neonate with PA/IVS treated by radiofrequency perforation and subsequent stenting of the arterial duct.