Journal of JCIC

Protein-losing enteropathy (PLE) after the Fontan operation is a serious complication that is difficult to manage. One treatment option for PLE is the creation of a fenestration to improve hemodynamic status. The Brockenbrough puncture has traditionally been used to create a Fontan fenestration. The current patient was a 4-year-old boy with hypoplastic left heart syndrome who had undergone a fenestrated extracardiac Fontan operation at the age of 2 years and 4 months. The surgical fenestration closed spontaneously 3 months after Fontan completion. The patient developed PLE at the age of 4 years and 9 months, and underwent catheterization at the age of 4 years and 11 months. The site of Fontan fenestration could not be identified on angiography or transesophageal echocardiography. Intracardiac echocardiography indicated that the region of the spontaneously closed fenestration was thinner than the Fontan conduit and associated with posterior echo enhancement. The region was penetrated with Nykanen™ RF wire, and balloon dilatation was performed up to a final diameter of 6 mm. The present case indicates that intracardiac echocardiography can be useful for identifying the region of fenestration, and that Nykanen™ RF wire can be a viable option for reopening a surgically created fenestration.