Catheter-Based Completion of the Fontan Circuit
A Nonsurgical Approach
A 3-year-old girl had an unbalanced atrioventricular septal defect, hypoplastic left ventricle, and aortic coarctation. She underwent placement of a right ventricle-to-pulmonary artery conduit and arch reconstruction as a newborn (Sano procedure). A bidirectional cavopulmonary anastomosis was performed at 5 months of age, at which time a blind connection was created between the roof of the right atrium (RA) and the undersurface of the pulmonary artery (PA) for potential percutaneous completion of the total cavopulmonary (Fontan) circulation (Figures 1 and 2⇓).
At catheterization, access from the right internal jugular vein allowed needle perforation of the PA and passage into the RA through the previous surgical connection. A guide wire was externalized through the femoral vein and a 12-Fr sheath positioned in the PA. A 60-mm covered stent (NuMed Inc, Hopkinton, NY) was then placed to connect the inferior vena cava (IVC) to the PA, completing the Fontan circuit (Figure 3). There was immediate normalization in arterial oxygen saturation. Shortly after extubation, however, hypoxemia developed because of stent migration from the IVC into the RA. A second covered stent was placed to bridge the gap between the IVC and the first stent, with a complete obliteration of the right-to-left shunt (Figure 3). At the 3 month follow-up visit, normalization of oxygen saturation had persisted. To maintain stent patency, daily aspirin and clopidogrel were administered.