Persistent fenestration may be a marker for physiologic intolerance after Fontan completion

Background We sought to evaluate the medium-term implications of fenestration status.

Conclusions Persistent fenestration was a marker for physiologic intolerance as noted by increased rates of mortality and a higher incidence of Fontan failure/complications. The specificity of pre-Fontan physiologic data for fenestration status may not have the fidelity needed for long-term care and thus, the consequences of decision making regarding fenestration status may not be determined until well after the operation.

Results The fenestration was closed in 260 patients: 185 as a catheter intervention (62%) and 75 (25%) spontaneously. Forty patients (13%) had the fenestration open at a median follow-up period of 5.05 years. Of these patients, catheter-based closure failed in 10 (3%). There was no statistically significant difference in pre-Fontan hemodynamic parameters, such as pulmonary artery pressure and pulmonary vascular resistance between the patients with open fenestration and the ones with closed fenestration. Patients with an open fenestration had significantly more late deaths (P <.001), Fontan failure (P =.021), and Fontan complications (P =.011) compared with those with a closed fenestration. Multivariable Cox regression revealed open fenestration (P <.001) and indeterminate ventricular morphology (P =.002) as risk factors for death/Fontan failure, and ventricular dysfunction (P =.014) and open fenestration (P =.009) as risk factors for Fontan complications.