Surgical results of unifocalization revision

Midline unifocalization has been developed for the surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. All patients will eventually require reoperation due to the presence of a conduit, and some may also require revision of the distal unifocalized bed. The purpose of this study was to evaluate the surgical results of unifocalization revision.

This was a retrospective review of 254 patients who underwent midline unifocalization for treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Forty-eight of 254 patients (18%) have subsequently undergone unifocalization revision. Thirty-two of these patients had previously undergone a single-stage complete repair, whereas 16 had a unifocalization and placement of a central shunt.

For the 32 patients who previously underwent a complete repair, there have been no early or late deaths. The peak systolic pulmonary artery to aortic pressure ratio was 0.44 ± 0.11 after the initial repair and increased to 0.82 ± 0.18 before revision. The pressure ratio decreased to 0.41 ± 0.09 after revision. Three of 32 patients (9%) have subsequently undergone a second unifocalization revision. The 16 patients who previously had a unifocalization/shunt underwent unifocalization revision and complete repair (n = 14) and revision and repeat shunt (n = 2). There was 1 operative mortality and 3 late deaths (25% total) in this cohort. Three (25%) of 12 survivors have subsequently undergone a second unifocalization revision.

The data demonstrate that unifocalization revision can be performed with a successful outcome in a majority of patients. Patients who underwent an initial unifocalization/shunt had a higher failure rate than patients who were initially repaired.