Outcomes of surgery for young children with multivessel pulmonary vein stenosis

We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery.

Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records.

At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant.

Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option.