Comprehensive Approach to Rehabilitation of Atretic Pulmonary Veins

All articles of this category (opens in new window) Background: Pulmonary vein stenosis (PVS) is characterized by relentless progression. Pulmonary vein atresia presents end-stage development of this disease, leading to severe pulmonary hypertension and lung capacity loss. We present our approach to surgical recanalization and further treatment of atretic pulmonary veins. Methods: Six children underwent repair of P vs. with atresia from 2020 to 2025. Preoperative planning included CT with 3D reconstruction and evaluation of catheter angiography (when performed previously). All patients underwent surgical recanalization of one to two atretic pulmonary veins during P vs. repair. Pulmonary veins presenting as atretic fibers were identified and incised until the lumen was reached with aggressive resection of fibrosis prior to completion of atriopericardial “sutureless” anastomosis. All patients remained under rigorous surveillance with echocardiography, CT, and catheter angiography according to the individual plan. Results: P vs. were primary in four and postsurgical in two children. Two patients received previous interventional treatment for PVS. The median age at surgical P vs. repair was 5 months (3–67). All patients showed a reduction of lung parenchymal congestion in postoperative CT. Five patients were receiving sirolimus as antiproliferative therapy. Median follow-up was 28.5 months (1–108). Two patients underwent reoperation for PVS, and three underwent catheter reinterventions. RV pressure was below 40% of LV pressure in five patients and 100% in one patient. Overall, 11 atretic veins were recanalized, with 8 of them remaining patent at the last follow-up. There was one death during follow-up. All surviving patients remain in good functional status. Conclusion: Repair of pulmonary vein stenosis bears a high rate of recurrence, especially in the presence of atresia. Recanalization of pulmonary veins may decompress lung segments and allow for redistribution and collateralization of pulmonary blood flow. A comprehensive multidisciplinary approach consisting of aggressive initial surgery, expected reinterventions, and antiproliferative therapy may achieve rehabilitation of atretic pulmonary veins. Publication History Article published online: 17 February 2026 © 2026. Thieme. All rights reserved. Georg Thieme Verlag KG Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany