Recurrent pulmonary vein stenosis in a pediatric bilateral lung transplant recipient

Pulmonary vein stenosis (PVS) is characterized by luminal narrowing from neointimal proliferation, leading to the obstruction of pulmonary venous blood flow. Lung transplantation is considered a definitive treatment for patients with severe and progressive PVS that is unresponsive to treatment. We present a case in which a three-year-old female who received a bilateral lung transplant for PVS was diagnosed with disease recurrence in multiple veins. This case highlights the need to discuss the possibility of PVS returning during pre-transplant counseling and advocates for frequent monitoring in the post-transplant period.