Background: Congenital pulmonary vein stenosis is a uniformly fatal disease when left untreated. Transcatheter techniques (for example, balloon dilation and stent placement) have proved to be only temporizing measures, and previous surgical attempts at treatment of this entity have provided little improvement and few survivors.
Methods: Over the last 4 years, 6 patients with congenital pulmonary vein stenosis have been treated at our institution, 3 of whom underwent bilateral sequential lung transplantation.
Results: The 3 patients who underwent bilateral lung transplantation are alive and well 6 to 24 months after transplantation. The other 3 died of complications of the disease before donor lungs became available.
Conclusions: Making the diagnosis of congenital pulmonary vein stenosis requires a high index of suspicion, and referral for lung transplantation should be made as soon as the diagnosis is reached. Lung transplantation has resulted in good-quality short to medium-term survival for 3 patients with this otherwise untreatable disease.